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Cadório MJ, Oliveira J, Gama J, Duarte C. Kikuchi-Fujimoto disease and primary Sjögren's syndrome coexisting: A case-based literature review. Mod Rheumatol Case Rep 2025; 9:110-116. [PMID: 39305128 DOI: 10.1093/mrcr/rxae058] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/09/2024] [Revised: 08/24/2024] [Accepted: 09/17/2024] [Indexed: 01/18/2025]
Abstract
Kikuchi-Fujimoto Disease (KFD), also known as Kikuchi histiocytic necrotising lymphadenitis, is an extremely rare and benign condition that mostly affects young women. It is characterised by lymph node involvement with a predilection for the cervical region, commonly presenting with tender lymphadenopathy and a low-grade fever. The diagnosis requires excisional lymph node biopsy with immunohistochemical analysis. KFD is mostly self-limiting within a few weeks to months, with only some patients requiring symptomatic relief with non-steroidal anti-inflammatory drugs or corticosteroids, and a minority developing recurrent episodes of the disease. Importantly, it has been reported in association with systemic lupus erythematosus and, to a lesser extent, other immune-mediated inflammatory rheumatic diseases, such as Sjögren's syndrome, whose clinical presentation itself may include lymphadenopathy. In this paper, we present an unusual case of a woman with primary Sjögren syndrome (pSS) and a past medical history relevant for lymphoma, sarcoidosis, and thymoma, who later developed KFD, a particularly challenging diagnosis in this setting. We then performed a literature review of the association between KFD and pSS, gathering a total of 13 patients, and focusing epidemiological, clinical, and laboratory data.
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Affiliation(s)
| | - João Oliveira
- Rheumatology Department, Coimbra Local Health Unit, Coimbra, Portugal
| | - João Gama
- Anatomical Pathology Department, Coimbra Local Health Unit, Coimbra, Portugal
| | - Cátia Duarte
- Rheumatology Department, Coimbra Local Health Unit, Coimbra, Portugal
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2
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Deb A, Fernandez V, Kilinc E, Bahmad HF, Camps NS, Sriganeshan V, Medina AM. Kikuchi-Fujimoto Disease: A Case Series and Review of the Literature. Diseases 2024; 12:271. [PMID: 39589945 PMCID: PMC11592699 DOI: 10.3390/diseases12110271] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/26/2024] [Revised: 10/22/2024] [Accepted: 10/28/2024] [Indexed: 11/28/2024] Open
Abstract
Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare, self-limiting disorder characterized by fever typically lasting for 1 week up to 1 month and painful necrotizing lymphadenopathy, primarily affecting young adults of Asian ancestry. Although the exact cause remains unclear, infectious and autoimmune mechanisms have been implicated in the pathogenesis of the disease. In this case series, we aim to describe the histopathological features of KFD over a ten-year period at Mount Sinai Medical Center of Florida, and review the current understanding of its pathogenesis, clinical presentation, diagnosis, and management. A retrospective review of our pathology database between January 2013 and May 2024 was performed to identify patients diagnosed with KFD at our institution. Eight cases of KFD were identified, with a mean age of 35 years (range 24-49) and slight male predilection (5:3). Three patients exhibited leukopenia, and two had concurrent HIV infection. One patient developed systemic lupus erythematosus (SLE), and another developed IgA nephropathy during follow-up. Histopathological examination revealed the characteristic features of KFD, including lymph node architectural effacement, histiocytic infiltration, and necrosis. In conclusion, KFD remains a diagnostic challenge due to its overlapping clinical features with other infectious and autoimmune diseases, particularly SLE. While most cases resolve spontaneously, long-term follow-up is warranted due to the potential for recurrence and autoimmune associations.
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Affiliation(s)
- Arunima Deb
- The Arkadi M. Rywlin M.D. Department of Pathology and Laboratory Medicine, Mount Sinai Medical Center, Miami Beach, FL 33140, USA; (A.D.); (V.F.); (E.K.); (V.S.); (A.M.M.)
| | - Vielka Fernandez
- The Arkadi M. Rywlin M.D. Department of Pathology and Laboratory Medicine, Mount Sinai Medical Center, Miami Beach, FL 33140, USA; (A.D.); (V.F.); (E.K.); (V.S.); (A.M.M.)
| | - Ekim Kilinc
- The Arkadi M. Rywlin M.D. Department of Pathology and Laboratory Medicine, Mount Sinai Medical Center, Miami Beach, FL 33140, USA; (A.D.); (V.F.); (E.K.); (V.S.); (A.M.M.)
| | - Hisham F. Bahmad
- The Arkadi M. Rywlin M.D. Department of Pathology and Laboratory Medicine, Mount Sinai Medical Center, Miami Beach, FL 33140, USA; (A.D.); (V.F.); (E.K.); (V.S.); (A.M.M.)
| | - Nicholas S. Camps
- Department of Internal Medicine, Mount Sinai Medical Center, Miami Beach, FL 33140, USA;
| | - Vathany Sriganeshan
- The Arkadi M. Rywlin M.D. Department of Pathology and Laboratory Medicine, Mount Sinai Medical Center, Miami Beach, FL 33140, USA; (A.D.); (V.F.); (E.K.); (V.S.); (A.M.M.)
- Department of Pathology, Herbert Wertheim College of Medicine, Florida International University, Miami, FL 33199, USA
| | - Ana Maria Medina
- The Arkadi M. Rywlin M.D. Department of Pathology and Laboratory Medicine, Mount Sinai Medical Center, Miami Beach, FL 33140, USA; (A.D.); (V.F.); (E.K.); (V.S.); (A.M.M.)
- Department of Pathology, Herbert Wertheim College of Medicine, Florida International University, Miami, FL 33199, USA
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Sheikh JZ, Kila L, Amin I, Khan U, Casserly B. Kikuchi-Fujimoto Disease With Isolated Mediastinal Lymphadenopathy: A Rare Presentation of an Even Rarer Disease. Cureus 2024; 16:e72921. [PMID: 39628743 PMCID: PMC11613967 DOI: 10.7759/cureus.72921] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 11/03/2024] [Indexed: 12/06/2024] Open
Abstract
Kikuchi-Fujimoto disease (KFD) is a rare self-limiting condition presenting as fever and cervical lymphadenopathy, with only two reported cases with isolated mediastinal lymphadenopathy. Lack of awareness about this condition often results in a high rate of misdiagnosis. We present a case of a 29-year-old Indian male with fever, mucocutaneous ulcers, weight loss, and mediastinal lymphadenopathy on CT. Extensive investigations ruled out malignancy and autoimmune and infectious causes. Endobronchial ultrasound-guided fine needle aspiration revealed necrotizing lymphadenitis, indicating KFD, followed by symptomatic management and reassurance. Awareness is crucial to avoid misdiagnosis and unnecessary treatment due to its potentially serious differentials. Long-term follow-up is imperative due to its association with autoimmune conditions.
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Affiliation(s)
| | - Louay Kila
- Respiratory Medicine, University Hospital Limerick, Limerick, IRL
| | - Irfan Amin
- Respiratory Medicine, University Hospital Limerick, Limerick, IRL
| | - Umar Khan
- Respiratory Medicine, University Hospital Limerick, Limerick, IRL
| | - Brian Casserly
- Respiratory Medicine, University Hospital Limerick, Limerick, IRL
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Fuwa M, Tamai Y, Kato A, Asano M, Mori I, Watanabe D, Morita H. Serum Soluble IL-2 Receptors Are Elevated in Febrile Illnesses and Useful for Differentiating Clinically Similar Malignant Lymphomas from Kikuchi Disease: A Cross-Sectional Study. J Clin Med 2024; 13:3248. [PMID: 38892959 PMCID: PMC11173182 DOI: 10.3390/jcm13113248] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/13/2024] [Revised: 05/25/2024] [Accepted: 05/29/2024] [Indexed: 06/21/2024] Open
Abstract
Background: The use of serum soluble interleukin 2 receptor (sIL-2R) for the diagnosis of febrile illnesses has not been examined. In this study, febrile patients were classified according to etiology and disease, and serum sIL-2R levels were evaluated. We determined whether serum sIL-2R is a useful marker for differentiating between malignant lymphoma (ML) and non-ML patients and between patients with ML and Kikuchi disease, which present similar clinical manifestations. Methods: This study was a cross-sectional study and included 344 patients with uncomplicated hemophagocytic syndrome, who had a fever of 38 °C or higher within 1 week of admission to our institution. Patient serum sIL-2R was measured, and the serum sIL-2R values are shown as median and IQR. Results: Serum sIL-2R increased above the upper reference limit in all disease groups with fever. The serum sIL-2R level in ML patients (n = 13) was 4760 (2120-6730) U/mL and significantly higher (p < 0.001) than the level of 998 (640-1625) U/mL in non-ML patients (n = 331). The serum sIL-2R level in ML patients (n = 13) was also significantly higher (p < 0.001) compared with that in patients with Kikuchi disease (n = 20; 705 (538-1091) U/mL). Conclusions: Serum sIL-2R tends to exceed the upper reference limit in patients with febrile illnesses. We conclude that the measurement of serum sIL-2R is useful for differentiating ML from non-ML and ML from Kikuchi disease.
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Affiliation(s)
- Masayuki Fuwa
- Department of General Medicine and Comprehensive Internal Medicine, Gifu University Graduate School of Medicine, Gifu 501-1194, Japan; (Y.T.); (A.K.); (M.A.); (I.M.); (H.M.)
| | - Yuya Tamai
- Department of General Medicine and Comprehensive Internal Medicine, Gifu University Graduate School of Medicine, Gifu 501-1194, Japan; (Y.T.); (A.K.); (M.A.); (I.M.); (H.M.)
| | - Ayaka Kato
- Department of General Medicine and Comprehensive Internal Medicine, Gifu University Graduate School of Medicine, Gifu 501-1194, Japan; (Y.T.); (A.K.); (M.A.); (I.M.); (H.M.)
| | - Motochika Asano
- Department of General Medicine and Comprehensive Internal Medicine, Gifu University Graduate School of Medicine, Gifu 501-1194, Japan; (Y.T.); (A.K.); (M.A.); (I.M.); (H.M.)
| | - Ichiro Mori
- Department of General Medicine and Comprehensive Internal Medicine, Gifu University Graduate School of Medicine, Gifu 501-1194, Japan; (Y.T.); (A.K.); (M.A.); (I.M.); (H.M.)
| | - Daichi Watanabe
- Center for Advanced Medical Care and Clinical Training, Gifu University Hospital, Gifu 501-1194, Japan;
| | - Hiroyuki Morita
- Department of General Medicine and Comprehensive Internal Medicine, Gifu University Graduate School of Medicine, Gifu 501-1194, Japan; (Y.T.); (A.K.); (M.A.); (I.M.); (H.M.)
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Cheng MH, Xie LJ. Distinguishing Kikuchi-Fujimoto disease from lymphoma in patients by clinical and PET/CT features. Medicine (Baltimore) 2024; 103:e37779. [PMID: 38640333 PMCID: PMC11029934 DOI: 10.1097/md.0000000000037779] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/17/2023] [Revised: 01/19/2024] [Accepted: 03/12/2024] [Indexed: 04/21/2024] Open
Abstract
To develop a scheme for distinguishing Kikuchi-Fujimoto disease (KFD) from lymphoma in patients presenting enlarged lymph nodes (LNs) predominantly on the upper side of the diaphragm. From November 2015 to August 2023, 32 KFD patients and 38 lymphoma patients were pathologically confirmed and enrolled in this retrospectively study. Clinical and 18F-fluorodeoxyglucose positron emission tomography (PET)/computed tomography (CT) features were collected. When comparing those PET/CT parameters, we set 5 models with different research objects: (1) all affected LNs; (2) the 5 largest affected LNs in terms of maximum diameter; (3) the 5 largest affected LNs in terms of maximum standard uptake values (SUVmax); (4) the largest affected LNs in terms of maximum diameter; (5) the largest affected LNs in terms of SUVmax. Compared to lymphoma patients, KFD patients were younger; and with higher incidence of fever, arthralgia, abnormal serum white blood cell, lactate dehydrogenase (LDH) and splenomegaly; lower incidence of affected LNs perinodal infiltration, necrosis and conglomeration; more affected LNs in Head and Neck nodes (particularly in level II) and Axillary in KFD (P ˂ .05). PET/CT parameters presented as various difference in each model. Finally, 11 clinical and PET/CT features (age ≤ 34, with fever, arthralgia, abnormal white blood cell, abnormal LDH, and without node necrosis and node conglomeration have a score of 2 each; splenomegaly, perinodal infiltration, median maximum diameter ≤ 20.5 and median SUVmax ≤ 7.1 of affected LNs in model 2 have score of 1 each) were selected as scheme items for distinguishing KFD from lymphoma. Individuals who have a total score > 8, meet the criteria for KFD. Sensitivity and specificity were high: 86.8% (95% CI: 71.9%, 95.5%) and 96.9% (95% CI: 83.7%, 99.5%), AUC = 0.975 (95% CI: 90.5%, 99.6%), respectively. It can effectively distinguish KFD from lymphoma by clinical and PET/CT parameters.
