Clinical Investigations

Pulmonary Manifestations of Sjögren's Syndrome
Ju-Hong Shi^a, Hong-Rui Liu^b, Wen-Bing Xu^a, Rui-E. Feng^b, Zhu-Hua Zhang^c, Xin-Lun Tian^a, Yuan-Jue Zhu^a

Departments of
^aPulmonary Medicine,
^bPathology and
^cRadiology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China

Address of Corresponding Author

Respiration 2009;78:377-386 (DOI: 10.1159/000214841)

  Key Words

• Interstitial lung diseases
• Interstitial pneumonias
• Lung biopsy
• Small airway diseases
• Sjögren's syndrome

  Abstract

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. Objectives: 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.

Copyright © 2009 S. Karger AG, Basel

  Author Contacts

Ju-Hong Shi
Division of Pulmonary Medicine, Peking Union Medical College Hospital
No. 1 Suaifuyuan, Dongcheng District
Beijing 100730 (China)
Tel./Fax +86 10 6529 5004, E-Mail juhong_shi@hotmail.com

  Article Information

Received: October 15, 2008
Accepted after revision: February 19, 2009
Published online: April 22, 2009
Number of Print Pages : 10
Number of Figures : 4, Number of Tables : 3, Number of References : 47