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Pulmonary manifestations of Sjögren's syndrome.
Respiration. 2009; 78(4):377-86.R

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.

Authors+Show Affiliations

Department of Pulmonary Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing 100730, China. juhong_shi@hotmail.comNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

19390161

Citation

Shi, Ju-Hong, et al. "Pulmonary Manifestations of Sjögren's Syndrome." Respiration; International Review of Thoracic Diseases, vol. 78, no. 4, 2009, pp. 377-86.
Shi JH, Liu HR, Xu WB, et al. Pulmonary manifestations of Sjögren's syndrome. Respiration. 2009;78(4):377-86.
Shi, J. H., Liu, H. R., Xu, W. B., Feng, R. E., Zhang, Z. H., Tian, X. L., & Zhu, Y. J. (2009). Pulmonary manifestations of Sjögren's syndrome. Respiration; International Review of Thoracic Diseases, 78(4), 377-86. https://doi.org/10.1159/000214841
Shi JH, et al. Pulmonary Manifestations of Sjögren's Syndrome. Respiration. 2009;78(4):377-86. PubMed PMID: 19390161.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Pulmonary manifestations of Sjögren's syndrome. AU - Shi,Ju-Hong, AU - Liu,Hong-Rui, AU - Xu,Wen-Bing, AU - Feng,Rui-E, AU - Zhang,Zhu-Hua, AU - Tian,Xin-Lun, AU - Zhu,Yuan-Jue, Y1 - 2009/04/22/ PY - 2008/10/15/received PY - 2009/02/19/accepted PY - 2009/4/25/entrez PY - 2009/4/25/pubmed PY - 2014/1/10/medline SP - 377 EP - 86 JF - Respiration; international review of thoracic diseases JO - Respiration VL - 78 IS - 4 N2 - 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. SN - 1423-0356 UR - https://www.unboundmedicine.com/medline/citation/19390161/Pulmonary_manifestations_of_Sjögren's_syndrome_ L2 - https://www.karger.com?DOI=10.1159/000214841 DB - PRIME DP - Unbound Medicine ER -