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Affiliation(s)
- Mu-Hua Cheng
- Department of Nuclear Medicine, The Third Affiliated Hospital of Sun Yat-Sen University, Guangzhou, Guangdong, China
| | - Liang-Jun Xie
- Department of Nuclear Medicine, The Third Affiliated Hospital of Sun Yat-Sen University, Guangzhou, Guangdong, China
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Razak AA, Shanmugasundaram S. Kikuchi-Fujimoto disease, a rare benign disease with atypical histomorphology: more than meets the eye. Pathology 2024; 56:382-390. [PMID: 38296677 DOI: 10.1016/j.pathol.2023.10.017] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/08/2023] [Revised: 10/03/2023] [Accepted: 10/18/2023] [Indexed: 02/02/2024]
Abstract
Kikuchi-Fujimoto disease (KFD) is a benign self-limiting condition primarily affecting young females. It usually presents with fever and cervical lymphadenopathy of unknown aetiology with a preponderance of the Asian population. Histopathology is critical in making an accurate diagnosis. While the typical microscopic features include paracortical necrosis with debris, histiocytosis with immunoblasts, and absent neutrophils, rarely, KFD can show atypical features like marked immunoblastic proliferation mimicking lymphoma, demonstrate vasculitis mimicking lupus erythematosus, etc. The diagnosis is extremely challenging if such features occur in cases with generalised lymphadenopathy, which is infrequent in KFD. The study aims to describe the morphological, clinical, and immunohistochemical features of KFD and determine the frequency of the atypical features. We also analysed the subtle histological and immunohistochemical features that aid in the diagnosis of atypical cases. Cases reported as KFD over a period of 6 years were retrieved from the archives of histopathology. The morphological features were categorised as typical and atypical. In the atypical cases, the features that aided in the correct diagnosis of KFD were analysed. Out of the 42 cases evaluated, 23.9% (n=10) had generalised lymphadenopathy; 57.2% (n=24) were women with a median age of 25 years. Leukopenia was observed in 42% (n=13) of patients. Typical features were present in 76.2% (n=32) cases and 23.8% (n=10) presented with atypical features. Eight cases were antinuclear antibody-positive. Atypical features included five (50%) cases with vasculitis and panniculitis, and three (30%) cases with large, atypical cells for which immunohistochemistry (IHC) was performed. In two of these cases, the patent sinuses, absence of neutrophils, and IHC with CD68 aided the diagnosis. There is an overlap of clinical and histopathological features between KFD and malignant lymphomas and systemic lupus erythematosus. Given the fact that the atypical features (23.8%) are not rare occurrences in KFD, correlations with clinical findings and ancillary studies are essential to avoid misdiagnosis and inadvertent therapy.
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Affiliation(s)
- Ahlam Abdul Razak
- Department of Pathology, PSG Institute of Medical Sciences and Research, Coimbatore, India
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Chen Q, Zhang J, Huang H, Qiu T, Jin Z, Shi Y, Zhu H, Fan L, Li J, Shi W, Miao Y. Histiocytic necrotizing lymphadenitis with hemophagocytic lymphohistiocytosis in adults: A single-center analysis of 5 cases. Immun Inflamm Dis 2024; 12:e1202. [PMID: 38411294 PMCID: PMC10898206 DOI: 10.1002/iid3.1202] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/26/2023] [Revised: 02/05/2024] [Accepted: 02/13/2024] [Indexed: 02/28/2024] Open
Abstract
BACKGROUND Histiocytic necrotizing lymphadenitis (HNL) is a self-limited inflammatory disease of unknown pathogenesis. A very small fraction of patients with HNL could develop hemophagocytic lymphohistiocytosis (HLH), a hyperinflammatory disorder. These patients are diagnosed as HNL with HLH (HNL-HLH). HNL-HLH in the pediatric population has been systemically studied, however, the clinical, laboratory, and radiological features and outcomes of adult patients with HNL-HLH remain to be explored. We aimed to explore the clinical, laboratory, and radiological features and outcomes of adult patients with HNL-HLH. METHODS We collected the clinical data of patients with HNL-HLH admitted to the First Affiliated Hospital of Nanjing Medical University from October 2010 to June 2015. All the patients underwent lymph node biopsy and have a pathological diagnosis of HNL. The age, gender, clinical presentation, lymph node signs, laboratory findings and imaging data, and pathological findings of the patients were collected. RESULTS In this study, we reported five adult patients with HNL-HLH. All five patients showed enlarged lymph nodes and prolonged fever. Laboratory findings were consistent with the diagnosis of HLH. 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) showed enlarged lymph nodes with increased FDG uptake and splenic hypermetabolism could be present. All the patients responded well to corticosteroids and had a good prognosis. Two of the five patients were diagnosed with systemic lupus erythematosus during the follow-up. CONCLUSIONS Our study demonstrated that adult patients with HNL-HLH showed distinct clinical, laboratory, and radiological features. And the prognosis is good and patients could be managed with steroids and supportive care.
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Affiliation(s)
- Qingqing Chen
- Department of Hematology, Pukou CLL CenterThe First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, Collaborative Innovation Center for Cancer Personalized MedicineNanjingChina
| | - Jing Zhang
- Department of Hematology, Pukou CLL CenterThe First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, Collaborative Innovation Center for Cancer Personalized MedicineNanjingChina
| | - Huijun Huang
- Department of Hematology, Pukou CLL CenterThe First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, Collaborative Innovation Center for Cancer Personalized MedicineNanjingChina
| | - Tonglu Qiu
- Department of Hematology, Pukou CLL CenterThe First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, Collaborative Innovation Center for Cancer Personalized MedicineNanjingChina
| | - Ze Jin
- Department of Hematology, Pukou CLL CenterThe First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, Collaborative Innovation Center for Cancer Personalized MedicineNanjingChina
| | - Yu Shi
- Department of Hematology, Pukou CLL CenterThe First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, Collaborative Innovation Center for Cancer Personalized MedicineNanjingChina
| | - Huayuan Zhu
- Department of Hematology, Pukou CLL CenterThe First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, Collaborative Innovation Center for Cancer Personalized MedicineNanjingChina
| | - Lei Fan
- Department of Hematology, Pukou CLL CenterThe First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, Collaborative Innovation Center for Cancer Personalized MedicineNanjingChina
| | - Jianyong Li
- Department of Hematology, Pukou CLL CenterThe First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, Collaborative Innovation Center for Cancer Personalized MedicineNanjingChina
- National Clinical Research Center for Hematologic DiseasesThe First Affiliated Hospital of Soochow UniversitySuzhouChina
| | - Wenyu Shi
- Department of OncologyAffiliated Hospital of Nantong UniversityNantongJiangsuChina
- Department of HematologyAffiliated Hospital of Nantong UniversityNantongJiangsuChina
| | - Yi Miao
- Department of Hematology, Pukou CLL CenterThe First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, Collaborative Innovation Center for Cancer Personalized MedicineNanjingChina
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Liu J, Jiang L, Yu G, Qu G, Cai L. A rare co-existence of histiocytic necrotizing lymphadenitis with metastatic papillary thyroid carcinoma and review of the literature. Diagn Pathol 2024; 19:14. [PMID: 38218846 PMCID: PMC10788022 DOI: 10.1186/s13000-024-01441-0] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/21/2023] [Accepted: 01/07/2024] [Indexed: 01/15/2024] Open
Abstract
Histiocytic necrotizing lymphadenitis (HNL) is a benign, self-limiting disease that is rare clinically. The coexistence of HNL and tumor is rarer. We report a male patient who was preoperatively diagnosed with papillary thyroid carcinoma with cervical lymph nodes metastasis, and the postoperative pathological examination showed histiocytic necrotizing lymphadenitis combined with metastatic papillary thyroid carcinoma in the same single lymph node. More interestingly, Epstein‒Barr virus was positive in these lymph nodes by in situ hybridization. This may suggest a trigger for the coexistence of the two diseases.
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Affiliation(s)
- Jing Liu
- Weifang Medical University, Weifang, China
- Department of Pathology, The Affiliated Yantai Yuhuangding Hospital of Qingdao University, Yantai, China
| | - Lei Jiang
- Department of Pathology, The Affiliated Yantai Yuhuangding Hospital of Qingdao University, Yantai, China
| | - Guohua Yu
- Department of Pathology, The Affiliated Yantai Yuhuangding Hospital of Qingdao University, Yantai, China
| | - Guimei Qu
- Department of Pathology, The Affiliated Yantai Yuhuangding Hospital of Qingdao University, Yantai, China
| | - Li Cai
- Department of Pathology, The Affiliated Yantai Yuhuangding Hospital of Qingdao University, Yantai, China.
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9
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Anini EM, AlBaik TM, Ibrahim AT, AbuKaresh NA, Albandak M, Alshalaldeh R, Natsheh M, Abunejma FM. Kikuchi-Fujimoto Disease: A Case Report of Prolonged Fever and Lymphadenopathy in a Young Girl. J Investig Med High Impact Case Rep 2024; 12:23247096241246618. [PMID: 38605558 PMCID: PMC11010749 DOI: 10.1177/23247096241246618] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/04/2024] [Revised: 02/13/2024] [Accepted: 03/22/2024] [Indexed: 04/13/2024] Open
Abstract
Kikuchi-Fujimoto disease (KFD) is a benign and self-limited disorder that usually presents with regional cervical lymphadenopathy and fever. We report a case of a 12-year-old female who complained of fever, night sweating, significant weight loss, and tender right cervical lymph node enlargement for 2 months. A full workup including laboratory tests and imaging studies, an excisional biopsy, and histopathological analysis were done, and the diagnosis of KFD was confirmed. The patient was treated with analgesia and oral prednisolone, resulting in good improvement. A high degree of clinical suspicion is imperative for physicians, given the rarity of the disease and the associated diagnostic challenges.
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10
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Nam KH, Choi Y. A rare encounter: Kikuchi-Fujimoto disease presenting atypically after BNT162b2 COVID-19 vaccination: a case report. Scand J Rheumatol 2023; 52:705-707. [PMID: 37606559 DOI: 10.1080/03009742.2023.2241760] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/30/2023] [Accepted: 07/25/2023] [Indexed: 08/23/2023]
Affiliation(s)
- K-H Nam
- Department of Dermatology, Jeonbuk National University Medical School, Jeonju, South Korea
- Research Institute of Clinical Medicine of Jeonbuk National University - Biomedical Research Institute, Jeonbuk National University, Jeonju, South Korea
| | - Y Choi
- Research Institute of Clinical Medicine of Jeonbuk National University - Biomedical Research Institute, Jeonbuk National University, Jeonju, South Korea
- Department of Internal Medicine, Division of Rheumatology, Jeonbuk National University Medical School, Jeonju, South Korea
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11
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Nair PR, Bankar NJ, Choudhary A, Shrivastava D. Kikuchi Fujimoto Lymphadenitis: A Rare Association With COVID-19 Vaccination. Cureus 2023; 15:e45979. [PMID: 37900436 PMCID: PMC10600953 DOI: 10.7759/cureus.45979] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/17/2023] [Accepted: 09/25/2023] [Indexed: 10/31/2023] Open
Abstract
Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is an uncommon cause of protracted cervical lymphadenopathy in both children and adults. Although the majority of cases have been documented in Asia, this disease has been characterized globally since it was first identified in Japan in 1972. KFD's etiology is not entirely understood, although various theories have been postulated. Usually benign, Kikuchi-Fujimoto disease resolves within six months. KFD must be distinguished from other causes of chronic lymphadenopathy, such as lymphoma, inflammatory illnesses, autoimmune conditions, viral lymphadenopathy, and also from tuberculosis, especially in India, where it is still endemic. Here, we present the case report of a healthcare worker with a known case of hypothyroidism and autoimmune skin disorder who presented with prolonged fever, joint pain, and generalized lymphadenopathy post-COVID vaccination and was diagnosed as having KFD on biopsy, which can be associated with a very unusual presentation of this disease.
