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成人斯蒂尔病:一种对标准治疗无效的非典型表现。

Adult-Onset Still's Disease: An Atypical Presentation Refractory to Standard Treatment.

作者信息

Esmaeili-Ghahfarokhi Melody, Kim Brandon H, Bozorgnia Farshid

机构信息

Internal Medicine, University of California Irvine Medical Center, Orange, USA.

Rheumatology, University of California Irvine Medical Center, Orange, USA.

出版信息

Cureus. 2024 Jul 11;16(7):e64369. doi: 10.7759/cureus.64369. eCollection 2024 Jul.

Abstract

A previously healthy young female of Southeast Asian descent presented with a two-week history of polyarthritis, urticarial rash, sore throat, and 8.6 kg of unintentional weight loss. The initial workup revealed a positive parvovirus B19 polymerase chain reaction with hyperferritinemia. The patient was diagnosed with adult-onset Still's disease (AOSD) secondary to parvovirus B19 infection. Bone marrow biopsy also showed evidence of hemophagocytic lymphohistiocytosis. Viral and bacterial infections may trigger AOSD in genetically susceptible hosts either via an unknown mechanism or by direct cytotoxic effect. This case shows an atypical presentation of AOSD, as well as the challenge in diagnosing and treating AOSD complicated by macrophage activation syndrome refractory to standard treatment.

摘要

一名既往健康的东南亚裔年轻女性,出现了两周的多关节炎、荨麻疹样皮疹、咽痛病史,且非故意体重减轻8.6千克。初步检查显示细小病毒B19聚合酶链反应阳性伴高铁蛋白血症。该患者被诊断为继发于细小病毒B19感染的成人斯蒂尔病(AOSD)。骨髓活检也显示有噬血细胞性淋巴组织细胞增生症的证据。病毒和细菌感染可能通过未知机制或直接细胞毒性作用,在遗传易感性宿主中引发AOSD。本病例展示了AOSD的非典型表现,以及诊断和治疗并发巨噬细胞活化综合征且对标准治疗难治的AOSD所面临的挑战。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5bed/11316915/a755f77f4915/cureus-0016-00000064369-i01.jpg

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