Bouvry Diane, Naccache Jean-Marc, Valeyre Dominique
Service de pneumologie, hôpital Avicenne, GHU Nord, Assistance publique-hôpitaux de Paris et UFR SMBH Bobigny, Uuniversité Paris 13, 93009 Bobigny.
Rev Prat. 2007 Dec 31;57(20):2258-65.
Sarcoidosis is a multisystem disorder of unknown aetiology characterized by immune granuloma in involved tissue with predilection for lung and lymphoid system. The sarcoidosis aetiology remains unknown. It could result of genetic predisposition and exposure to specific enviromental factors. The chest X-ray is abnormal in 90% of cases with adenopathies and/or massive pulmonary infiltration with or without fibrosis. The different aspects are classified in 4 stages. The diagnosis is done when clinical and radiological usual signs are present with histological granuloma without caseum and in the absence of any other granulomatous disease. Most of the time, sarcoidosis spontaneously heals in 2 to 3 years. 10 to 30% of cases are chronic and often more severe. The disease could lastly generate a pulmonary fibrosis with a potential respiratory insufficiency, cor pulmonale, or aspergillus infection. Cardiac, neurologic, throat, kidney or ophthalmologic lesions or hypercalcemia may also be of bad prognosis. Sarcoidosis is lethal in 0.5 to 5% of cases. Mild disease does not need to be treated. Systemic corticosteroid for 12 months at least is the treatment for more severe diseases. Antimalarials drugs and immunomodulatory agents may be used if corticosteroids cannot be used or failed. TNFalpha recently proposed is currently evaluated.
结节病是一种病因不明的多系统疾病，其特征为受累组织中出现免疫性肉芽肿，好发于肺和淋巴系统。结节病的病因仍不清楚。它可能是遗传易感性和接触特定环境因素所致。90%的病例胸部X线检查异常，表现为淋巴结肿大和/或有或无纤维化的大量肺部浸润。不同表现分为4期。当出现临床和放射学常见征象，伴有无干酪样坏死的组织学肉芽肿，且无任何其他肉芽肿性疾病时，可作出诊断。大多数情况下，结节病在2至3年内可自发痊愈。10%至30%的病例呈慢性，且往往病情更严重。该疾病最终可能导致肺纤维化，伴有潜在的呼吸功能不全、肺心病或曲霉菌感染。心脏、神经、咽喉、肾脏或眼科病变或高钙血症也可能预后不良。结节病在0.5%至5%的病例中是致命的。轻症无需治疗。对于病情较重的疾病，至少需使用全身性皮质类固醇治疗12个月。如果不能使用皮质类固醇或治疗失败，可使用抗疟药和免疫调节剂。最近提出的肿瘤坏死因子α目前正在进行评估。