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Affiliation(s)
- Priya R Nair
- Obstetrics and Gynaecology, Datta Meghe Medical College, Datta Meghe Institute of Higher Education and Research, Wardha, IND
| | - Nandkishor J Bankar
- Microbiology, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education and Research, Wardha, IND
| | - Amruta Choudhary
- Obstetrics and Gynaecology, Datta Meghe Medical College, Datta Meghe Institute of Higher Education and Research, Wardha, IND
| | - Deepti Shrivastava
- Obstetrics and Gynaecology, Datta Meghe Medical College, Datta Meghe Institute of Higher Education and Research, Wardha, IND
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12
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Craig JW, Farinha P, Jiang A, Lytle A, Skinnider B, Slack GW. Kikuchi-Fujimoto Disease Following COVID-19 Vaccination: Experience at a Population-Based Referral Center. Am J Clin Pathol 2023; 160:114-118. [PMID: 37526500 DOI: 10.1093/ajcp/aqad032] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/15/2022] [Accepted: 03/03/2023] [Indexed: 08/02/2023] Open
Abstract
OBJECTIVES Multiple case reports describe Kikuchi-Fujimoto disease (KFD) following COVID-19 vaccination, but the true nature of this phenomenon is unknown. The purpose of this study was to further assess the relationship between KFD and COVID-19 vaccination at the population level. METHODS Confirmed KFD cases from January 2018 to April 2022 were identified from provincial pathology archives and analyzed in the context of vaccination statistics from public health resources. RESULTS Our statistical models provide evidence of a temporal association between KFD and both antecedent COVID-19 vaccine administration as well as age-stratified vaccination rates. Eight new cases of plausible COVID-19 vaccine-associated KFD are presented, collectively exhibiting clinical and pathologic features that overlap substantially with those of idiopathic KFD. CONCLUSIONS Our findings indicate that KFD is observed in association with COVID-19 vaccination and suggest that mechanistic studies are warranted.
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Affiliation(s)
- Jeffrey W Craig
- Department of Pathology, University of Virginia Health System, Charlottesville, VA, USA
| | - Pedro Farinha
- Centre for Lymphoid Cancer and Laboratory Medicine, BC Cancer, Vancouver, Canada
- Department of Pathology and Laboratory Medicine, BC Cancer, Vancouver, Canada
- Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, Canada
| | - Aixiang Jiang
- Centre for Lymphoid Cancer and Laboratory Medicine, BC Cancer, Vancouver, Canada
- Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, Canada
| | - Andrew Lytle
- Centre for Lymphoid Cancer and Laboratory Medicine, BC Cancer, Vancouver, Canada
- Department of Pathology and Laboratory Medicine, BC Cancer, Vancouver, Canada
| | - Brian Skinnider
- Centre for Lymphoid Cancer and Laboratory Medicine, BC Cancer, Vancouver, Canada
- Department of Pathology and Laboratory Medicine, BC Cancer, Vancouver, Canada
- Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, Canada
| | - Graham W Slack
- Centre for Lymphoid Cancer and Laboratory Medicine, BC Cancer, Vancouver, Canada
- Department of Pathology and Laboratory Medicine, BC Cancer, Vancouver, Canada
- Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, Canada
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13
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Mahajan VK, Sharma V, Sharma N, Rani R. Kikuchi-Fujimoto disease: A comprehensive review. World J Clin Cases 2023; 11:3664-3679. [PMID: 37383134 PMCID: PMC10294163 DOI: 10.12998/wjcc.v11.i16.3664] [Citation(s) in RCA: 17] [Impact Index Per Article: 8.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/27/2022] [Revised: 03/29/2023] [Accepted: 04/27/2023] [Indexed: 06/02/2023] Open
Abstract
Kikuchi-Fujimoto disease, a rare form of necrotizing lymphadenitis, is an uncommon, benign, self-limiting disorder of obscure etiology. It affects mostly young adults of both genders. Clinically, it presents with fever and lymphadenopathy of a firm to rubbery consistency frequently involving cervical lymph nodes while weight loss, splenomegaly, leucopenia, and elevated erythrocyte sedimentation rate feature in severely affected patients. Cutaneous involvement occurs in about 30%-40% of cases as facial erythema and nonspecific erythematous papules, plaques, acneiform or morbilliform lesions of great histologic heterogeneity. Both Kikuchi-Fujimoto disease and systemic lupus erythematosus share an obscure and complex relationship as systemic lupus erythematosus may occasionally precede, develop subsequently, or sometimes be associated concurrently with Kikuchi-Fujimoto disease. It is often mistaken for non-Hodgkin lymphoma while lupus lymphadenitis, cat-scratch disease, Sweet's syndrome, Still's disease, drug eruptions, infectious mononucleosis, and viral or tubercular lymphadenitis are other common differentials. Fine needle aspiration cytology mostly has features of nonspecific reactive lymphadenitis and immunohistochemistry studies usually show variable features of uncertain diagnostic value. Since its diagnosis is exclusively from histopathology, it needs to be evaluated more carefully; an early lymph node biopsy will obviate the need for unnecessary investigations and therapeutic trials. Its treatment with systemic corticosteroids, hydroxychloroquine, or antimicrobial agents mostly remains empirical. The article reviews clinicoepidemiological, diagnostic, and management aspects of KFD from the perspective of practicing clinicians.
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Affiliation(s)
- Vikram K Mahajan
- Department of Dermatology, Venereology and Leprosy, Dr. Radhakrishnan Government Medical College, Hamirpur 177001, Himachal Pradesh, India
| | - Vikas Sharma
- Department of Dermatology, Venereology and Leprosy, Dr. Radhakrishnan Government Medical College, Hamirpur 177001, Himachal Pradesh, India
| | - Neeraj Sharma
- Department of Dermatology, Venereology and Leprosy, Dr. Radhakrishnan Government Medical College, Hamirpur 177001, Himachal Pradesh, India
| | - Ritu Rani
- Department of Dermatology, Venereology and Leprosy, Dr. Radhakrishnan Government Medical College, Hamirpur 177001, Himachal Pradesh, India
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14
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Alshekaili J, Nasr I, Al-Rawahi M, Ansari Z, Al Rahbi N, Al Balushi H, Al Zadjali S, Al Kindi M, Al-Maawali A, Cook MC. A homozygous loss-of-function C1S mutation is associated with Kikuchi-Fujimoto disease. Clin Immunol 2023; 252:109646. [PMID: 37209807 DOI: 10.1016/j.clim.2023.109646] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/02/2023] [Revised: 05/03/2023] [Accepted: 05/10/2023] [Indexed: 05/22/2023]
Abstract
BACKGROUND Kikuchi-Fujimoto disease (KFD) is a self-limited inflammatory disease of unknown pathogenesis. Familial cases have been described and defects in classical complement components C1q and C4 have been identified in some patients. MATERIAL AND METHODS We describe genetic and immune investigations of a 16 years old Omani male, a product of consanguineous marriage, who presented with typical clinical and histological features of KFD. RESULTS We identified a novel homozygous single base deletion in C1S (c.330del; p. Phe110LeufsTer23) resulting in a defect in the classical complement pathway. The patient was negative for all serological markers of SLE. In contrast, two female siblings (also homozygous for the C1S mutation), one has autoimmune thyroid disease (Hashimoto thyroiditis) and a positive ANA and the other sibling has serology consistent with SLE. CONCLUSION We report the first association between C1s deficiency and KFD.
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Affiliation(s)
- Jalila Alshekaili
- Department of Microbiology and Immunology, Sultan Qaboos University Hospital, Sultan Qaboos University, Muscat, Oman.
| | - Iman Nasr
- Department of Adult Allergy and Clinical Immunology, The Royal Hospital, Muscat, Oman
| | - Mohammed Al-Rawahi
- Department of Hematology, Sultan Qaboos University Hospital, Sultan Qaboos University, Muscat, Oman
| | - Zainab Ansari
- Department of Adult Allergy and Clinical Immunology, The Royal Hospital, Muscat, Oman
| | | | - Hamed Al Balushi
- Department of Microbiology and Immunology, Sultan Qaboos University Hospital, Sultan Qaboos University, Muscat, Oman
| | - Shoaib Al Zadjali
- Department of Hematology, Sultan Qaboos University Hospital, Sultan Qaboos University, Muscat, Oman
| | - Mahmood Al Kindi
- Department of Microbiology and Immunology, Sultan Qaboos University Hospital, Sultan Qaboos University, Muscat, Oman
| | - Almundher Al-Maawali
- Genetics Department, Sultan QaboosUniversity Hospital, Sultan Qaboos University, Muscat, Oman
| | - Matthew C Cook
- Department of Immunology and Infectious Disease, John Curtin School of Medical Research, Australian National University, Canberra, NSW, Australia; Department of Medicine, University of Cambridge, United Kingdom; Centre for Personalised Immunology, John Curtin School of Medical Research, Australian National University, Canberra, NSW, Australia.
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15
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Muacevic A, Adler JR. Kikuchi's Disease Diagnosed by an Excisional Biopsy in a Patient With COVID-19. Cureus 2023; 15:e35251. [PMID: 36825075 PMCID: PMC9942247 DOI: 10.7759/cureus.35251] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 02/21/2023] [Indexed: 02/23/2023] Open
Abstract
COVID-19 is an ongoing pandemic caused by the novel coronavirus SARS-CoV-2. The clinical features of COVID-19 are myriad. Though it is a multisystem illness, it predominantly involves the respiratory system. There have been case reports on rare manifestations of COVID-19, of which COVID-19-related Kikuchi's disease is one of them. To our knowledge, this is the third reported case in the world. We report a lady in her late 60s with COVID-19 infection and secondary bacterial pneumonia, which necessitated ICU admission, having ongoing fever spikes with high inflammatory markers and leukopenia. She was also found to have tender cervical lymphadenopathy on the third week of illness, whose biopsy revealed histiocytic necrotizing lymphadenitis in keeping with Kikuchi's disease. The patient had an uneventful recovery in two weeks without any intervention. The pathophysiology of COVID-19-related Kikuchi's disease is unclear. However, COVID-19 is a viral illness that involves changes in interleukins. The latter is postulated in Kikuchi's disease.
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16
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Wang N, Zhu Y, Zhang J, Pan S, Wang H. Clinical scoring approach for detection of histiocytic necrotising lymphadenitis in adults. Intern Med J 2023; 53:84-88. [PMID: 32896970 DOI: 10.1111/imj.15042] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/12/2020] [Revised: 08/25/2020] [Accepted: 08/25/2020] [Indexed: 01/27/2023]
Abstract
BACKGROUND Histiocytic necrotising lymphadenitis (HNL) is rare and can be easily ignored. AIMS To summarise the characteristics of HNL and find a simple scoring approach to detect HNL in adult patients. METHODS Adult patients with lymphadenopathy diagnosed by lymph node biopsy were enrolled. Chi-squared test and t-test were used to determine the significant variables. The cut-off values and scores assigned to each factor were performed by receiver operating characteristic (ROC) curves and coefficients in the logistic regression respectively. The performance of the scoring system was evaluated by ROC curves. RESULTS There were 32 HNL cases and 1162 other cases in the present study. These features, including age, the frequency of presentations of fever, cervical and painful lymph nodes, decrease of white blood cells (WBC), ratio of neutrophil to WBC (N ratio) and elevated lactate dehydrogenase (LDH), were different between patients with HNL and other diseases. Based on the multivariate analysis, the scoring approach was defined as follows: score = 3 (fever) + 2 (cervical lymphadenopathy) + 2 (decreased WBC) + 1 (decreased N ratio) + 2 (elevated LDH). The cut-off was score 4. This approach performed will detect HNL with an area under the curve of 0.889. CONCLUSION The present study suggests that the novel scoring approach we put forward might be useful to detect HNL in adult patients though further studies are needed.
