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1
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Pacchiarini MC, Di Mario F, Greco P, Fiaccadori E, Rossi GM. The Controversial Role of Glucocorticoids in Atheroembolic Renal Disease: A Narrative Review. J Clin Med 2024; 13:6441. [PMID: 39518580 PMCID: PMC11546646 DOI: 10.3390/jcm13216441] [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: 09/22/2024] [Revised: 10/20/2024] [Accepted: 10/22/2024] [Indexed: 11/16/2024] Open
Abstract
Cholesterol crystal embolism (CCE) is an underrecognized multisystemic disease caused by the displacement of cholesterol crystals from atheromatous aortic plaques to distal vascular beds, leading to ischemic injury of target organs, particularly the kidneys, i.e., atheroembolic renal disease (ARD). According to recent research, cellular necrosis, induced by crystal-induced cytotoxicity, enhances the autoinflammatory cascade of the NLPR3 inflammasome, leading in turn to the so-called "necroinflammation". The purported involvement of the latter in CCE offers a rationale for the therapeutic approach with anti-inflammatory drugs such as glucocorticoids, the use of which has long been a matter of debate in CCE. Diagnostic delay and no consistent evidence regarding efficacious treatment, leading to inconsistency in clinical practice, may worsen the already poor prognosis of ARD. The possible role of glucocorticoids in the treatment of ARD is thereby herein explored in a narrative fashion, analyzing the limited data from case reports and clinical trials.
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Affiliation(s)
- Maria Chiara Pacchiarini
- Department of Medicine and Surgery, University of Parma, 43126 Parma, Italy
- Nephrology Unit, University Hospital of Parma, 43126 Parma, Italy
| | - Francesca Di Mario
- Department of Medicine and Surgery, University of Parma, 43126 Parma, Italy
- Nephrology Unit, University Hospital of Parma, 43126 Parma, Italy
| | - Paolo Greco
- Department of Medicine and Surgery, University of Parma, 43126 Parma, Italy
- Nephrology Unit, University Hospital of Parma, 43126 Parma, Italy
| | - Enrico Fiaccadori
- Department of Medicine and Surgery, University of Parma, 43126 Parma, Italy
- Nephrology Unit, University Hospital of Parma, 43126 Parma, Italy
- Laboratorio di Immunopatologia Renale “Luigi Migone”, University of Parma, 43126 Parma, Italy
| | - Giovanni Maria Rossi
- Department of Medicine and Surgery, University of Parma, 43126 Parma, Italy
- Nephrology Unit, University Hospital of Parma, 43126 Parma, Italy
- Laboratorio di Immunopatologia Renale “Luigi Migone”, University of Parma, 43126 Parma, Italy
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2
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Cheng DJ, Li L, Zheng XY, Tang SF. Idiopathic cholesterol crystal embolism with atheroembolic renal disease and blue toes syndrome: A case report. World J Clin Cases 2022; 10:9162-9167. [PMID: 36157649 PMCID: PMC9477034 DOI: 10.12998/wjcc.v10.i25.9162] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/27/2022] [Revised: 06/28/2022] [Accepted: 07/25/2022] [Indexed: 02/05/2023] Open
Abstract
BACKGROUND Cholesterol crystal embolization (CCE) is a multisystemic and fatal disease with multiple clinical manifestations; however, there are few cases of idiopathic CCE. Here we report a patient with idiopathic CCE accompanied by atheroembolic renal disease and blue toes who had a relatively good prognosis in the short-term due to early treatment with corticosteroids and statins.
CASE SUMMARY A 76-year-old man complained of coldness, numbness and purple color change in his left foot for 7 d. He had a feeling of fatigue, constipation, foamy urine, poor appetite and sleep. He had a lacunar infarction for 5 years and hypertension for 9 mo. Laboratory results showed elevated eosinophils, cholesterol, uric acid, serum creatinine, urea and 24 h urine analysis revealed proteinuria. A renal biopsy revealed atheroembolic renal disease. Taken together, these findings strongly supported the diagnosis of idiopathic CCE and atheroembolic renal disease.
