Idiopathic multicentric Castleman disease with pulmonary and cutaneous lesions treated with tocilizumab: A case report.

Autor:	Han PY; Department of Rheumatology and Immunology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025, China., Chi HH; Department of Rheumatology and Immunology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025, China., Su YT; Department of Rheumatology and Immunology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025, China. suyt2015@163.com.
Jazyk:	angličtina
Zdroj:	World journal of clinical cases [World J Clin Cases] 2020 Oct 26; Vol. 8 (20), pp. 4922-4929.
DOI:	10.12998/wjcc.v8.i20.4922
Abstrakt:	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. Competing Interests: Conflict-of-interest statement: The authors declare that they have no conflicts of interest. (©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.)
Databáze:	MEDLINE
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