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Affiliation(s)
- Ning Wang
- Department of Anesthesiology, The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China.,Department of Emergency, Xinhua Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China
| | - Yingying Zhu
- Department of Emergency, Xinhua Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China
| | - Jiaojiao Zhang
- Department of Emergency, Xinhua Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China
| | - Shuming Pan
- Department of Emergency, Xinhua Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China
| | - Hairong Wang
- Department of Emergency, Xinhua Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China
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17
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Rezigh A, Garza-Garcia GA, Minter D, Shekarchian S. Mirror Mirror: An Exercise in Clinical Reasoning. J Gen Intern Med 2023; 38:228-232. [PMID: 36266378 PMCID: PMC9849643 DOI: 10.1007/s11606-022-07820-x] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/30/2022] [Accepted: 09/15/2022] [Indexed: 01/22/2023]
Affiliation(s)
- Austin Rezigh
- Department of Internal Medicine, University of Texas Southwestern, Dallas, TX, USA.
| | | | - Daniel Minter
- Department of Medicine, University of California San Francisco, San Francisco, CA, USA
| | - Sharmin Shekarchian
- Department of Medicine, Stanford University, Palo Alto Veterans Affairs Medical Center, Palo Alto, CA, USA
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18
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GRANİT SEMAVİ D, ULAŞ T. Highlighting steroid indications for Kikuchi Fujimoto disease: a case report. CUKUROVA MEDICAL JOURNAL 2022. [DOI: 10.17826/cumj.1176368] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/02/2023] Open
Abstract
Kikuchi Fujimoto disease (KFD) is a rare, generally benign and self limited condition with an unknown etiology. There isn’t a guideline for treatment of KFD, recommendations for treatment are based on clinical experience and management is typically supportive. However, sometimes more ominous course of the disease may be experienced. Thus, for the patients with severe disease, refractory symptoms and recurrences, more definite and efficient treatment options are required. Here, we aimed to emphasize the indications of steroid treatment via a case of KFD resolved after systemic steroids.
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19
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Chisholm KM, Bohling SD, Tsuchiya KD, Paulson VA. A Malignant Mimicker: Features of Kikuchi-Fujimoto Disease in the Pediatric Population. Pediatr Dev Pathol 2022; 25:538-547. [PMID: 35583198 DOI: 10.1177/10935266221103882] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Abstract
BACKGROUND Kikuchi-Fujimoto disease (KFD) is a rare, benign, and self-limited disease that presents with cervical lymphadenopathy and systemic symptoms. Histologic evaluation is often necessary to differentiate KFD from other entities. METHODS Electronic medical records and diagnostic material were reviewed for 14 children diagnosed with KFD and 6 children diagnosed with infectious mononucleosis (IM) from 2013-2021. Four cases of KFD were further characterized using targeted DNA-based next-generation sequencing. RESULTS Systemic symptoms were present in 86% (n = 12/14) of KFD patients, the most common being fever. Laboratory values worrisome for malignancy included cytopenia(s) (n = 9/12), elevated ESR and/or CRP (n = 9/12), elevated ferritin (n = 7/7), and elevated LDH (n = 7/10). Histologically, lymph nodes showed characteristic necrotic foci without neutrophils surrounded by MPO+ "crescentic" histiocytes. Immunoblasts and CD123+ plasmacytoid dendritic cells (pDCs) were also increased surrounding the necrosis. IM lymph nodes showed similar features when necrosis was present but increases in pDCs were patchy and rare neutrophils were seen in the necrotic foci. Molecular analysis of 4 KFD cases did not identify pathogenic variants. CONCLUSION While the signs/symptoms of KFD are worrisome, there are pathologic features that help differentiate it from potential mimics. We did not identify characteristic molecular features to aid in the work-up of these cases.
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Affiliation(s)
- Karen M Chisholm
- Department of Laboratories, 7274Seattle Children's Hospital, Seattle, WA, USA.,Department of Laboratory Medicine and Pathology, 7284University of Washington, Seattle, WA, USA
| | - Sandra D Bohling
- Department of Laboratories, 7274Seattle Children's Hospital, Seattle, WA, USA.,Department of Laboratory Medicine and Pathology, 7284University of Washington, Seattle, WA, USA
| | - Karen D Tsuchiya
- Department of Laboratory Medicine and Pathology, 7284University of Washington, Seattle, WA, USA
| | - Vera A Paulson
- Department of Laboratory Medicine and Pathology, 7284University of Washington, Seattle, WA, USA
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20
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Jagadeesan S, Rani S, Porwal YC, Patel P. Lupus and Kikuchi-Fujimoto Disease: A Combination for Catastrophe. Cureus 2022; 14:e27423. [PMID: 36051719 PMCID: PMC9420304 DOI: 10.7759/cureus.27423] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 07/28/2022] [Indexed: 11/20/2022] Open
Abstract
Kikuchi-Fujimoto disease (KFD) is a self-limiting disease of idiopathic origin affecting young women characterized by unexplained fever and lymphadenopathy. It has been usually found to be associated with autoimmunity, of which systemic lupus erythematosus (SLE) is the most outstanding. Fever and lymphadenopathy carry a broad differential, and a missed diagnosis of a rare condition such as Kikuchi can lead to inappropriate treatment in an otherwise benign condition. Therefore, careful examination and histologic confirmation of the diagnosis are critical.
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21
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Achappa B, Herath NC, Sebastian B, Dsouza NV, RAGHURAM PAVANMANIBETTU, Holla R, Chowta N, Kini JR. Kikuchi-Fujimoto disease in a tertiary care teaching hospital in Coastal South India: A 8-year retrospective study. F1000Res 2022; 11:492. [PMID: 35811790 PMCID: PMC9226704 DOI: 10.12688/f1000research.109832.1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 03/11/2022] [Indexed: 11/20/2022] Open
Abstract
Background: Kikuchi-Fujimoto disease (KFD) is a rare, benign condition of unknown etiology, presenting as cervical lymphadenitis. Lymphadenopathy is usually tender and maybe associated with systemic symptoms. Despite the extensive literature on this disease, it continues to be misdiagnosed owing to its misleading clinical presentation.
Methods:
A retrospective hospital-based descriptive cross-sectional study was conducted in tertiary care hospitals from 2011 to 2019. All patients with confirmed KFD diagnosis were included and after ethics committee approval the clinical details and histopathological data was retrieved from the medical records department and analyzed.
Results:
A total of 67 cases were included. The mean age was 26.9±11.3 years with a female: male ratio of 1.9:1. There were 50 patients with tender cervical lymphadenopathy which was the most common clinical presentation. The mean length and width of palpable lymph nodes were 2.3±1.0 cm and 2.2±0.7 cm respectively. Histology revealed proliferative stage in majority of patients (
n=40, 59.7%). Lymphadenopathy resolved in 83.6% within 2 months. There were 42 patients who had complete recovery with symptomatic treatment within a period of 9 months.
Conclusions:
KFD is prevalent in young, female patients of Asian descent and often presents as tender cervical lymphadenopathy. Early diagnosis with excisional lymph node biopsy is crucial to avoid unnecessary investigations and treatment. Treatment is symptomatic unless complicated, where steroid therapy is considered. KFD has an excellent prognosis with almost no risk of fatality.
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Affiliation(s)
- Basavaprabhu Achappa
- Department of Internal Medicine, Kasturba Medical College, Mangalore, Manipal Academy of Higher Education, Manipal, India
| | - Nipuni Chamathka Herath
- Department of Internal Medicine, Kasturba Medical College, Mangalore, Manipal Academy of Higher Education, Manipal, India
| | - Bodhi Sebastian
- Department of Internal Medicine, Kasturba Medical College, Mangalore, Manipal Academy of Higher Education, Manipal, India
| | - Nikhil Victor Dsouza
- Department of Internal Medicine, Kasturba Medical College, Mangalore, Manipal Academy of Higher Education, Manipal, India
| | - PAVAN MANIBETTU RAGHURAM
- Department of Internal Medicine, Kasturba Medical College, Mangalore, Manipal Academy of Higher Education, Manipal, India
| | - Ramesh Holla
- Department of Community Medicine, Kasturba Medical College, Mangalore, Manipal Academy of Higher Education, Manipal, India
| | - Nithyananda Chowta
- Department of Internal Medicine, Kasturba Medical College, Mangalore, Manipal Academy of Higher Education, Manipal, India
| | - Jyoti Ramanath Kini
- Department of Pathology, Kasturba Medical College, Mangalore, Manipal Academy of Higher Education, Manipal, India
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22
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Al Ghadeer HA, AlKadhem SM, AlMajed MS, AlAmer HM, AlHabeeb JA, Alomran SH, AlMajed AS. Kikuchi-Fujimoto Disease Following COVID-19. Cureus 2022; 14:e21049. [PMID: 35155016 PMCID: PMC8824308 DOI: 10.7759/cureus.21049] [Citation(s) in RCA: 9] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 01/07/2022] [Indexed: 11/05/2022] Open
Abstract
Kikuchi-Fujimoto Disease (KFD) is a rare self-limiting condition of unknown etiology. It is characterized by fever, and lymphadenopathy most commonly involving posterior cervical lymph nodes. Although it is of uncertain etiology, it is associated with viral infections and autoimmune diseases. Distinction from lymphadenopathy-associated alternate disorders is crucial to avoid unneeded diagnostic procedures and treatment. KFD is diagnosed based on histopathologic examination of the excised lymph node. The management is supportive with favorable outcomes within a few weeks or months. In this case, we describe a 13-year-old boy who complained of painful cervical lymphadenopathy and fever for more than three weeks following COVID-19. Diagnostic workup has been established and KFD diagnosis made based on the histopathologic features of the involved lymph node. The patient showed complete recovery with no recurrence during follow-up. So, this case highlights the possible association between COVID-19 and KFD during this pandemic and keeping it in the differential diagnosis.
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23
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Khan AM, Ahmad M, Muhammad O, Taj S, Shiza ST. Kikuchi-Fujimoto Disease in a Young Female: A Case Report and Literature Review. Cureus 2021; 13:e19321. [PMID: 34900492 PMCID: PMC8649112 DOI: 10.7759/cureus.19321] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 11/06/2021] [Indexed: 11/25/2022] Open
Abstract
Kikuchi-Fujimoto disease (KFD) is histiocytic necrotizing lymphadenitis, a rare immune-mediated disorder presenting with lymphadenopathy, leukopenia, and occasionally fever. Herein we report a case of KFD who presented with anorexia, fever, and cervical lymphadenopathy. Lymph node biopsy and immunohistochemistry confirmed the diagnosis of KFD. She was treated with prednisolone and paracetamol, and her condition improved gradually on subsequent follow-up. A patient presenting with fever and lymphadenopathy leads to prompt investigations for common diseases such as tuberculosis and lymphoma. However, rare diseases like KFD must be kept in mind, and a lymph node biopsy followed by histopathologic examination and immunohistochemistry should be done to confirm the diagnosis.