CONCLUSION Atheroembolic renal disease and blue toes syndrome can be caused by idiopathic CCE, and early treatment with corticosteroids is effective but requires further investigation.
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Affiliation(s)
- De-Jin Cheng
- Division of Nephrology, The First Affiliated Hospital of Guangzhou University of Chinese Medicine, Guangzhou 510405, Guangdong Province, China
| | - Lin Li
- Division of Nephrology, Hua Du Hospitals of Traditional Chinese and Western Medicine, Guangzhou 510800, Guangdong Province, China
| | - Xiang-Yue Zheng
- Division of Ophthalmology, The First Affiliated Hospital of Guangzhou University of Chinese Medicine, Guangzhou 510405, Guangdong Province, China
| | - Shui-Fu Tang
- Division of Nephrology, The First Affiliated Hospital of Guangzhou University of Chinese Medicine, Guangzhou 510405, Guangdong Province, China
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3
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Falcão LP, Fernandes S, Costa AC, Teixeira C, Raimundo M, Silva S, Miranda M, Almeida ED. An atypical presentation of a severe and massive atheroembolic disease. J Bras Nefrol 2019; 41:142-144. [PMID: 30129967 PMCID: PMC6534016 DOI: 10.1590/2175-8239-jbn-2018-0013] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/06/2018] [Accepted: 06/28/2018] [Indexed: 11/27/2022] Open
Abstract
Atheroembolic renal disease (AERD) is a kidney manifestation of atherosclerosis as a systemic disease. AERD is defined as a renal impairment secondary to embolization of cholesterol crystals with consequent occlusion of renal vascularization. The current case report describes one patient with multiple risk factors but without any inciting event history who presents a very atypical clinical course of a severe and massive atheroembolic disease that developed spontaneously and silently.
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Affiliation(s)
- Luis Pedro Falcão
- Hospital Beatriz Ângelo, Departamento de Nefrologia, Loures, Portugal
| | - Sara Fernandes
- Hospital Beatriz Ângelo, Departamento de Nefrologia, Loures, Portugal
| | | | - Catarina Teixeira
- Hospital Beatriz Ângelo, Departamento de Nefrologia, Loures, Portugal
| | - Mário Raimundo
- Hospital Beatriz Ângelo, Departamento de Nefrologia, Loures, Portugal
| | - Sónia Silva
- Hospital Beatriz Ângelo, Departamento de Nefrologia, Loures, Portugal
| | - Margarida Miranda
- Hospital Beatriz Ângelo, Departamento de Oftalmologia, Loures, Portugal
| | - Edgar De Almeida
- Hospital Beatriz Ângelo, Departamento de Nefrologia, Loures, Portugal
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4
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Li X, Bayliss G, Zhuang S. Cholesterol Crystal Embolism and Chronic Kidney Disease. Int J Mol Sci 2017; 18:E1120. [PMID: 28538699 PMCID: PMC5485944 DOI: 10.3390/ijms18061120] [Citation(s) in RCA: 35] [Impact Index Per Article: 4.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/15/2017] [Revised: 05/19/2017] [Accepted: 05/20/2017] [Indexed: 01/01/2023] Open
Abstract
Renal disease caused by cholesterol crystal embolism (CCE) occurs when cholesterol crystals become lodged in small renal arteries after small pieces of atheromatous plaques break off from the aorta or renal arteries and shower the downstream vascular bed. CCE is a multisystemic disease but kidneys are particularly vulnerable to atheroembolic disease, which can cause an acute, subacute, or chronic decline in renal function. This life-threatening disease may be underdiagnosed and overlooked as a cause of chronic kidney disease (CKD) among patients with advanced atherosclerosis. CCE can result from vascular surgery, angiography, or administration of anticoagulants. Atheroembolic renal disease has various clinical features that resemble those found in other kidney disorders and systemic diseases. It is commonly misdiagnosed in clinic, but confirmed by characteristic renal biopsy findings. Therapeutic options are limited, and prognosis is considered to be poor. Expanding knowledge of atheroembolic renal disease due to CCE opens perspectives for recognition, diagnosis, and treatment of this cause of progressive renal insufficiency.
