Loddenkemper R
Inneren Abteilung, Lungenklinik Heckeshorn des Krankenhauses Zehlendorf, Berlin.
Z Gesamte Inn Med. 1990 Oct 1;45(19):578-80.
Pulmonary histiocytosis X is a histiocytic granulomatosis of yet unknown etiology and of partial immunopathogenesis. The incidence ist about 5% compared to sarcoidosis. Almost all patients are smokers, the age peak lies between 20 and 40 years. Leading symptoms are non-productive cough and dyspnea, often it is only detected radiologically by chance. The x-ray shows bilateral nodular, later reticular changes. The characteristic ring figures may be detected only by tomography. Until recently diagnosis was made exclusively by lung biopsy, but now bronchoalveolar lavage may be already diagnostic. Early corticosteroid therapy seems to prevent the progression to the fibrotic-bullous end-stage in almost all cases.
肺组织细胞增多症X是一种病因不明且部分为免疫发病机制的组织细胞肉芽肿病。与结节病相比,其发病率约为5%。几乎所有患者都是吸烟者,发病年龄高峰在20至40岁之间。主要症状为干咳和呼吸困难,通常仅通过放射学检查偶然发现。X线显示双侧结节状改变,后期为网状改变。特征性的环状影仅能通过体层摄影术检测到。直到最近,诊断还完全依靠肺活检,但现在支气管肺泡灌洗可能已具有诊断价值。早期使用皮质类固醇治疗似乎在几乎所有病例中都能防止病情发展到纤维化-大疱性终末期。