Case Report
Copyright ©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Oct 26, 2020; 8(20): 4922-4929
Published online Oct 26, 2020. doi: 10.12998/wjcc.v8.i20.4922
Idiopathic multicentric Castleman disease with pulmonary and cutaneous lesions treated with tocilizumab: A case report
Ping-Yang Han, Hui-Hui Chi, Yu-Tong Su
Ping-Yang Han, Hui-Hui Chi, Yu-Tong Su, Department of Rheumatology and Immunology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025, China
Ping-Yang Han, Department of Rheumatology and Immunology, the Affiliated Hospital of Hangzhou Normal University, Hangzhou 310015, Zhejiang Province, China
Author contributions: Han PY designed the research, collected the data, and drafted the manuscript; Chi HH collected the data and revised the manuscript; Su YT designed the research and revised the manuscript; All authors read and approved the final manuscript.
Supported by National Natural Science Foundation of China, No. 81801600; and Shanghai Sailing Program, No. 18YF1414500.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflicts of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Yu-Tong Su, MD, PhD, Doctor, Department of Rheumatology and Immunology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, No. 197 Ruijin Second Road, Shanghai 200025, China. suyt2015@163.com
Received: May 18, 2020
Peer-review started: May 18, 2020
First decision: June 7, 2020
Revised: June 24, 2020
Accepted: September 5, 2020
Article in press: September 5, 2020
Published online: October 26, 2020
Processing time: 160 Days and 12.7 Hours
Abstract
BACKGROUND

Human herpes virus-8 (HHV-8)-negative, idiopathic multicentric Castleman disease (iMCD) is a rare and life-threatening disorder driven by proinflammatory cytokines, which is still poorly understood. Pulmonary parenchyma lesion is a rare condition in iMCD, which mainly manifests as lymphocytic interstitial pneumonia and is an indicator of severe iMCD. Cutaneous lesion is also very rare and mainly occurs in Asians. There have been few reports of iMCD patients with both skin and lung parenchyma involvement.

CASE SUMMARY

We present a Chinese man who complained about a 3-year history of intermittent dry cough and a 2-year history of diffuse reddish-brown maculopapules. Laboratory examination revealed polyclonal hypergammaglobulinemia and hypercytokinemia including interleukin 6. Chest computed tomography revealed small patchy shadows with ground-glass nodules scattered in two lobes and mediastinal lymphadenopathy. The pathological result of the lymph node was consistent with the plasma cell type of Castleman disease. As serum human immunodeficiency virus test and HHV-8 staining of the lymph node were negative, the patient was finally diagnosed with HHV-8 negative iMCD. He was treated with tocilizumab at an intravenous (i.v.) dose of 8 mg/kg every 2 wk combined with methylprednisolone at an i.v. dose of 80 mg/d initially with gradual dose tapering. Partial remission was achieved 9 mo later.

CONCLUSION

iMCD with lung parenchyma and skin involvement is a rare condition that requires clinicians’ attention and awareness for early diagnosis.

Keywords: Multicentric Castleman disease; Lymphocytic interstitial pneumonia; Skin involvement; Interleukin 6; Tocilizumab; Case report

Core Tip: We report a case of idiopathic multicentric Castleman disease (iMCD) with pulmonary parenchyma and skin involvement as the primary clinical manifestations. The patient had presented to several tertiary hospitals for more than 3 years before he was finally diagnosed. iMCD is a rare systemic lymphoproliferative disease, and delays in diagnosis are common and remain a concern. Clinicians need to improve their understanding of iMCD. Combination therapy with anti-interleukin 6 (IL-6) monoclonal antibodies and glucocorticoids is an effective frontline treatment regimen, especially for those with an elevated level of serological IL-6.