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Affiliation(s)
- Xuezhu Li
- Division of Nephrology, Tongji University School of Medicine, Shanghai 200120, China.
| | - George Bayliss
- Department of Medicine, Rhode Island Hospital and Alpert Medical School, Brown University, Providence, RI 02903, USA.
| | - Shougang Zhuang
- Division of Nephrology, Tongji University School of Medicine, Shanghai 200120, China.
- Department of Medicine, Rhode Island Hospital and Alpert Medical School, Brown University, Providence, RI 02903, USA.
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5
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Igarashi Y, Akimoto T, Kobayashi T, Iwazu Y, Miki T, Otani-Takei N, Imai T, Sugase T, Masuda T, Takeda SI, Muto S, Nagata D. Performing Anticoagulation: A Puzzling Case of Cholesterol Embolization Syndrome. CLINICAL MEDICINE INSIGHTS-CASE REPORTS 2017; 10:1179547616684649. [PMID: 28469497 PMCID: PMC5398301 DOI: 10.1177/1179547616684649] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/05/2016] [Accepted: 11/21/2016] [Indexed: 12/04/2022]
Abstract
The avoidance of any form of anticoagulation is advised in cases of cholesterol embolization syndrome (CES). We herein describe a case of CES in a man with a history of unprovoked pulmonary embolism for which warfarinization was performed. Despite anecdotal reports of successful anticoagulation in CES patients with certain indications, irreversible renal failure, which was sufficiently severe to require chronic hemodialysis, eventually developed in our patient. Our results emphasize the pitfalls of this procedure, which imply its limited feasibility and safety. Several therapeutic concerns associated with this case are also discussed.
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Affiliation(s)
- Yusuke Igarashi
- Division of Nephrology, Department of Internal Medicine, Jichi Medical University, Shimotsuke-Shi, Japan
| | - Tetsu Akimoto
- Division of Nephrology, Department of Internal Medicine, Jichi Medical University, Shimotsuke-Shi, Japan
| | - Takahisa Kobayashi
- Division of Nephrology, Department of Internal Medicine, Jichi Medical University, Shimotsuke-Shi, Japan
| | - Yoshitaka Iwazu
- Division of Nephrology, Department of Internal Medicine, Jichi Medical University, Shimotsuke-Shi, Japan
| | - Takuya Miki
- Division of Nephrology, Department of Internal Medicine, Jichi Medical University, Shimotsuke-Shi, Japan
| | - Naoko Otani-Takei
- Division of Nephrology, Department of Internal Medicine, Jichi Medical University, Shimotsuke-Shi, Japan
| | - Toshimi Imai
- Division of Nephrology, Department of Internal Medicine, Jichi Medical University, Shimotsuke-Shi, Japan
| | - Taro Sugase
- Division of Nephrology, Department of Internal Medicine, Jichi Medical University, Shimotsuke-Shi, Japan
| | - Takahiro Masuda
- Division of Nephrology, Department of Internal Medicine, Jichi Medical University, Shimotsuke-Shi, Japan
| | - Shin-Ichi Takeda
- Division of Nephrology, Department of Internal Medicine, Jichi Medical University, Shimotsuke-Shi, Japan
| | - Shigeaki Muto
- Division of Nephrology, Department of Internal Medicine, Jichi Medical University, Shimotsuke-Shi, Japan
| | - Daisuke Nagata
- Division of Nephrology, Department of Internal Medicine, Jichi Medical University, Shimotsuke-Shi, Japan
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6
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Abstract
Atheromatous embolization is a multisystem disease complicating advanced atherosclerosis. It occurs most often as a complication of angiography, an endovascular procedure or cardiovascular surgery. Atheromatous embolization can present in a subtle manner where it is often under-recognized, or with catastrophic results including myocardial infarction, strake or acute renal failure. It may mimic other disease processes and often goes underdiagnosed and undertreated. A high clinical suspicion is the key to diagnosis. Atheromatous embolization results in significant morbidity and mortality; therefore, early recognition followed by aggressive management may help to prevent end-organ damage and improve overall clinical outcomes. Management strategies should include risk factor modification, prevention of further insults by discontinuing or avoiding predisposing factors, supportive treatment and interventional or surgical approaches to remove the atheroembolic source. Atheromatous embolization is expected to increase as our population ages and the epidemics of diabetes mellitus and obesity increase.
