结节病研究进展

Research progress on sarcoidosis

结节病是一种以非干酪性肉芽肿为病理特征的系统性疾病。其病因和发病机制尚不明确，但是近年来取得了不少新的认识，尤其是在遗传因素和免疫学因素方面。如人白细胞抗原HI-ADRB1基因的多形性与发病的关系，炎性趋化因子CCI-15等多种细胞因子在发病过程中所起的作用等。多种高新技术应用于诊断过程以获得病理学结果，支气管肺泡灌洗液中淋巴细胞计数和CD4／CD8比值测定对诊断和鉴别诊断的意义更加明确。治疗方面皮质类同醇及免疫抑制剂仍然是首选，但基于激素的不良反应及对病因和发病机制的新认识，一些新疗法，如抗肿瘤坏死因子a治疗等，已逐渐成为治疗快速进展和（或）难治性的结节病的一个新选择，并取得了可喜的结果。现将以上进展作一综述。

Sarcoidosis is a multisystem inflammatory disease of unknown etiology and pathogenesis, pathologically characterized by noncaseating granulomas. But many new recognitions are acquired in recent years, especially on genetic and immunological factors. For example, the relationship between human leukocyte antigen HLA DRB1 gene polymorphism and pathogenesis, the effects of chcmokines such as CCI.15 on the development of this disease. Many new techniques are used for the diagnosis process to obtain pathologic result. The significance of lymphocyte count and CD4/CD8 ratio in bronchoalveolar lavage fluid is more definite in diagnosis and differential diagnosis. The corticosteroids and immunosuppressive agents are still the first choice to treat the disease. But based on the adverse effect of corticosteroids and the new knowledge about etiology and pathogenesis,some new therapies, such as anti-tumor necrosis factor α, have become new choices on treating rapid progressing and/or refractory sarcoidosis and achieved encouraging results. This article summaries the above progress.