Interstitial lung disease in primary Sjögren's syndrome. Clinical-pathological evaluation and response to treatment

Am J Respir Crit Care Med. 1996 Sep;154(3 Pt 1):794-9. doi: 10.1164/ajrccm.154.3.8810621.


Interstitial disease is a recognized form of lung involvement in primary Sjögren's syndrome in which frequency and severity have not yet been established. We studied 20 patients 61.2 +/- 36.4 mo after the first symptoms of the syndrome that fulfilled criteria for both xerostomia and xerophtalmia. Eighteen patients exhibited pulmonary symptoms; nine presented a restrictive functional pattern. The chest roentgenogram disclosed interstitial involvement in nine patients. Gallium-67 lung scan presented hypercaptation in 15 of 19 patients studied. Thirteen patients who submitted to bronchoalveolar lavage presented higher cell counts with increases of lymphocytes and/or polymorphonuclear cells. All patients had abnormal results in at least one of the above. Lung biopsy, undertaken in 12 patients, showed a whole spectrum of interstitial disease, from a follicular bronchiolitis to a lymphoid interstitial pneumonia and finally fibrosis with honeycombing. One patient presented with associated sarcoid granulomas. We treated 11 patients with an azathioprine-based regimen and found a significant improvement in the forced vital capacity (p < 0.05) after at least 6 mo when compared with nontreated patients. We conclude that the frequency of interstitial lung disease is high in Sjögren's syndrome and that an azathioprine-based treatment can favorably change the outcome in this population.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Azathioprine / therapeutic use*
  • Bronchoalveolar Lavage Fluid / immunology
  • Female
  • Humans
  • Lung Diseases, Interstitial / complications
  • Lung Diseases, Interstitial / drug therapy*
  • Lung Diseases, Interstitial / pathology
  • Male
  • Middle Aged
  • Predictive Value of Tests
  • Respiratory Function Tests
  • Sjogren's Syndrome / complications*
  • Sjogren's Syndrome / pathology
  • Sjogren's Syndrome / physiopathology


  • Azathioprine