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Affiliation(s)
- Yin Ping Liew
- Department of Cardiovascular Medicine, Section of Vascular Medicine, Cleveland Clinic Foundation, Cleveland, Ohio 44195, USA
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7
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Motegi SI, Abe M, Shimizu A, Tamura A, Hatori T, Nakano A, Hasegawa A, Kurabayashi M, Ishikawa O. Cholesterol Crystal Embolization: Skin Manifestation, Gastrointestinal and Central Nervous Symptom Treated with Corticosteroid. J Dermatol 2014; 32:295-8. [PMID: 15863854 DOI: 10.1111/j.1346-8138.2005.tb00766.x] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/23/2004] [Accepted: 11/30/2004] [Indexed: 11/30/2022]
Abstract
Cholesterol crystal embolization (CCE) is characterized by tissue ischemia secondary to occlusion of small arteries. It may occur spontaneously but more often follows radiological interventional procedures or vascular surgery. This systemic disease affects multiple organs, including skin, kidney, brain, eye, and gastrointestinal tract. We reported a Japanese male CCE patient with cutaneous manifestations of livedo reticularis, diarrhea, clouding of consciousness, and acute renal failure. Histopathological examination demonstrated multiple biconvex clefts in a vessel of the subcutis. Corticosteroid administration improved his consciousness, diarrhea and skin lesions. Awareness of the skin manifestations of CCE is essential for dermatologists to make an early diagnosis and prescribe appropriate treatment.
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MESH Headings
- Administration, Oral
- Aged
- Anti-Inflammatory Agents/therapeutic use
- Crystallization
- Diagnosis, Differential
- Embolism, Cholesterol/complications
- Embolism, Cholesterol/diagnosis
- Embolism, Cholesterol/drug therapy
- Embolism, Cholesterol/pathology
- Humans
- Male
- Myocardial Infarction/complications
- Prednisolone/therapeutic use
- Renal Insufficiency/complications
- Renal Insufficiency/diagnosis
- Renal Insufficiency/drug therapy
- Renal Insufficiency/pathology
- Skin Diseases, Vascular/complications
- Skin Diseases, Vascular/diagnosis
- Skin Diseases, Vascular/drug therapy
- Skin Diseases, Vascular/pathology
- Syndrome
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Affiliation(s)
- Sei-ichiro Motegi
- Department of Dermatology, Gunma University Graduate School of Medicine, Gunma 371-8511, Japan
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8
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Masuda J, Tanigawa T, Nakamori S, Sawai T, Murata T, Ishikawa E, Yamada N, Nakamura M, Ito M. Use of corticosteroids in the treatment of cholesterol crystal embolism after cardiac catheterization: a report of four Japanese cases. Intern Med 2013; 52:993-8. [PMID: 23648720 DOI: 10.2169/internalmedicine.52.9255] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/13/2023] Open
Abstract
Cholesterol crystal embolism (CCE) is a serious complication associated with invasive vascular procedures. The prognosis of the renal involvement type of CCE is very poor, and there is currently no established treatment, other than supportive therapy. We herein report four cases of CCE with severe atherosclerosis wherein the renal function progressively deteriorated after cardiac catheterization. In three of the four patients, low-dose corticosteroids (0.3 mg/kg/day) improved the renal function, whereas the fourth patient died from CCE of the digestive system. This report reviews the literature on CCE and discusses possible therapeutic options.
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Affiliation(s)
- Jun Masuda
- Department of Cardiology and Nephrology, Mie University Graduate School of Medicine, Japan.
