Pulmonary manifestations of primary Sjogren's syndrome: a clinical, radiologic, and pathologic study

Am J Respir Crit Care Med. 2005 Mar 15;171(6):632-8. doi: 10.1164/rccm.200403-417OC. Epub 2004 Dec 3.


Rationale: Clinicopathologic pulmonary manifestations associated with primary Sjogren's syndrome have yet to be reviewed in a large series since the recognition of nonspecific interstitial pneumonia (NSIP) as a distinct histologic pattern.

Objectives: To determine clinical presentations, high-resolution computed tomographic (HRCT) and histologic findings of the lung disease associated with primary Sjogren's syndrome in the light of NSIP, and to analyze prognosis of the disease.

Methods: On the basis of 33 cases (31 surgical lung biopsies and 2 autopsies) collected consecutively from multiple centers, we have retrospectively evaluated clinical, radiologic, and pathologic manifestations of the disease. Prognostic factors were identified by univariate and multivariate analysis.

Measurements and main results: We found that NSIP was the most frequently seen histologic pattern (20 of 33 cases [61%], 19 fibrosing and 1 cellular). Bronchiolar diseases and amyloid and malignant lymphoma were seen less frequently. HRCT-pathologic correlation resulted in a 94% positive predictive value of CT-NSIP pattern for pathologic diagnosis of NSIP, whereas the diagnostic value of HRCT was low (15%) with an HRCT pattern other than NSIP, data that may influence the decision to biopsy. The 5-year survival rate was 84% overall and 83% in patients with NSIP. Multivariate analysis on all patients showed that low Pa(O(2)) (p = 0.02) and presence of microscopic honeycombing (p = 0.04) were independently associated with survival. Patients with NSIP showed lower vital capacity (mean +/- SD: 68.5 +/- 16.6%pred) than patients without NSIP (92.5 +/- 18.6%pred; p < 0.001).

Conclusion: Among a diversity of pulmonary lesions in primary Sjogren's syndrome, NSIP was the commonest histologic pattern and had a favorable prognosis.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Biopsy
  • Female
  • Humans
  • Lung / diagnostic imaging*
  • Lung / pathology
  • Lung Diseases, Interstitial / diagnostic imaging
  • Lung Diseases, Interstitial / etiology*
  • Lung Diseases, Interstitial / pathology
  • Male
  • Middle Aged
  • Multivariate Analysis
  • Predictive Value of Tests
  • Proportional Hazards Models
  • Retrospective Studies
  • Sjogren's Syndrome / complications*
  • Survival Analysis
  • Survival Rate
  • Time Factors
  • Tomography, X-Ray Computed