Clinical features of interstitial lung disease in patients with primary Sj(o)gren's syndrome / 中华风湿病学杂志

ABSTRACT

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