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9
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Nakayama M, Izumaru K, Nagata M, Ikeda H, Nishida K, Hasegawa E, Ohta Y, Tsuchihashi T, Urabe K. The Effect of Low-Dose Corticosteroids on Short- and Long-Term Renal Outcome in Patients with Cholesterol Crystal Embolism. Ren Fail 2011; 33:298-306. [DOI: 10.3109/0886022x.2011.560403] [Citation(s) in RCA: 23] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/30/2023] Open
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10
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Faria B, Vidinha J, Pêgo C, Garrido J, Lemos S, Lima C, Sorbo G, Gomes EL, Carvalho T, Loureiro P, Sousa T. Atheroembolic renal disease with rapid progression and fatal outcome. Clin Exp Nephrol 2010; 15:159-63. [PMID: 21069411 DOI: 10.1007/s10157-010-0363-3] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/17/2010] [Accepted: 09/29/2010] [Indexed: 11/24/2022]
Abstract
Atheroembolic renal disease is caused by foreign-body reaction to cholesterol crystals flushed from the atherosclerotic plaques into the small-vessel system of the kidneys. It is an underdiagnosed entity, mostly related to vascular procedures and/or anticoagulation, and prognosis is considered to be poor. Besides the benefit of aggressive medical prevention of further embolic events, use of steroid therapy has been associated with greater survival. Here we report a case of a patient with a multisystemic presentation of the disease days after performance of percutaneous coronary intervention and anticoagulation initiation due to an episode of myocardial infarction. Renal, cutaneous, ophthalmic, neurological, and possibly muscular and mesenteric involvement was diagnosed. Although medical treatment with corticosteroids and avoidance of further anticoagulation was applied, the patient rapidly progressed to end-stage renal disease requiring hemodialysis and died 6 months after diagnosis. This is a case of catastrophic progression of the disease resistant to therapeutic measures. Focus on diagnosis and more efficient preventive and therapeutic protocols are therefore needed.
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Affiliation(s)
- Bernardo Faria
- Nephrology and Dialysis Unit, Hospital São Teotónio-Viseu, Av Rei Dom Duarte, 3504 509 Viseu, Portugal.
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11
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Paraskevas KI, Koutsias S, Mikhailidis DP, Giannoukas AD. Cholesterol Crystal Embolization:A Possible Complication of Peripheral Endovascular Interventions. J Endovasc Ther 2008; 15:614-25. [DOI: 10.1583/08-2395.1] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/21/2022]
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12
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Filip JR, Dillon RS. Treatment of end-stage "trash feet" with the end-diastolic pneumatic boot. Angiology 2008; 59:214-9. [PMID: 18403461 DOI: 10.1177/0003319707305984] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Abstract
This study reassessed the clinical effect of Circulator Boot (CB) therapy in patients with cholesterol embolization syndrome (CES) of the lower extremities. The medical records were reviewed of 27 patients consecutively referred to the Bryn Mawr Wound Care and Vascular Center with CES who had not responded to previous therapies. All patients with CES referred from January 1, 1997, to September 19, 2005, were followed up and included in the study. The alternate therapy offered for most patients at the time of referral was limb amputation. The median age of the patients was 65 years (age range, 46-84 years) at the time of diagnosis. Healing of CES was observed after a median interval of 11 months (range, 3-32 months) following the initiation of CB therapy. The total number of legs treated was 41. Of 41 legs, 33 (81%) were totally healed, 6 (15%) improved, and 2 (5%) were amputated. After an initial period of improvement, one patient died a month later of causes unrelated to CES or CB therapy. Another patient improved and discontinued treatment before he was totally healed. Cholesterol embolization syndrome is seen predominantly in patients following cardiac or vascular procedures but may occur spontaneously. The CB seems to be the only effective noninvasive therapy for CES. Early initiation of therapy is essential to minimize tissue loss and patient discomfort.
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Affiliation(s)
- John R Filip
- Department of Internal Medicine, Bryn Mawr Hospital, Bryn Mawr, PA 19010, USA.
