Bégin R, Massé S, Cantin A, Ménard H A, Bureau M A
Am J Med. 1982 May;72(5):743-50. doi: 10.1016/0002-9343(82)90539-3.
Previous investigations of airway disease in rheumatoid patients have been oriented toward establishing the prevalence of the disease, but the pathogenesis and the time course of the airflow obstruction in rheumatoid disease are still unclear. In this study, we analysed the clinical, serial pulmonary function and histopathologic data of six rheumatoid patients who had never smoked but who had airflow limitations documented repeatedly up to 10 years previously. We have attempted to characterize the site, nature and evolution of the chronic airway disease in this group of patients. Bronchiectasis was excluded in all patients by bilateral bronchography. Clinical and histopathologic evidence of the Sjörgen autoimmune exocrinopathy was documented in five of the patients, and the sixth patient had lymphoplasmocytic infiltrates of the labial glands without obstruction of the lumen or destruction. By pulmonary function tests and histopathologic examination of four open lung biopsies, the airway disease was found to be located predominantly in the peripheral airways of the lung. On each biopsy, the lesions were in different stages of activity, but on all specimens there was a definite predilection for selective bronchiolar injury. Early stage lesions were characterized by mononuclear cell infiltrates of the peribronchiolar tissue which led to deformation of airway lumen, focal mucosal extension and ulceration. Subsequently, the inflammatory reaction was replaced by fibroblastic proliferation, and in the end stage of the disease, there was complete obliteration of many bronchioles by collagenized fibroblastic tissue. From regression analyses of serial pulmonary function tests of these patients, it was concluded that (1) the airway disease in our patients who did not smoke progressed inevitably but not uniformly and (2) deterioration of pulmonary functions was more rapid in our patients than it was in the cigarette smokers who had chronic obstructive lung disease. This study also documents major dysfunctions of the chest wall mechanics which appear to contribute to the restriction of lung volumes in some rheumatoid patients.
以往对类风湿患者气道疾病的研究主要致力于确定该疾病的患病率，但类风湿疾病中气流阻塞的发病机制和病程仍不清楚。在本研究中，我们分析了6例从不吸烟但在过去10年中反复出现气流受限的类风湿患者的临床、系列肺功能和组织病理学数据。我们试图描述这组患者慢性气道疾病的部位、性质和演变。通过双侧支气管造影排除了所有患者的支气管扩张。5例患者有干燥综合征自身免疫性外分泌病的临床和组织病理学证据，第6例患者唇腺有淋巴细胞浸润，管腔未阻塞或未破坏。通过肺功能测试和4例开胸肺活检的组织病理学检查，发现气道疾病主要位于肺的外周气道。每次活检时，病变处于不同的活动阶段，但在所有标本中都有明确的选择性细支气管损伤倾向。早期病变的特征是细支气管周围组织单核细胞浸润，导致气道管腔变形、局灶性黏膜延伸和溃疡。随后，炎症反应被成纤维细胞增殖取代，在疾病末期，许多细支气管被胶原化的成纤维组织完全闭塞。通过对这些患者系列肺功能测试的回归分析得出结论：(1)我们这些不吸烟患者的气道疾病不可避免地进展，但并非均匀进展；(2)我们患者的肺功能恶化比患有慢性阻塞性肺疾病的吸烟者更快。本研究还记录了胸壁力学的主要功能障碍，这似乎导致了一些类风湿患者肺容积受限。
