原发性干燥综合征继发肺间质病变的临床特点

Clinical features of interstitial lung disease in patients with primary Sj（o）gren＇s syndrome

目的 探讨原发性干燥综合征（pSS）继发肺间质病变（ILD）的临床、实验室及病理特点.方法 收集我院2006-2008年住院的15例pSS-ILD的临床资料,与同期住院的18例pSS无ILD进行比较.其中8例pSS-ILD有随访,观察治疗后胸部高分辨CT（HRCT）的变化情况.结果①pSS-ILD的发病年龄[（57±11）岁]比pSS无ILD[（43±11）岁]晚（P〈0.01）.6例 pSS-ILD患者首发症状为咳嗽、咯痰、胸闷和（或）活动后气促,出现呼吸道临床表现、心脏受累及肺动脉高压（PAH）较pSS无ILD明显增多（P〈0.01）.②pSS-ILD血清免疫球蛋白G（IgG）水平[（23±8）g/L]比pSS无ILD[（17±5）g/L]明显增高（P〈0.01）.③pSS-ILD胸部HRCT常见网格状、条索状、结节状影,8例胸膜受累,蜂窝状改变常见（5例）,3例出现PAH.8例患者治疗6个月,4例胸部HRCT吸收好转,2例无明显变化,2例病变进展.④病理常见肺泡壁及肺泡组织局灶性纤维化,肺泡腔内有炎细胞浸润,间质多灶性淋巴细胞浸润,1例患者符合非特异性间质性肺炎（NSIP）改变.结论 pSS-ILD的发病年龄较大,易出现呼吸道临床表现,心脏受累及继发PAH,血清IgG水平高.1/3患者HRCT可见蜂窝状改变,多数患者治疗后病变吸收好转.病理可见NSIP改变.

Objective To explore the clinical,laboratory,and pathologic manifestations of the interstitial lung disease（ILD）associated with primary Sj（o）gren＇s syndrome（pSS,pSS-ILD）.Methods Clinical data of 15 patients with pSS-ILD admitted to our hospital from 2006 to 2008 were collected and the different features were compared with 18 patients with pSS without ILD.Eight patients with pSS-ILD were followed up and observed the changes of high-resolution computed tomography （HRCT）.Results The age at onset was later in pSS-ILD[（57±11）years]than in pSS without ILD[（43±11）years]（P〈0.01）.The initial symptoms in six of patients with pSS-ILD were cough,expectoration,chest distress or dyspnea upon exertion.The respiratory clinical manifestations,circulationsystem involvement and pulmonary artery hypertension （PAH） were more common in pSS-ILD than in pSS without ILD（P〈0.01）.Compared with patients with pSS without ILD,patients with pSS-ILD had significantly higher serum IgG level（P〈0.01）.The common findings of HRCT included reticular changes,irregular linear hyper-attenuating areas,and nodules.Pleural involvement was found in 8 patients,honeycomb change in 5 patients and PAH in 3 patients.After treated for 6 months of 8 patients,radiological findings improved in 4 patients,stable in 2 patients,and worse in 2 patients.The comlnon histological findings included focal fibrosis in alveolar wall and alveolus with and alveolar space inflammatory cell infiltration,interstitial inflammation with mulifocal lymphocyte infiltration.One patient had the pathological changes of nonspecific interstitial pneumonia（NSIP）.Conclusion The age at onset of pSS-ILD is late and tends to produce respiratory symptoms and prone to have circulationsystem involvement such as PAH and elevated serum IgG level.Honeycomb change in HRCT can be seen in one third of patients and most patients can improve after treatment,however.NSIP can be observed in histopathologieal