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Pulmonary manifestations of Sjögren's syndrome
Respiration, 04/27/09

Shi JH et al. – Study reports that the histopathologic patterns of primary Sjögren's syndrome-associated interstitial lung disease (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.

Methods

Investigation of the pathological characteristics of PSS-ILD and their relationship with HRCT lung function tests
14 pts diagnosed as PSS who underwent surgical lung biopsy
Histopathologic findings, radiologic findings and lung function tests were analyzed

Results

Study included 13 women; median age was 46 yrs
Most pts 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 f/u period (median 38 mo), most pts improved or remained stable
The patient with constrictive bronchiolitis died from progression of primary disease