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Affiliation(s)
- Aadil M Khan
- Internal Medicine, Ganesh Shankar Vidyarthi Memorial Medical College, Kanpur, IND
| | - Moinuddin Ahmad
- Internal Medicine, Ganesh Shankar Vidyarthi Memorial Medical College, Kanpur, IND
| | - Owaise Muhammad
- General Medicine, Lugansk State Medical University, Kyiv, UKR
| | - Shafaq Taj
- Internal Medicine, Deccan College of Medical Sciences, Hyderabad, IND
| | - Saher T Shiza
- Internal Medicine, Deccan College of Medical Sciences, Hyderabad, IND
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24
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Hoan L, Minh Hang L, Tuan Linh L, Thi Tra My T, Ngoc Minh T, Duc Thinh D, Ngoc Cuong N. A Rare Case of Kikuchi-Fujimoto Disease in a Young Female Patient. AMERICAN JOURNAL OF CASE REPORTS 2021; 22:e933377. [PMID: 34802030 PMCID: PMC8614063 DOI: 10.12659/ajcr.933377] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Abstract
Patient: Female, 25-year-old
Final Diagnosis: Kikuchi-Fujimoto disease
Symptoms: Neck pain • palpable mass neck
Medication: —
Clinical Procedure: —
Specialty: Pathology • Pulmonology • Radiology
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Affiliation(s)
- Le Hoan
- Department of Respiratory Medicine, Hanoi Medical University Hospital, Hanoi, Vietnam
| | - Le Minh Hang
- Department of Respiratory Medicine, Hanoi Medical University Hospital, Hanoi, Vietnam
| | - Le Tuan Linh
- Diagnostic Imaging and Interventional Radiology Center, Hanoi Medical University Hospital, Hanoi, Vietnam
| | - Thieu Thi Tra My
- Diagnostic Imaging and Interventional Radiology Center, Hanoi Medical University Hospital, Hanoi, Vietnam
| | - Tran Ngoc Minh
- Department of Pathology, Hanoi Medical University Hospital, Hanoi, Vietnam
| | - Dang Duc Thinh
- Department of Pathology, Hanoi Medical University Hospital, Hanoi, Vietnam
| | - Nguyen Ngoc Cuong
- Diagnostic Imaging and Interventional Radiology Center, Hanoi Medical University Hospital, Hanoi, Vietnam
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25
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Sato T, Kanda K, Kawamura Y, Takeuchi M. Sub internal limiting membrane hemorrhage followed by bilateral optic disc hemorrhage in Kikuchi-Fujimoto disease: a case report. BMC Ophthalmol 2021; 21:355. [PMID: 34620137 PMCID: PMC8499551 DOI: 10.1186/s12886-021-02106-y] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/22/2021] [Accepted: 09/14/2021] [Indexed: 11/10/2022] Open
Abstract
Background Kikuchi-Fujimoto disease (KFD) is a necrotizing lymphadenitis, and presents fever of unknown origin and cervical lymphadenopathy. Ocular complications are unusual in KFD. Here we report a case of sub internal limiting membrane (ILM) hemorrhage followed by bilateral optic disc hemorrhage in KFD. Case presentation A 16-year-old Japanese man perceived a sudden decrease of right vision 3 days after onset of fever with unknown origin and left cervical lymphadenopathy. At presentation, visual acuity (VA) of right eye was 0.05 in decimal chart (1.30: converted to logarithm of minimum angle of resolution: logMAR). Fundus photograph showed extensive sub-ILM hemorrhage in right eye, and optic disc hemorrhages in both eyes. Fluorescein angiography presented hypo- and hyperfluorescences in optic disc of right eye, and hyperfluorescence in the disc of left eye. To make a definitive diagnosis, cervical lymph node biopsy was performed, and KFD was diagnosed pathologically. Thereafter, fever, headache and the cervical lymphadenopathy disappeared spontaneously. The sub-ILM hemorrhage was drained into the vitreous cavity by neodymium:yttrium-aluminum-garnet laser (Nd: YAG) hyaloidotomy. VA recovered to 1.5 (− 0.18: logMAR VA) in right eye. Conclusion Sub-ILM hemorrhage and optic disc hemorrhage are a KFD-related ocular complication. Supplementary Information The online version contains supplementary material available at 10.1186/s12886-021-02106-y.
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Affiliation(s)
- Tomohito Sato
- Department of Ophthalmology, National Defense Medical College, 3-2 Namiki, Tokorozawa, Saitama, 359-8513, Japan
| | - Koji Kanda
- Department of Ophthalmology, National Defense Medical College, 3-2 Namiki, Tokorozawa, Saitama, 359-8513, Japan
| | - Yusuke Kawamura
- Department of General Medicine, National Defense Medical College, Saitama, Japan
| | - Masaru Takeuchi
- Department of Ophthalmology, National Defense Medical College, 3-2 Namiki, Tokorozawa, Saitama, 359-8513, Japan.
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26
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Park S, Kim JY, Ryu YJ, Lee H. Kikuchi Cervical Lymphadenitis in Children: Ultrasound Differentiation From Common Infectious Lymphadenitis. JOURNAL OF ULTRASOUND IN MEDICINE : OFFICIAL JOURNAL OF THE AMERICAN INSTITUTE OF ULTRASOUND IN MEDICINE 2021; 40:2069-2078. [PMID: 33263358 DOI: 10.1002/jum.15584] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 04/21/2020] [Revised: 10/20/2020] [Accepted: 10/26/2020] [Indexed: 06/12/2023]
Abstract
OBJECTIVES To investigate ultrasound (US) features of enlarged cervical lymph nodes (LNs) to differentiate between Kikuchi disease (KD) and other common types of infectious lymphadenitis in an East Asian pediatric patient population. METHODS A total of 142 pediatric patients with KD and 45 patients with infectious lymphadenitis (suppurative lymphadenitis [n = 29], nontuberculous mycobacterial lymphadenitis [n = 9], and tuberculous lymphadenitis [n = 7]) were included. The clinical characteristics, laboratory results, and US features of LNs were reviewed. The area under the curve (AUC) from a receiver operating characteristic curve analysis was used as a diagnostic accuracy measure. RESULTS A multiple clustered adjacent pattern, bilaterality, an even size, posterior neck involvement, no enlargement, an elongated-to-ovoid shape, homogeneous hypoechogenicity, a well-defined margin, presence of an echogenic fatty hilum, no intranodal gross necrosis, increased perinodal fat echogenicity, and no increased echogenicity of the adjacent sternocleidomastoid muscle were significant US features of the affected LNs to discriminate KD from infectious lymphadenitis (P < .05). Homogeneous hypoechogenicity in KD showed the highest AUC (0.930) as a single variable (95% confidence interval, 0.88-0.96). The AUCs were increased in 3 combination models with 2 US features: homogeneous echogenicity and 1 of 3 other US features (increased perinodal fat echogenicity, 0.935; number of affected LNs, 0.947; and LN shape, 0.949). CONCLUSIONS Homogeneous hypoechogenicity of LNs was a significant US feature with the highest diagnostic accuracy in differentiating KD from common infectious lymphadenitis on a univariate analysis. In the combination model, US features of an elongated-to-ovoid shape and homogeneous hypoechogenicity showed the highest diagnostic accuracy.
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Affiliation(s)
- Sunyoung Park
- Department of Radiology, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, South Korea
| | - Ji Young Kim
- Department of Radiology, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, South Korea
| | - Young Jin Ryu
- Department of Radiology, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, South Korea
| | - Hyunju Lee
- Department of Pediatrics, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, South Korea
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Ahmed Z, Quadir H, Hakobyan K, Gaddam M, Kannan A, Ojinnaka U, Mostafa JA. Kikuchi-Fujimoto Disease: A Rare Cause of Cervical Lymphadenopathy. Cureus 2021; 13:e17021. [PMID: 34522502 PMCID: PMC8425500 DOI: 10.7759/cureus.17021] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/25/2021] [Accepted: 08/09/2021] [Indexed: 12/24/2022] Open
Abstract
Kikuchi-Fujimoto disease (KFD) is a rare benign disease, clinically characterized by fever and tender cervical lymphadenopathy affecting the posterior cervical lymph nodes. This disease is usually accompanied by night sweats, rashes, and headaches. It generally affects young individuals, especially females, of Oriental-Asian origin. The etiology of KFD remains uncertain, but associations have been noted with viral diseases including Epstein-Barr virus (EBV), herpes simplex virus (HSV), and varicella-zoster virus (VZV), as well as autoimmune disorders including systemic lupus erythematosus (SLE) and Sjogren's syndrome. This review points out the etiology of KFD with cervical lymphadenopathy alongside its clinical presentation, histological highlights, lab investigations, complications, and treatment. Accurate diagnosis of this disease depends on lymph node excisional biopsy. Three histological patterns of KFD are recognized: proliferative, necrotizing, and xanthomatous. Distinction from lymphadenopathy-associated alternate disorders (e.g., SLE, malignancy, tuberculosis, or another infectious lymphadenitis) is essential to ensure appropriate therapy. This self-limited condition entails nonsteroidal anti-inflammatory drugs (NSAIDs) for pain relief with consideration of corticosteroids and hydroxychloroquine in severe cases.
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Affiliation(s)
- Zubayer Ahmed
- Internal Medicine, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA
| | - Huma Quadir
- Internal Medicine/Family Medicine, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA
| | - Knkush Hakobyan
- Diagnostic Radiology, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA
| | - Mrunanjali Gaddam
- Internal Medicine, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA
| | - Amudhan Kannan
- General Surgery, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA
| | - Ugochi Ojinnaka
- Family Medicine, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA
| | - Jihan A Mostafa
- Psychiatry, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA
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28
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Nelson ND, Meng W, Rosenfeld AM, Bullman S, Sekhar Pedamallu C, Nomburg JL, Wertheim GB, Paessler ME, Pinkus G, Hornick JL, Meyerson M, Luning Prak ET, Pillai V. Characterization of Plasmacytoid Dendritic Cells, Microbial Sequences, and Identification of a Candidate Public T-Cell Clone in Kikuchi-Fujimoto Disease. Pediatr Dev Pathol 2021; 24:193-205. [PMID: 33530869 DOI: 10.1177/1093526620987961] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/02/2023]
Abstract
OBJECTIVES Kikuchi-Fujimoto disease (KFD) is a self-limited lymphadenitis of unclear etiology. We aimed to further characterize this disease in pediatric patients, including evaluation of the CD123 immunohistochemical (IHC) staining and investigation of potential immunologic and infectious causes. METHODS Seventeen KFD cases and 12 controls were retrospectively identified, and the histologic and clinical features were evaluated. CD123 IHC staining was quantified by digital image analysis. Next generation sequencing was employed for comparative microbial analysis via RNAseq (5 KFD cases) and to evaluate the immune repertoire (9 KFD cases). RESULTS In cases of lymphadenitis with necrosis, >0.85% CD123+ cells by IHC was found to be six times more likely in cases with a final diagnosis of KFD (sensitivity 75%, specificity 87.5%). RNAseq based comparative microbial analysis did not detect novel or known pathogen sequences in KFD. A shared complementarity determining region 3 (CDR3) sequence and use of the same T-cell receptor beta variable region family was identified in KFD LNs but not controls, and was not identified in available databases. CONCLUSIONS Digital quantification of CD123 IHC can distinguish KFD from other necrotizing lymphadenitides. The presence of a unique shared CDR3 sequence suggests that a shared antigen underlies KFD pathogenesis.
