Pulmonary manifestations of Sjögren's syndrome.
BACKGROUND
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.


OBJECTIVE
To investigate the pathological characteristics of PSS-associated interstitial lung disease (PSS-ILD) and their relationship with HRCT lung function tests.


METHODS
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.


RESULTS
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.


CONCLUSIONS
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
Ju-Hong
JH
Department of Pulmonary Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing 100730, China. juhong_shi@hotmail.com
Liu
Hong-Rui
HR
Xu
Wen-Bing
WB
Feng
Rui-E
RE
Zhang
Zhu-Hua
ZH
Tian
Xin-Lun
XL
Zhu
Yuan-Jue
YJ
D000328:Adult; D018501:Antirheumatic Agents / Q000627:therapeutic use; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D008168:Lung / Q000473:pathology*; D017563:Lung Diseases, Interstitial / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D008297:Male; D008875:Middle Aged; D011239:Prednisolone / Q000627:therapeutic use; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D012859:Sjogren's Syndrome / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D014057:Tomography, X-Ray Computed
D018501:Antirheumatic Agents; D005938:Glucocorticoids; D003520:Cyclophosphamide; D011239:Prednisolone