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1例抗合成酶综合征患者在糖皮质激素减量过程中同时发生进行性多灶性白质脑病合并免疫重建炎症综合征的病例报告

A case report of simultaneous PML-IRIS during corticosteroids tapering in a patient with an anti-synthetase syndrome.

作者信息

Martin-Blondel Guillaume, Brassat David, Dumas Hervé, Uro-Coste Emmanuelle, Adoue Daniel, Lassmann Hans, Clanet Michel

机构信息

Department of Infectious and Tropical Diseases, Toulouse University Hospital, Toulouse, France ; INSERM UMR 1043, Centre de Physiopathologie, Toulouse-Purpan, France ; Université Toulouse III, Toulouse F-31000, France.

INSERM UMR 1043, Centre de Physiopathologie, Toulouse-Purpan, France ; Université Toulouse III, Toulouse F-31000, France ; Department of Neurology, Toulouse University Hospital, Toulouse, France.

出版信息

F1000Res. 2013 Dec 23;2:283. doi: 10.12688/f1000research.2-283.v1. eCollection 2013.

Abstract

We report a case of simultaneous progressive multifocal leukoencephalopathy-associated immune reconstitution inflammatory syndrome (PML-IRIS) during corticosteroid tapering in a patient with an anti-synthetase syndrome. We describe the challenges associated with the diagnosis and the management of this emerging inflammatory neurological condition in this immunocompromised patient with a severe rheumatic disease. We highlight that, in the setting of IRIS, the low-level of the JC virus viral load requires a sensitive PCR assay before excluding PML.

摘要

我们报告了1例抗合成酶综合征患者在糖皮质激素减量过程中同时发生进行性多灶性白质脑病相关免疫重建炎症综合征(PML-IRIS)的病例。我们描述了在这位患有严重风湿性疾病的免疫功能低下患者中,诊断和管理这种新出现的炎性神经系统疾病所面临的挑战。我们强调,在IRIS的情况下,在排除PML之前,JC病毒载量低需要采用灵敏的PCR检测方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9ed4/3968897/2999a45edcd1/f1000research-2-3354-g0000.jpg

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