Interstitial lung disease in primary Sjögren syndrome
Joseph G Parambil1, Jeffrey L Myers, Rebecca M Lindell
1Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN 55905, USA.
Chest
|November 14, 2006
Summary
Primary Sjögren syndrome (pSS) can cause various lung conditions. Usual interstitial pneumonia (UIP) patterns often worsen, and acute exacerbations can be fatal in patients with pSS-associated interstitial lung disease (ILD).
Area of Science:
- Pulmonary Medicine
- Rheumatology
- Pathology
Background:
- Primary Sjögren syndrome (pSS) is linked to diverse interstitial lung disease (ILD) histological findings.
- Understanding these patterns is crucial for managing pSS patients with lung involvement.
Purpose of the Study:
- To analyze the histopathologic patterns of ILD in patients with pSS.
- To correlate these findings with clinical presentation, imaging, and patient outcomes.
Main Methods:
- Retrospective review of 18 patients with pSS and suspected ILD who underwent lung biopsy.
- Analysis of histopathologic findings, radiologic features (chest X-ray, HRCT), and clinical outcomes.
Main Results:
- Common histopathologic patterns included nonspecific interstitial pneumonia (NSIP), organizing pneumonia (OP), usual interstitial pneumonia (UIP), lymphocytic interstitial pneumonia, pulmonary lymphoma, and amyloidosis.
- Patients with UIP histology showed a tendency for disease progression.
- Seven patients (39%) died, with three deaths attributed to acute exacerbation of interstitial pneumonia.
Conclusions:
- A wide spectrum of histological patterns can manifest as ILD in pSS.
- UIP is associated with a poorer prognosis in pSS-associated ILD.
- Acute exacerbations represent a significant cause of mortality in this patient group.
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