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[重症胸外结节病]

[Severe extrathoracic sarcoidosis].

作者信息

Chapelon Abric C

机构信息

Service de médecine interne II, CHU Pitié-Salpêtrière, 47-83, boulevard de l'Hôpital, 75013 Paris, France.

出版信息

Rev Med Interne. 2011 Feb;32(2):80-5. doi: 10.1016/j.revmed.2010.08.024.

Abstract

Sarcoidosis may affect functional outcome and sometimes may be a life-threatening condition. Functional outcome is particularly compromised by cutaneous, ocular, nasal, osteoarticular manifestations and some neurological expression. Central nervous system involvement and cardiac sarcoidosis could be life-threatening, as laryngeal and renal involvement. Diagnosis is sometimes very difficult to obtain. Several tissue biopsies are usually necessary to rule out all differential diagnosis. Extrathoracic manifestations of severe sarcoidosis require a corticosteroid therapy, initiated, if life-threatening at a daily dose of 1 mg/kg, usually associated with an other immunosuppressive drug (methotrexate, cyclophosphamide, anti TNFα).

摘要

结节病可能影响功能预后,有时可能是一种危及生命的疾病。皮肤、眼部、鼻部、骨关节表现及一些神经症状会特别影响功能预后。中枢神经系统受累和心脏结节病可能危及生命,喉和肾受累情况也是如此。有时很难做出诊断。通常需要多次组织活检以排除所有鉴别诊断。严重结节病的胸外表现需要使用皮质类固醇治疗,如果危及生命则以每日1毫克/千克的剂量开始,通常联合其他免疫抑制药物(甲氨蝶呤、环磷酰胺、抗TNFα)使用。

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