Pulmonary manifestations of Sjögren's syndrome.

Primary Sjögren's syndrome (PSS) is associated with various histological patterns of interstitial lung disease. Although chest images and lung function studies showed that lung involvement predominantly occurs in small airways, pathological findings were not consistent with the results of high-resolution CT (HRCT) and lung function tests. To investigate the pathological characteristics of PSS-associated interstitial lung disease (PSS-ILD) and their relationship with HRCT lung function tests. Fourteen patients diagnosed as PSS who underwent surgical lung biopsy in Peking Union Medical College Hospital from October 2000 to October 2006 were reviewed. Histopathologic findings, radiologic findings and lung function tests were analyzed. The study included 13 women. The median age was 46 years. Most patients presented with dyspnea and cough. CT scans revealed bilateral ground-glass, consolidative, reticular and nodular opacities and cyst lesions. The histological patterns included nonspecific interstitial pneumonia (NSIP) cellular pattern associated with organizing pneumonia (OP), NSIP mixed pattern associated with OP, noncaseating granulomas, chronic bronchiolitis, follicular bronchiolitis, constrictive bronchiolitis, lymphocytic interstitial pneumonia associated with follicular bronchiolitis, NSIP mixed pattern associated with follicular bronchiolitis, NSIP mixed pattern coexisting with chronic bronchiolitis, OP associated with chronic bronchiolitis, and noncaseating granulomas coexisting with OP. Treatment included prednisone and cyclophosphamide. During the follow-up period (median 38 months), most patients improved or remained stable. The patient with constrictive bronchiolitis died from progression of primary disease. The histopathologic patterns of PSS-ILD included lung interstitial involvement and small airway involvement or both. Corticosteroid therapy combined with cyclophosphamide was administered with a favorable response in the majority of patients.

Shi JH ，Liu HR ，Xu WB ，Feng RE ，Zhang ZH ，Tian XL ，Zhu YJ ... -  《-》

Interstitial lung disease in primary Sjögren syndrome.

Parambil JG ，Myers JL ，Lindell RM ，Matteson EL ，Ryu JH ... -  《CHEST》

A retrospective analysis of distinguishing features of chest HRCT and clinical manifestation in primary Sjögren's syndrome-related interstitial lung disease in a Chinese population.

Dong X ，Zhou J ，Guo X ，Li Y ，Xu Y ，Fu Q ，Lu Y ，Zheng Y ... -  《-》

Pulmonary manifestations of primary Sjögren's syndrome.

Sarkar PK ，Patel N ，Furie RA ，Talwar A ... -  《-》

Pulmonary manifestations of primary Sjogren's syndrome: a clinical, radiologic, and pathologic study.

Ito I ，Nagai S ，Kitaichi M ，Nicholson AG ，Johkoh T ，Noma S ，Kim DS ，Handa T ，Izumi T ，Mishima M ... -  《AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE》