Tillie-Leblond I, Crestani B, Perez T, Nunes H
Service de Pneumologie et D'immuno-Allergologie, Hôpital Calmette, Institut Pasteur de Lille, Université de Lille II et CHRU, France.
Rev Mal Respir. 2012 Dec;29(10):1254-63. doi: 10.1016/j.rmr.2012.10.020. Epub 2012 Nov 22.
The association of inflammatory involvement of the distal airways or bronchiolitis and systemic diseases is essentially observed in Sjögren's syndrome, rheumatoid arthritis and chronic inflammatory bowel disease. Bronchiolitis may be mainly cellular in nature, often involving lympho-monocytic cells, and sometimes associated with lymphoid follicles, as in Sjögren's syndrome. It may also, particularly in rheumatoid arthritis, be constrictive, with peribronchiolar fibrosis. This type is associated with a worse prognosis, with possible progression to chronic respiratory insufficiency. The diagnosis of bronchiolitis should be suspected in any atypical form of asthma, or recurrent "bronchitis", and it is essential to look for extrarespiratory symptoms and auto-antibodies to establish the diagnose of systemic disease. The CT appearances coupled with the evaluation of pulmonary function parameters usually lead to the diagnosis. In severe and/or rapidly progressive cases treatment-combining corticosteroids with immunosuppressive drugs may be prescribed, but often with disappointing results. In these cases, lung transplantation should be considered in young patients.
远端气道炎症累及或细支气管炎与全身性疾病的关联主要见于干燥综合征、类风湿关节炎和慢性炎症性肠病。细支气管炎本质上可能主要是细胞性的，常累及淋巴细胞，有时与淋巴滤泡有关，如在干燥综合征中。在类风湿关节炎中，它也可能是缩窄性的，伴有细支气管周围纤维化。这种类型预后较差，可能进展为慢性呼吸功能不全。对于任何非典型形式的哮喘或复发性“支气管炎”，都应怀疑细支气管炎的诊断，寻找呼吸外症状和自身抗体对于确诊全身性疾病至关重要。CT表现结合肺功能参数评估通常可得出诊断。在严重和/或快速进展的病例中，可能会开具皮质类固醇与免疫抑制药物联合治疗的处方，但结果往往令人失望。在这些情况下，对于年轻患者应考虑肺移植。