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13
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Jucgla A, Moreso F, Muniesa C, Moreno A, Vidaller A. Cholesterol embolism: Still an unrecognized entity with a high mortality rate. J Am Acad Dermatol 2006; 55:786-93. [PMID: 17052483 DOI: 10.1016/j.jaad.2006.05.012] [Citation(s) in RCA: 31] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/31/2006] [Revised: 04/28/2006] [Accepted: 05/05/2006] [Indexed: 12/27/2022]
Abstract
BACKGROUND Cholesterol embolism (CE) is an increasingly common but often underdiagnosed medical problem. The recognition of clinical manifestations of CE is the first step toward a correct diagnosis. OBJECTIVE Our aim was to characterize the features of CE and the risk factors for fatal outcome. METHODS Clinical records of patients with clinical and histopathologic diagnoses of CE seen from January 1993 through March 2003 were reviewed. RESULTS Twenty-six male patients were identified. Mean age was 64 years (range, 48-88 years). All patients had two or more risk factors for atherosclerosis. All but one patient had preexisting symptomatic atherosclerotic disease. At least one precipitating factor was identified in 23 patients (88%). Diagnosis of CE at admission was made in 9 patients only (35%). Cutaneous lesions (88%) and renal failure (73%) were the most common clinical findings. Complications (dialysis, acute pulmonary edema, amputation, or gastrointestinal surgery) were present in 21 patients (81%), and 15 patients died (58%). Previous chronic renal failure was the only variable associated with mortality (relative risk: 4.54, 95% confidence interval 1.26-16.6; P = .02). LIMITATIONS The results were obtained from patients admitted to a university hospital. This fact may have selected a higher proportion of severe cases. CONCLUSIONS CE was frequently misdiagnosed. Skin lesions were the most common clinical findings and skin biopsy provided histologic confirmation in most of the patients. Chronic renal failure was the only factor related to death.
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Affiliation(s)
- Anna Jucgla
- Department of Dermatology, Hospital Universitari de Bellvitge, University of Barcelona, Spain.
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14
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Cheng C, Tsuneyama K, Zheng H, Oya T, Cui Z, Feril LB, Takano Y. Enhanced scavenging of lipid substances is a possible effect of corticosteroids in the treatment of cholesterol crystal embolism. Pathol Res Pract 2006; 202:591-8. [PMID: 16814943 DOI: 10.1016/j.prp.2006.04.005] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/03/2006] [Accepted: 04/04/2006] [Indexed: 10/24/2022]
Abstract
Cholesterol crystal embolism (CCE) is a systemic refractory disease especially prevalent amongst elderly patients suffering from atherosclerosis. Treatment of this condition remains controversial due to difficulties in diagnosis. Corticosteroid therapy may be an important treatment option despite its elusive mechanisms. To clarify the role of corticosteroid in CCE therapy, we collected the samples from six autopsied subjects with CCE, three of whom were clinically given various doses of corticosteroid to investigate stable atherosclerosis-related substances, advanced glycation end-products (AGE), and several AGE receptors such as scavenger receptor class B type 1 (SR-B1), receptor for AGE (RAGE), and galectin-3 in the liver tissues and atherosclerotic areas by immunostaining using a tissue macro-array technique. An intense expression of AGE and its receptors was identified in the enlarged Kupffer cells of CCE cases, which were given relatively high doses of corticosteroid. In addition, numerous mononuclear cells in the intimal atheromatous plaque presented strong expressions of AGE and SR-B1. In conclusion, we speculated that corticosteroid treatment for CCE may upregulate the activations, including phagocytic capacity of Kupffer cells mediated by overexpression of RAGE and scavenger receptors, resulting in efficient clearance of the lipid substances from the blood circulation released from atherosclerotic areas.
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Affiliation(s)
- Chunmei Cheng
- Department of Pathology (I), School of Medicine, University of Toyama, 2630 Sugitani, Toyama 930-0194, Japan
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15
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Matsumura T, Matsumoto A, Ohno M, Suzuki S, Ohta M, Suzuki E, Takenaka K, Hirata Y, Fujita T, Nagai R. A Case of Cholesterol Embolism Confirmed by Skin Biopsy and Successfully Treated with Statins and Steroids. Am J Med Sci 2006; 331:280-3. [PMID: 16702800 DOI: 10.1097/00000441-200605000-00010] [Citation(s) in RCA: 14] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
Although cholesterol embolism syndrome was recognized as a clinicopathologic entity more than 50 years ago, it is attracting growing attention recently. It is a multisystemic disorder in which cholesterol crystals released from atherosclerotic plaques obstruct small arterioles, resulting in local ischemia and end-organ damage. There are no established treatments, and with the limited treatment options available, it is important to make the diagnosis as early as possible. We present the case of a 68-year-old man with cholesterol embolism who had a few fluttering atheromas in the aorta, as demonstrated by transesophageal ultrasonography. The diagnosis was confirmed by skin biopsy, and treatment with statins and steroids proved effective, as renal failure progressively improved. This case emphasizes the importance of early diagnosis and shows the possible therapeutic effects of statins and steroids for patients with this syndrome.