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Affiliation(s)
- Nya D Nelson
- Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.,Department of Pathology and Laboratory Medicine, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania
| | - Wenzhao Meng
- Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, Pennsylvania
| | - Aaron M Rosenfeld
- Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, Pennsylvania
| | - Susan Bullman
- Broad Institute of Massachusetts Institute of Technology and Harvard, Cambridge, Massachusetts.,Department of Medical Oncology and Center for Cancer Genome Discovery, Dana-Farber Cancer Institute, Boston, Massachusetts.,Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts
| | - Chandra Sekhar Pedamallu
- Broad Institute of Massachusetts Institute of Technology and Harvard, Cambridge, Massachusetts.,Department of Medical Oncology and Center for Cancer Genome Discovery, Dana-Farber Cancer Institute, Boston, Massachusetts.,Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts
| | - Jason L Nomburg
- Broad Institute of Massachusetts Institute of Technology and Harvard, Cambridge, Massachusetts.,Department of Medical Oncology and Center for Cancer Genome Discovery, Dana-Farber Cancer Institute, Boston, Massachusetts.,Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts
| | - Gerald B Wertheim
- Department of Pathology and Laboratory Medicine, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania
| | - Michele E Paessler
- Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.,Department of Pathology and Laboratory Medicine, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania
| | - Geraldine Pinkus
- Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts
| | - Jason L Hornick
- Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts
| | - Matthew Meyerson
- Broad Institute of Massachusetts Institute of Technology and Harvard, Cambridge, Massachusetts.,Department of Medical Oncology and Center for Cancer Genome Discovery, Dana-Farber Cancer Institute, Boston, Massachusetts.,Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts
| | - Eline T Luning Prak
- Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, Pennsylvania
| | - Vinodh Pillai
- Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.,Department of Pathology and Laboratory Medicine, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania
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29
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Akhavanrezayat A, Cooper JD, Hassan M, Pham BH, Nguyen QD, Farr AK. Bilateral preretinal hemorrhage associated with Kikuchi-Fujimoto disease. Am J Ophthalmol Case Rep 2021; 22:101041. [PMID: 33732946 PMCID: PMC7940994 DOI: 10.1016/j.ajoc.2021.101041] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/04/2021] [Revised: 02/03/2021] [Accepted: 02/20/2021] [Indexed: 11/19/2022] Open
Abstract
Purpose To present a case of a patient with human immunodeficiency virus (HIV) disease and Kikuchi-Fujimoto disease (KFD) who presented with a unique pattern of retinopathy. Observations A 7-year-old Taiwanese girl with HIV disease who was recently diagnosed with KFD had a sudden onset of blurry vision in both eyes one month after her KFD systemic symptoms had relatively resolved. Ophthalmic examination showed decreased visual acuity in both eyes (OU). On fundus examination, she had bilateral preretinal, subhyaloid, and vitreous hemorrhage that was more severe than anemic retinopathy. Conclusion Ocular manifestations in Kikuchi-Fujimoto disease are rare; however, if they occur, presentations may vary. The exact etiology of the disease has remained elusive and controversial. This case is the first report of a patient with HIV disease and KFD presenting with ocular involvement. Furthermore, bilateral preretinal, subhyaloid, and vitreous hemorrhage, which was beyond anemic retinopathy, is an unprecedented manifestation of KFD that has not been previously reported. This case highlights the necessity for clinicians to consider all possible differential diagnoses when evaluating patients with similar findings to identify the best therapeutic approach and avoid unnecessary treatment.
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Affiliation(s)
- Amir Akhavanrezayat
- Byers Eye Institute, Stanford University School of Medicine, Palo Alto, CA, USA
| | - Joseph D. Cooper
- Division of AIDS Medicine & Infectious Diseases, Department of Medicine, Santa Clara Valley Health & Hospital System, San Jose, CA, USA
| | - Muhammad Hassan
- Byers Eye Institute, Stanford University School of Medicine, Palo Alto, CA, USA
| | - Brandon H. Pham
- Byers Eye Institute, Stanford University School of Medicine, Palo Alto, CA, USA
| | - Quan Dong Nguyen
- Byers Eye Institute, Stanford University School of Medicine, Palo Alto, CA, USA
| | - Arman K. Farr
- Retina Institute of The Carolina and Macular Degeneration Center, Charlotte, NC, USA
- Corresponding author.
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30
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Garcia-Cirera S, Calvet J, Martin-Marimon JC, García-Chamón RB, Gratacós J. PET-CT SCAN TO DIAGNOSE AND DEMONSTRATE RESOLUTION OF LUPUS LYMPHADENITIS WITH BELIMUMAB. Rheumatology (Oxford) 2021; 60:e324-e325. [PMID: 33693531 DOI: 10.1093/rheumatology/keab226] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/15/2021] [Accepted: 02/23/2021] [Indexed: 11/12/2022] Open
Affiliation(s)
- S Garcia-Cirera
- Rheumatology Department, Parc Taulí University Hospital, I3PT Research Institute (UAB), Sabadell, Spain
| | - J Calvet
- Rheumatology Department, Parc Taulí University Hospital, I3PT Research Institute (UAB), Sabadell, Spain
| | | | - R B García-Chamón
- Anatomic Pathology Department, Parc Taulí University Hospital, I3PT Research Institute (UAB), Sabadell, Spain
| | - J Gratacós
- Rheumatology Department, Parc Taulí University Hospital, I3PT Research Institute (UAB), Sabadell, Spain
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31
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Hemophagocytic Lymphohistiocytosis Associated with Histiocytic Necrotizing Lymphadenitis: A Clinical Study of 13 Children and Literature Review. J Pediatr 2021; 229:267-274.e3. [PMID: 32956695 DOI: 10.1016/j.jpeds.2020.08.063] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/26/2020] [Revised: 07/14/2020] [Accepted: 08/21/2020] [Indexed: 01/06/2023]
Abstract
OBJECTIVE To analyze the clinical characteristics and prognosis of pediatric hemophagocytic lymphohistiocytosis (HLH) associated with histiocytic necrotizing lymphadenitis (HNL). STUDY DESIGN We retrospectively collected the clinical data of all children with HNL-HLH enrolled in Beijing Children's Hospital from 2007 to 2019. The control patients with Epstein-Barr virus-associated HLH and simple HNL (not associated with HLH) were case matched (1:2). The clinical features and prognosis were analyzed by case-control study. Cases of HNL-HLH in the literature were reviewed. RESULTS The male-to-female ratio of the 13 patients in our center was 9:4. The mean age of the patients at disease onset was 8.1 ± 1.2 years, younger than that of the 16 patients in the literature (P = .017). Clinical presentations, especially rash and splenomegaly, and laboratory examination of HNL-HLH group were statistically different from Epstein-Barr virus-HLH group, simple HNL group, and patients reported in the literature (P < .05). Three patients were treated with immunosuppressive drugs or chemotherapy owing to poor control of HLH. One patient died, and all 12 remaining patients survived, 2 of which developed autoimmune diseases. Kaplan-Meier survival curves showed no statistical difference among the 3 groups (P > .05). CONCLUSIONS HNL-HLH is more common in school- and preschool-age children. Most patients have a favorable prognosis. Some patients suffer from relapses or develop autoimmune diseases. Prolonged follow-up should be carried out for patients with HNL-HLH.
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32
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Jiwani RA, Jourdan DN, Pona A, Donthi D, Stalls JS, Rehana RW. Kikuchi Fujimoto disease: sinister presentation, good prognosis. J Community Hosp Intern Med Perspect 2021; 11:72-75. [PMID: 33552420 PMCID: PMC7850461 DOI: 10.1080/20009666.2020.1824332] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/24/2022] Open
Abstract
Kikuchi-Fujimoto disease (KFD) is a rare, benign, self-limiting necrotizing lymphadenitis of unknown etiology. The disease can affect people of all ages and of any sex and ethnicity. Tissue biopsy is needed for accurate diagnosis. The condition commonly masquerades as more sinister conditions such as malignancy and rheumatologic disorders, but has a much better prognosis. Treatment is generally supportive but patients may require corticosteroids with eventual spontaneous resolution. We discuss a case of KFD in a 34-year-old male and highlight the need for prompt and accurate diagnosis.
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Affiliation(s)
- Rahim A Jiwani
- Department of Internal Medicine, East Carolina University/Brody School of Medicine, Greenville, NC, USA
| | - Daniel N Jourdan
- Brody School of Medicine at East Carolina University, Greenville, NC, USA
| | - Adrian Pona
- Department of Internal Medicine, East Carolina University/Brody School of Medicine, Greenville, NC, USA
| | - Deepak Donthi
- Department of Pathology and Laboratory Medicine, East Carolina University/Brody School of Medicine, Greenville, NC, USA
| | - J Stephen Stalls
- Department of Pathology and Laboratory Medicine, East Carolina University/Brody School of Medicine, Greenville, NC, USA
| | - Rita W Rehana
- Department of Internal Medicine, Henry Ford Macomb Hospital, Clinton Township, USA
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33
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Pan YT, Cao LM, Xu Y, Fan ZD, Yu HG. Kikuchi-Fujimoto Disease With Encephalopathy in Children: Case Reports and Literature Review. Front Pediatr 2021; 9:727411. [PMID: 34660488 PMCID: PMC8519585 DOI: 10.3389/fped.2021.727411] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/18/2021] [Accepted: 09/08/2021] [Indexed: 01/19/2023] Open
Abstract
Background: Kikuchi-Fujimoto disease (KFD) is a benign and self-limiting disease characterized by regional lymphadenitis and low-grade fever. Encephalopathy may present in children with KFD. We present three cases of KFD with encephalopathy in children and a literature review. Methods: Literature published between 2010 and 2020 was reviewed to understand the clinical features, laboratory findings, and treatments for encephalopathy occurring in children with KFD. Results: The interval between KFD and onset of neurological symptoms was 10 days to 3 months. Laboratory results were normal, except for high protein levels in cerebrospinal fluid findings. Brain magnetic resonance imaging (MRI) findings include hyperintense T2 and FLAIR signal in the supratentorial white matter, deep gray matter, brain stem, cerebellum, temporal lobes, pons, and basal ganglia. Glucocorticoids and immunoglobulin could be effective for treating KFD with encephalopathy. Conclusion: The early clinical manifestations of KFD with encephalopathy in children lack specificity, and the diagnosis is mainly based on CSF analysis and brain MRI findings. Early and timely immunomodulatory therapy is effective and can improve the prognosis of patients with KFD with encephalopathy.
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Affiliation(s)
- Yu-Ting Pan
- Department of Rheumatology and Immunology, Children's Hospital of Nanjing Medical University, Nanjing, China
| | - Li-Ming Cao
- Department of Infectious Disease, Children's Hospital of Nanjing Medical University, Nanjing, China
| | - Yan Xu
- Department of Rheumatology and Immunology, Children's Hospital of Nanjing Medical University, Nanjing, China
| | - Zhi-Dan Fan
- Department of Rheumatology and Immunology, Children's Hospital of Nanjing Medical University, Nanjing, China
| | - Hai-Guo Yu
- Department of Rheumatology and Immunology, Children's Hospital of Nanjing Medical University, Nanjing, China
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34
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Hua CZ, Chen YK, Chen SZ, Gu WZ, Shu Q. Histiocytic Necrotizing Lymphadenitis Mimicking Acute Appendicitis in a Child: A Case Report. Front Pediatr 2021; 9:682738. [PMID: 34604132 PMCID: PMC8484880 DOI: 10.3389/fped.2021.682738] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/25/2021] [Accepted: 08/06/2021] [Indexed: 11/13/2022] Open
Abstract
Background: Histiocytic necrotizing lymphadenitis, also known as Kikuchi-Fujimoto disease (KFD), is a self-limiting inflammatory disease with low incidence and high misdiagnosis rate in children. Furthermore, cases where the clinical presentation resembles acute appendicitis are very rare. Case Presentation: A 14-year-old boy was misdiagnosed as acute appendicitis and received operative treatment at his early visit. He suffered from abdominal pain, vomiting, diarrhea, fever, and lymphadenitis at the ileocecal junction, which were found by B-ultrasonography examination and surgery. Lymphadenectomy, as well as appendectomy, was performed, and KFD was identified by pathological examination. The patient was transferred to our hospital for further therapy because of recurrent fever and abdominal pain after the appendectomy. His temperature became normal after methylprednisolone was administered, and no recurrence was observed till now during follow-up. Conclusions: Necrotizing lymphadenitis involving mesenteric lymph nodes may cause acute-appendicitis-like symptom; KFD should be a diagnostic consideration for mesenteric lymphadenitis.
