Valeyre D, Uzunhan Y, Bouvry D, Naccache J-M, Nunes H
Université Paris Nord, EA2364 and Assistance publique-hôpitaux de Paris, Avicenne hospital, Pulmonary Department.
Acta Clin Belg. 2008 Nov-Dec;63(6):408-13. doi: 10.1179/acb.2008.084.
Sarcoidosis is a multisystemic immune disorder of unknown cause characterized by the formation of epithelioid granuloma in involved organs particularly the lung and the lymphatic system. Consistent recent advances have been made on the clinical, therapeutical and pathogenetic aspects.
Various clinical phenotypes are better characterized and efforts to grade the severity could facilitate genetic studies and therapeutic trials. Pulmonary hypertension and some infrequent or atypical pulmonary manifestations as well as some rare extrapulmonary localizations have been thoroughly studied. Sarcoidosis-like granulomatosis due to identified causes constitute interesting models and have to be differentiated from sarcoidosis. THERAPEUTICAL ASPECTS: Anti-TNFalpha drugs have been investigated and infliximab has been proved efficient even though some limits to its efficiency have to be taken into account. PATHOGENETIC ASPECTS: Finally, advances concerning the pathogenesis, particularly genetic predisposing factors and phenotype/genotype correlations have been made.
结节病是一种病因不明的多系统免疫紊乱疾病，其特征是在受累器官尤其是肺和淋巴系统中形成上皮样肉芽肿。近期在临床、治疗和发病机制方面均取得了持续进展。
各种临床表型得到了更好的界定， severity分级的努力有助于基因研究和治疗试验。肺动脉高压以及一些不常见或非典型的肺部表现以及一些罕见的肺外定位已得到深入研究。由已确定病因引起的结节病样肉芽肿病构成了有趣的模型，必须与结节病相鉴别。治疗方面：已对抗TNFα药物进行了研究，英夫利昔单抗已被证明有效，尽管必须考虑其有效性的一些局限性。发病机制方面：最后，在发病机制方面取得了进展，特别是在遗传易感性因素以及表型/基因型相关性方面。