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Affiliation(s)
- Takayoshi Matsumura
- Department of Cardiovascular Medicine, University of Tokyo, Bunkyo-ku, Tokyo, Japan
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16
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Koga JI, Ohno M, Okamoto K, Nakasuga K, Ito H, Nagafuji K, Shimono N, Koga H, Hayashida A, Arita T, Maruyama T, Kaji Y, Harada M. Cholesterol embolization treated with corticosteroids--two case reports. Angiology 2005; 56:497-501. [PMID: 16079936 DOI: 10.1177/000331970505600420] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
Abstract
Cholesterol embolization (CE) is a potentially serious complication associated with invasive arterial maneuvers, in which standard therapy has not been established. We experienced two cases of CE in patients with severe atherosclerosis whose renal function deteriorated within a few months after invasive arterial maneuvers. CE was confirmed either by renal biopsy (case 1) or skin biopsy (case 2). Oral administration of prednisolone at a daily dose of 30 mg (0.4 mg/kg) was effective to improve their renal function. Our observation suggests that corticosteroid therapy may be beneficial in some patients with CE.
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Affiliation(s)
- Jun-ichiro Koga
- Department of Medicine and Biosystemic Science, Kyushu University Graduate School of Medical Sciences, Fukuoka, Japan
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17
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Kraemer M, Linden D, Berlit P. The spectrum of differential diagnosis in neurological patients with livedo reticularis and livedo racemosa. A literature review. J Neurol 2005; 252:1155-66. [PMID: 16133722 DOI: 10.1007/s00415-005-0967-9] [Citation(s) in RCA: 53] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/01/2005] [Revised: 06/16/2005] [Accepted: 06/28/2005] [Indexed: 10/25/2022]
Abstract
Livedo is a cutaneous sign of striking violaceous netlike patterned erythema of the skin. This dermatological phenomenon is of special interest in the differential diagnosis in neurological patients. In 1907 Ehrmann distinguished two different patterns of livedo: the pathological livedo racemosa and the physiological livedo reticularis. Despite important clinical differences, in the English language literature the heading livedo reticularis is still used for all types of livedo. A literature review about the spectrum of differential diagnosis in patients with livedo reticularis (especially cutis marmorata and amantadine-induced livedo reticularis) and livedo racemosa (especially Sneddon's syndrome, Divry-van Bogaert syndrome, systemic lupus erythematosus, antiphospholipid antibody syndrome, polyarteritis nodosa, cholesterol embolization syndrome, livedoid vasculopathy and haematological diseases) is provided.
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Affiliation(s)
- Markus Kraemer
- Department of Neurology, Alfried Krupp von Bohlen und Halbach Hospital, Alfried Krupp Str. 21, 45117 Essen, Germany.
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Sos TA, Trost DW. What is the Natural History of an Asymptomatic Renal Artery Stenosis: Is Treatment of Asymptomatic Lesions Justified? J Vasc Interv Radiol 2005. [DOI: 10.1016/s1051-0443(05)70082-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/18/2022] Open
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19
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Hagiwara N, Toyoda K, Nakayama M, Inoue T, Yasumori K, Ibayashi S, Okada Y. Renal cholesterol embolism in patients with carotid stenosis: a severe and underdiagnosed complication following cerebrovascular procedures. J Neurol Sci 2004; 222:109-12. [PMID: 15240205 DOI: 10.1016/j.jns.2004.04.003] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/04/2003] [Revised: 03/04/2004] [Accepted: 04/06/2004] [Indexed: 11/16/2022]
Abstract
Here, we report two cases with rapidly progressive renal failure, caused by cholesterol crystal embolism (CCE), after an angiography for carotid artery stenosis. The diagnosis was determined by histological examination and from clinical symptoms, including livedo reticularis and eosinophilia. Neurologists and neuroradiologists tend to underdiagnose CCE, which results from the same atherosclerotic risk factors as cerebrovascular disease. We need to understand more about CCE and identify its unique clinical symptoms to enable an early diagnosis and treatment.
