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抗 MDA5 抗体阳性皮肌炎伴快速进展性间质性肺炎,在接受皮质类固醇和环孢素治疗期间出现肾病综合征。

Anti-MDA5 Antibody-positive Dermatomyositis with Rapidly Progressive Interstitial Pneumonia Presenting with Nephrotic Syndrome during Treatment with Corticosteroids and Cyclosporine.

机构信息

Department of Nephrology, Dokkyo Medical University, Saitama Medical Center, Japan.

Department of Pathology, Dokkyo Medical University, Saitama Medical Center, Japan.

出版信息

Intern Med. 2022 Jul 1;61(13):2007-2012. doi: 10.2169/internalmedicine.8311-21. Epub 2021 Nov 13.

Abstract

A 50-year-old Japanese woman with anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5 antibody)-positive dermatomyositis presenting with rapidly progressive interstitial pneumonia was treated with corticosteroids and cyclosporine. She developed nephrotic syndrome during the treatment regimen with corticosteroids and cyclosporine. A kidney biopsy revealed a thrombotic microangiopathy (TMA) glomerular lesion. Anti-MDA5 antibody-positive dermatomyositis is prone to severe interstitial lung disease (ILD) and is often exacerbated and refractory to treatment. Renal symptoms might be due to TMA of the kidney, and this may be a sign that more intensive treatment is needed. Patients sometimes develop acute kidney injury, which may be due to the TMA.

摘要

一位 50 岁的日本女性,患有抗黑色素瘤分化相关基因 5 抗体(抗 MDA5 抗体)阳性皮肌炎,表现为快速进展性间质性肺炎,接受了皮质类固醇和环孢素治疗。在皮质类固醇和环孢素治疗方案中,她出现了肾病综合征。肾脏活检显示血栓性微血管病(TMA)肾小球病变。抗 MDA5 抗体阳性皮肌炎易发生严重的间质性肺病(ILD),并且经常加重且对治疗有抗性。肾脏症状可能是由于肾脏的 TMA,这可能是需要更强化治疗的迹象。患者有时会发生急性肾损伤,这可能是由于 TMA 引起的。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4ea1/9334254/17ec356f247a/1349-7235-61-2007-g001.jpg

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