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Affiliation(s)
- Chun-Zhen Hua
- Department of Infectious Diseases, Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, China
| | - Yu-Kai Chen
- Department of General Surgery, The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Shun-Zhi Chen
- Department of Pediatric Surgery, Shaoxing People's Hospital, Shaoxing, China
| | - Wei-Zhong Gu
- Department of Pathology, The Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, China
| | - Qiang Shu
- Department of Pediatric Surgery, The Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, China
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35
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Lee SM, Lim YT, Jang KM, Gu MJ, Lee JH, Lee JM. Hemophagocytic lymphohistiocytosis with recurrent Kikuchi-Fujimoto disease. Yeungnam Univ J Med 2020; 38:245-250. [PMID: 33171575 PMCID: PMC8225499 DOI: 10.12701/yujm.2020.00654] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/17/2020] [Accepted: 09/14/2020] [Indexed: 11/25/2022] Open
Abstract
Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a self-limiting lymphadenitis. It is a benign disease mainly characterized by high fever, lymph node swelling, and leukopenia. Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease with clinical symptoms similar to those of KFD, but it requires a significantly more aggressive treatment. A 19-year-old Korean male patient was hospitalized for fever and cervical lymphadenopathy. Variable-sized lymph node enlargements with slightly necrotic lesions were detected on computed tomography. Biopsy specimen from a cervical lymph node showed necrotizing lymphadenitis with HLH. Bone marrow aspiration showed hemophagocytic histiocytosis. The clinical symptoms and the results of the laboratory test and bone marrow aspiration met the diagnostic criteria for HLH. The patient was diagnosed with macrophage activation syndrome—HLH, a secondary HLH associated with KFD. He was treated with dexamethasone (10 mg/m2/day) without immunosuppressive therapy or etoposide-based chemotherapy. The fever disappeared within a day, and other symptoms such as lymphadenopathy, ascites, and pleural effusion improved. Dexamethasone was reduced from day 2 of hospitalization and was tapered over 8 weeks. The patient was discharged on day 6 with continuation of dexamethasone. The patient had no recurrence at the 18-month follow-up.
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Affiliation(s)
- Sang Min Lee
- Department of Pediatrics, Yeungnam University Hospital, Daegu, Korea
| | - Young Tae Lim
- Department of Pediatrics, Yeungnam University Hospital, Daegu, Korea
| | - Kyung Mi Jang
- Department of Pediatrics, Yeungnam University College of Medicine, Daegu, Korea
| | - Mi Jin Gu
- Department of Pathology, Yeungnam University College of Medicine, Daegu, Korea
| | - Jong Ho Lee
- Department of Laboratory Medicine, Yeungnam University College of Medicine, Daegu, Korea
| | - Jae Min Lee
- Department of Pediatrics, Yeungnam University College of Medicine, Daegu, Korea
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36
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Nair IR, Balan S, Phalak P, Daniel M. Clinicopathologic spectrum of necrotizing lymphadenitis. INDIAN J PATHOL MICR 2020; 63:60-63. [PMID: 32031124 DOI: 10.4103/ijpm.ijpm_622_19] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Abstract
Background Necrotizing lymphadenitis represents a group of diseases characterized by non-granulomatous inflammation and necrosis of the lymph node, caused by a variety of infective and inflammatory diseases, most common being Kikuchi-Fujimoto disease, acute Epstein Barr viral infection and systemic lupus erythematosis (1). Objectives To study the morphological features in lymph nodes in cases of necrotizing lymphadenitis, to correlate them with specific etiological conditions.Materials and methods-58 cases of necrotizing lymphadenitis were reviewed and categorized into different etiological sub types, i.e. acute EBV lymphadenitis, lupus lymphadenitis and the rest as Kikuchis lymphadenitis. Morphological features studied were presence of vascular proliferation, periadenitis, foamy macrophage, neutrophil and plasma cell infiltrate. Clinical follow up was done. Results 62.2% of cases were Kikuchis lymphadenitis. Both lupus and Kikuchis had a female preponderance (78% and 62% respectively). Among the morphological parameters, plasma cell infiltration and vascular proliferation showed significant association with lupus lymphadenitis. Kikuchis and EBV lymphadenitis showed self-limiting course, with only 2 cases of Kikuchis developing recurrence .4 cases developed complications. All cases of lupus lymphadenitis needed long term therapy. Conclusion Kikuchis lymphadenitis is the most common cause of necrotizing lymphadenitis, followed by lupus and acute EBV lyphadenitis.Young females were commonly affected in the first 2 groups. It is worthwhile to classify the cases of necrotizing lymphadenitis into etiological subgroups as the prognosis and treatment differ (2). Among the morphological features studied, plasma cell infiltrate and vascular proliferation were significantly associated with lupus lymphadenitis, hence can be used to predict etiology.
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Affiliation(s)
- Indu R Nair
- Department of Pathology, Amrita Institute of Medical Sciences, Kochi, Kerala, India
| | - Suma Balan
- Department of Pediatric Rheumatology, Amrita Institute of Medical Sciences, Kochi, Kerala, India
| | - Pooja Phalak
- Department of Pathology, Amrita Institute of Medical Sciences, Kochi, Kerala, India
| | - Menila Daniel
- Department of Pathology, Amrita Institute of Medical Sciences, Kochi, Kerala, India
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37
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Rare Presentation of Self-Limiting Kikuchi-Fujimoto Disease in Relapsing Nature. Case Rep Med 2020; 2020:9785104. [PMID: 33014067 PMCID: PMC7525287 DOI: 10.1155/2020/9785104] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/07/2020] [Revised: 09/13/2020] [Accepted: 09/16/2020] [Indexed: 11/21/2022] Open
Abstract
Background Kikuchi–Fujimoto disease (KFD) is a rare, benign, and self-limiting disease that is commonly associated with cervical lymphadenopathy and fever. The disease has a wide spectrum of clinical manifestations, and definitive diagnosis is based on the histological appearance in the excision biopsy of the lymph nodes. Recurrence of KFD is reported rarely. Case Presentation. A 56-year-old Iranian woman with a background history of thrombocytopenia presented with fever, malaise, loss of appetite, and weight loss with cervical lymphadenopathy. The excision biopsy of the cervical lymph nodes confirmed the diagnosis of KFD, and she made a full recovery with improvement of symptoms, regression of cervical lymph nodes, and normalization inflammatory markers. One year after remission, she presented with similar clinico-biochemical profile, and repeat biopsy confirmed KFD. Conclusion Although the rate of recurrence of the disease is very low, the treating physician should consider the possibility and confirm it histologically.
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38
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Shorkey AK, Laxer RM, Friedman JN. A 10-year-old girl with cervical lymphadenopathy, fever, and cytopenia. Paediatr Child Health 2020; 25:342-344. [DOI: 10.1093/pch/pxz114] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/17/2018] [Accepted: 07/17/2019] [Indexed: 11/14/2022] Open
Affiliation(s)
- Allyson K Shorkey
- Department of Paediatrics, The Hospital for Sick Children, The University of Toronto, Toronto, Ontario
- The Hospital for Sick Children, The University of Toronto, Toronto, Ontario
| | - Ronald M Laxer
- Department of Rheumatology, The Hospital for Sick Children, The University of Toronto, Toronto, Ontario
| | - Jeremy N Friedman
- Department of Paediatrics, The Hospital for Sick Children, The University of Toronto, Toronto, Ontario
- The Hospital for Sick Children, The University of Toronto, Toronto, Ontario
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39
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Cannon L, Wang E, Becker M. Diagnosis of Kikuchi-Fujimoto disease in an 11-year-old girl with fever and sickle cell disease. BMJ Case Rep 2020; 13:13/8/e234302. [PMID: 32843398 DOI: 10.1136/bcr-2020-234302] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/03/2022] Open
Abstract
Kikuchi-Fujimoto disease (KFD) is a rare lymphohistiocytic disorder which can cause prolonged fever and other systemic B symptoms including diffuse lymphadenopathy. Given its clinical presentation, there is often initial concern for lymphoma and diagnosis requires lymph node biopsy. It most frequently affects young women of Asian descent; it is less commonly encountered in paediatric patients. KFD is typically a benign, self-limited process, however, there is an association with development of systemic lupus erythematosus. Given its rarity, it remains unclear if KFD is associated with other chronic conditions. Here we present the third case of KFD occurring in a paediatric patient with sickle cell disease.
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Affiliation(s)
- Laura Cannon
- Division of Pediatric Rheumatology, Department of Pediatrics, Duke Children's Hospital and Health Center, Durham, North Carolina, USA
| | - Endi Wang
- Department of Pathology, Duke University Hospital, Durham, North Carolina, USA
| | - Mara Becker
- Division of Pediatric Rheumatology, Department of Pediatrics, Duke Children's Hospital and Health Center, Durham, North Carolina, USA
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40
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Crawshaw H, Rachakonda KS, Kim L, Enno A, Jegatheesan T. Intensive care management of a rare case of pyrexia of unknown origin (Kikuchi-Fujimoto disease). Anaesth Intensive Care 2020; 48:318-323. [PMID: 32731741 DOI: 10.1177/0310057x20937314] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Abstract
Kikuchi-Fujimoto disease is a rare, benign cause of necrotising lymphadenitis often presenting with fever. We describe a case of a 17-year-old boy with non-verbal autism presenting to our intensive care unit with prolonged fever of unknown cause. This case highlights the role of the intensive care unit in cases of diagnostic dilemma. The critical care community should be aware of Kikuchi-Fujimoto disease as although it is usually benign, it can rarely lead to acute airway compromise.
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Affiliation(s)
- Helena Crawshaw
- Department of General and Acute Care Medicine, Liverpool Hospital, Sydney, Australia
| | - Kanaka S Rachakonda
- Department of Intensive Care Medicine, Liverpool Hospital, Sydney, Australia
| | - Leah Kim
- Department of Intensive Care Medicine, Liverpool Hospital, Sydney, Australia
| | - Alar Enno
- Department of Anatomical Pathology, Liverpool Hospital, Sydney, Australia
| | - Thulasi Jegatheesan
- Department of General and Acute Care Medicine, Liverpool Hospital, Sydney, Australia
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41
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Huang X, Chen X, Tong SW, Wang Y, Cai J, Deng C, Zhang L. Kikuchi-Fujimoto disease complicated by aseptic meningitis and hemophagocytosis successfully treated with intrathecal dexamethasone. Heliyon 2020; 6:e04193. [PMID: 32577568 PMCID: PMC7305385 DOI: 10.1016/j.heliyon.2020.e04193] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/23/2020] [Revised: 05/30/2020] [Accepted: 06/08/2020] [Indexed: 11/15/2022] Open
Abstract
Kikuchi-Fujimoto disease (KFD) is thought to be a self-limited disease featuring fever and cervical lymphadenopathy; most cases having a favorable outcome. Severe disease and death are occasionally reported. Here we report a case of KFD complicated by hemophagocytosis and aseptic meningitis. The symptoms and laboratory parameters improved after systemic glucocorticoids, intravenous immunoglobulin and one dose of intrathecal dexamethasone. Clinicians should aware of this disease and make early diagnosis by lymph node biopsy to avoid over-treatment.