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Affiliation(s)
- Noriko Hagiwara
- Department of Cerebrovascular Disease, Cerebrovascular Center and Clinical Research Institute, National Kyushu Medical Center, 1-8-1 Jigyohama, Chuou, Fukuoka 810-8563, Japan
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20
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Funabiki K, Masuoka H, Shimizu H, Emi Y, Mori T, Ito M, Nakano T. Cholesterol crystal embolization (CCE) after cardiac catheterization: a case report and a review of 36 cases in the Japanese literature. ACTA ACUST UNITED AC 2003; 44:767-74. [PMID: 14587658 DOI: 10.1536/jhj.44.767] [Citation(s) in RCA: 41] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/18/2022]
Abstract
Cholesterol crystal embolization (CCE) is a complication of atherosclerosis. A 67-year-old Japanese man underwent coronary artery bypass grafting. After the surgery, he underwent coronary angiography via the right femoral artery. Twelve days later, he suddenly developed acalculous cholecystitis and was treated with antibiotics. Gradual deterioration in renal function, purplish discoloration of the distal portion of his toes, and eosinophilia were noted. We performed a skin biopsy and made a diagnosis of CCE. Cilostazol and intravenous heparin improved the symptoms and decreased the creatinine level. We retrospectively studied the clinical features of 36 cases registered with a diagnosis of CCE in the Japanese literature.
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Affiliation(s)
- Kaoru Funabiki
- Division of Internal Medicine, Ise General Hospital, Ise, Japan
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Donohue KG, Saap L, Falanga V. Cholesterol crystal embolization: an atherosclerotic disease with frequent and varied cutaneous manifestations. J Eur Acad Dermatol Venereol 2003; 17:504-11. [PMID: 12941082 DOI: 10.1046/j.1468-3083.2003.00710.x] [Citation(s) in RCA: 48] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/19/2022]
Abstract
In this paper the basic pathogenesis of cholesterol crystal embolization (CCE) is described, its clinical characteristics are presented and diagnosis and therapy are discussed. The main focus will be on the cutaneous manifestations; however, considering that CCE is a systemic illness, findings in other organs will also be highlighted, particularly the commonly involved renal and gastrointestinal systems.
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Affiliation(s)
- K G Donohue
- Department of Dermatology and Skin Surgery, Roger Williams Medical Center, Providence, Rhode Island, USA
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22
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Sos TA, Trost DW. PTRA and Stenting for Renal Insufficiency: What Do We Know about Outcomes? J Vasc Interv Radiol 2003. [DOI: 10.1016/s1051-0443(03)70146-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022] Open
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23
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Sheikh RA, Prindiville TP, Yasmeen S, Ruebner BH. Cholesterol crystal embolization presenting as a colonic pseudotumor: case report and review. Gastrointest Endosc 2001; 54:378-81. [PMID: 11522986 DOI: 10.1067/mge.2001.116567] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/10/2022]
Affiliation(s)
- R A Sheikh
- Division of Gastroenterology, Department of Medicine, San Joaquin General Hospital, Stockton, California 95201, USA
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Abstract
Aortic atheroembolization is a feared complication of invasive procedures such as arteriography, often leading to devastating complications including renal insufficiency. To date, even in cases with evolving renal failure, there is no recommended treatment. This case report describes the successful treatment with corticosteroids of a patient with deteriorating renal function after renal arteriography and angioplasty, resulting in rapid and sustained improvement in renal function. The implications of this observation are discussed.
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Affiliation(s)
- S J Mann
- New York Presbyterian Hospital--Weill/Cornell Medical Center, New York 10021, USA.
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26
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Sos TA. Stents versus Angioplasty in Renal Artery Stenosis. J Vasc Interv Radiol 2001. [DOI: 10.1016/s1051-0443(01)70063-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/18/2022] Open
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