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Affiliation(s)
- Xiaoyan Huang
- Department of Rheumatology, The University of Hong Kong- Shenzhen Hospital, Shenzhen, 518053, China
| | - Xixi Chen
- Department of Rheumatology, The University of Hong Kong- Shenzhen Hospital, Shenzhen, 518053, China
| | - Sun-Wing Tong
- Department of Pathology, The University of Hong Kong- Shenzhen Hospital, Shenzhen, 518053, China
| | - Yan Wang
- Department of Pathology, The University of Hong Kong- Shenzhen Hospital, Shenzhen, 518053, China
| | - Jifu Cai
- Department of Neurology, The University of Hong Kong- Shenzhen Hospital, Shenzhen, 518053, China
| | - Chaowen Deng
- Department of Microbiology, The University of Hong Kong- Shenzhen Hospital, Shenzhen, 518053, China
| | - Lijun Zhang
- Department of Rheumatology, The University of Hong Kong- Shenzhen Hospital, Shenzhen, 518053, China
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Kaur M, King S, Fotouhie A, Zangeneh TT. Unusual Presentations of Coccidioidomycosis in Conjunction With Autoimmune Syndromes: A Literature Review and Case Series. J Clin Rheumatol 2020; 26:e43-e47. [PMID: 32073532 DOI: 10.1097/rhu.0000000000000825] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Affiliation(s)
- Manjinder Kaur
- Division of Rheumatology, Department of Internal Medicine, University of Arizona, Tucson, AZ Department of Internal Medicine, College of Medicine, University of Arizona, Tucson, AZ Department of Internal Medicine, College of Medicine, University of Arizona, Tucson, AZ; Division of Infectious Disease, The Valley Fever Center for Excellence, Department of Internal Medicine, University of Arizona, Tucson, AZ
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Case Report of Kikuchi-Fujimoto Disease from Sub-Saharan Africa: An Important Mimic of Tuberculous Lymphadenitis. Case Rep Med 2020; 2020:4385286. [PMID: 31969905 PMCID: PMC6969645 DOI: 10.1155/2020/4385286] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/27/2019] [Revised: 10/08/2019] [Accepted: 12/19/2019] [Indexed: 11/20/2022] Open
Abstract
Kikuchi-Fujimoto disease (KFD) is a rare form of painful lymphadenopathy, usually cervical, which is more common in Southeast Asia and rarely reported from Africa. Symptoms are usually nonspecific (fever, night sweats, etc.), and can mimic more common diseases such as tuberculosis (TB) in endemic areas. We report a case of a 29-year-old black African woman who was admitted with headache, neck pain, fever, and lymphadenopathy. She was found to have aseptic meningitis, eventually attributed to TB based on cervical node biopsy, although further histology suggested KFD. Blood tests for systemic lupus erythematosus (SLE) were negative; she had already been commenced on anti-TB treatment and had responded well and so was continued with this therapy. She was also later diagnosed with Hashimoto's thyroiditis 3 months after her diagnosis of KFD. Five months after stopping TB treatment, she was readmitted with the same symptoms and associated painless lymphadenopathy. Repeat biopsy was morphologically similar to that of 2017, and repeat evaluation confirmed SLE. She has since been managed by a rheumatologist and continues to do well.
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Guleria S, Gupta A, Pilania RK, Pandiarajan V, Rawat A, Saikia UN, Singh S, Suri D. Kikuchi-Fujimoto Disease: An Under Recognized Cause of Fever with Lymphadenopathy. Indian J Pediatr 2020; 87:85. [PMID: 31520311 DOI: 10.1007/s12098-019-03070-8] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/28/2018] [Accepted: 08/22/2019] [Indexed: 11/25/2022]
Affiliation(s)
- Sandesh Guleria
- Pediatric Allergy and Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012, India
| | - Aman Gupta
- Pediatric Allergy and Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012, India
| | - Rakesh Kumar Pilania
- Pediatric Allergy and Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012, India
| | - Vignesh Pandiarajan
- Pediatric Allergy and Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012, India
| | - Amit Rawat
- Pediatric Allergy and Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012, India
| | - Uma Nahar Saikia
- Department of Histopathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
| | - Surjit Singh
- Pediatric Allergy and Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012, India
| | - Deepti Suri
- Pediatric Allergy and Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012, India.
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45
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Phenytoin-associated necrotising lymphadenitis mimicking Kikuchi disease. Pathology 2019; 51:650-653. [DOI: 10.1016/j.pathol.2019.04.010] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/16/2019] [Revised: 03/25/2019] [Accepted: 04/07/2019] [Indexed: 11/20/2022]
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46
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Sarfraz S, Rafique H, Ali H, Hassan SZ. Case Report: Kikuchi-Fujimoto Disease: A case of supraclavicular lymphadenopathy. F1000Res 2019; 8:1652. [PMID: 34394918 PMCID: PMC8343398 DOI: 10.12688/f1000research.19981.2] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 08/02/2021] [Indexed: 11/20/2022] Open
Abstract
Kikuchi-Fujimoto Disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare cause of cervical lymphadenopathy. Patients usually present with localized lymphadenopathy, fever and fatigue. Because of the poorly understood etiology, it can be mistaken for an infectious disease or even malignance. Here we discuss a case of KFD that initially presented with left sided cervical lymphadenopathy that later progressed to left supraclavicular lymph nodes. Due to its characteristic overlap with other disorders like tuberculous lymphadenitis and lymphoma, KFD remains an arduous diagnosis for physicians. Therefore, one should be made aware of symptoms that can lead to misdiagnosis in patients.
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Affiliation(s)
| | | | - Hassam Ali
- Quaid-e-azam Medical College, Bhawalpur, Pakistan
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Ahn SS, Lee B, Kim D, Jung SM, Lee SW, Park MC, Park YB, Hwang YG, Song JJ. Evaluation of macrophage activation syndrome in hospitalised patients with Kikuchi-Fujimoto disease based on the 2016 EULAR/ACR/PRINTO classification criteria. PLoS One 2019; 14:e0219970. [PMID: 31318961 PMCID: PMC6638985 DOI: 10.1371/journal.pone.0219970] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/13/2019] [Accepted: 07/06/2019] [Indexed: 11/18/2022] Open
Abstract
BACKGROUND To evaluate the impact of macrophage activation syndrome (MAS) on clinical features in patients with Kikuchi-Fujimoto disease (KFD) and to compare the features of MAS in KFD with those of adult-onset Still's disease (AOSD) and systemic lupus erythematosus (SLE). METHODS The medical records of febrile patients hospitalised with KFD between November 2005 and April 2017 were reviewed. Patients fulfilling the 2016 classification criteria for MAS were classified as having MAS. Clinical and laboratory features of patients with KFD with and without MAS were evaluated. Poor hospitalisation outcomes were defined as intensive care unit admission or in-hospital mortality. The treatment outcomes of MAS in KFD, AOSD, and SLE were also compared. RESULTS Among 78 patients hospitalised with KFD, 24 (30.8%) patients had MAS during admission. Patients with KFD and MAS more frequently required glucocorticoid treatment (66.7% vs 40.7%, p = 0.036) and had longer hospital stays than patients with KFD without MAS (12.5 vs 8.5 days, p<0.001). In addition, patients with MAS had worse hospitalisation outcomes than patients without MAS (29.2% vs. 0.0%, p<0.001). Among patients with MAS in KFD, AOSD, and SLE, the number of patients requiring glucocorticoid treatment after 3 months was significantly lower among patients with MAS and KFD (KFD 33.3%, AOSD 88.9%, SLE 100%, p<0.001). CONCLUSIONS The presence of MAS in KFD was associated with adverse clinical outcomes including higher steroid usage and worse hospitalisation outcomes. However, compared to those with AOSD and SLE, patients with MAS and KFD were less likely to require long-term glucocorticoid treatment.
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Affiliation(s)
- Sung Soo Ahn
- Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, South Korea
| | - Byeori Lee
- Department of Internal Medicine, Albert Einstein Medical Center Philadelphia, Philadelphia, Pennsylvania, United States of America
| | - Dam Kim
- Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, South Korea
| | - Seung Min Jung
- Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, South Korea
| | - Sang-Won Lee
- Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, South Korea
| | - Min-Chan Park
- Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, South Korea
| | - Yong-Beom Park
- Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, South Korea
| | - Yong Gil Hwang
- Department of Medicine, Division of Rheumatology and Clinical Immunology, University of Pittsburgh, Pittsburgh, Pennsylvania, United States of America
| | - Jason Jungsik Song
- Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, South Korea
- Institute for Immunology and Immunological Diseases, Yonsei University College of Medicine, Seoul, South Korea
- * E-mail:
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48
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Hwang JH, Yoo WH, An AR, Choi YJ. Coexistence of systemic lupus erythematosus with Kikuchi-Fujimoto disease involving the salivary gland, initially disguised as lymphoma. Rheumatology (Oxford) 2019; 58:550-553. [PMID: 30521051 DOI: 10.1093/rheumatology/key353] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 10/19/2018] [Indexed: 11/14/2022] Open
Affiliation(s)
- Jeong-Hwan Hwang
- Division of Infectious Disease, Research Institute of Clinical Medicine of Chonbuk National University-Biomedical Research Institute of Chonbuk National University Hospital, Jeonju, Republic of Korea
| | - Wan-Hee Yoo
- Division of Rheumatology, Department of Internal Medicine, Research Institute of Clinical Medicine of Chonbuk National University-Biomedical Research Institute of Chonbuk National University Hospital, Jeonju, Republic of Korea
| | - Ae-Ri An
- Department of Pathology, Research Institute of Clinical Medicine of Chonbuk National University-Biomedical Research Institute of Chonbuk National University Hospital, Jeonju, Republic of Korea
| | - Yun-Jung Choi
- Division of Rheumatology, Department of Internal Medicine, Research Institute of Clinical Medicine of Chonbuk National University-Biomedical Research Institute of Chonbuk National University Hospital, Jeonju, Republic of Korea
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Ozen ME, Orum MH, Kalenderoglu A, Atmaca M. Improvement of an atypical Kikuchi-Fujimoto disease (KFD) with antidepressant treatment: the first psychiatric approach to a KFD case. PSYCHIAT CLIN PSYCH 2019. [DOI: 10.1080/24750573.2018.1431352] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 10/18/2022] Open
Affiliation(s)
| | - Mehmet Hamdi Orum
- Department of Psychiatry, Adiyaman University Faculty of Medicine, Adiyaman, Turkey
| | - Aysun Kalenderoglu
- Department of Psychiatry, Adiyaman University Faculty of Medicine, Adiyaman, Turkey
| | - Murad Atmaca
- Department of Psychiatry, Firat University Faculty of Medicine, Elazig, Turkey
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Shahid S, Alam SH, Hadley I. An Unusual Presentation of Kikuchi-Fujimoto Disease with Recurrent Subdural Effusion. Cureus 2018; 10:e2302. [PMID: 29755898 PMCID: PMC5945271 DOI: 10.7759/cureus.2302] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/15/2022] Open
Abstract
A 24-year-old man complained of a right temporal headache for four weeks. The patient denied any trauma or previous anticoagulation use. He also reported tender right facial swelling. His physical exam was unrevealing except for right cranial nerve (CN) VI palsy, right parotid enlargement, and cervical adenopathy. Laboratory findings were significant for mild leukopenia at 3300 cells/uL. The computed tomography (CT) scan obtained showed a chronic left subdural effusion with a 4 mm midline shift and confirmed right parotid enlargement and cervical lymphadenopathy. Surgical burr hole evacuation was done and the fluid was sent for wound culture analysis. The infectious diseases service recommended initiating antibiotics, which were later stopped due to cerebrospinal fluid (CSF) cultures with no growth of any organisms. His CN VI palsy resolved during admission. The patient was discharged with follow-up for biopsy. The patient was lost to follow-up. The patient presented to the emergency department (ED) three months later, with a left-sided frontal headache. A repeat CT scan showed a new, right-sided fluid collection outside the brain parenchyma. Burr hole evacuation was done again and purulent fluid was drained. Antibiotics were held this time, but anti-tuberculous therapy was initiated empirically. The otolaryngology service was consulted and a lymph node biopsy was performed. The pathology showed histiocytic necrotizing lymphadenitis. A dural biopsy was done as well and was consistent with histiocytic necrotizing lymphadenitis involving the dura. Cultures from the subdural fluid did not grow any organism. The patient remained neurologically intact. He improved after surgery was done to drain the fluid and was managed by analgesics. The cultures from the extra-parenchymal fluid collection remained negative for pathogens and tuberculous mycobacteria. The patient was discharged with rheumatology clinic follow-up. He saw the rheumatologist six weeks after the discharge. During his clinic visit, the patient reported no recurrence of headaches, fevers, rash, or joint pain. Our patient had a rare presentation of Kikuchi-Fujimoto disease, in which he had a subdural fluid collection resulting in neurological complications that required surgical intervention.
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Affiliation(s)
- Sara Shahid
- Medicine, Lahore Medical And Dental College, Lahore, Pakistan
| | - Syed H Alam
- Department of Rheumatology, Temple University Hospital
| | - Indira Hadley
- Department of Rheumatology, John H Stroger J. Hospital of Cook County
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