The major histopathologic pattern of pulmonary fibrosis in scleroderma is nonspecific interstitial pneumonia

Sarcoidosis Vasc Diffuse Lung Dis. 2002 Jun;19(2):121-7.


Background: The prognosis of pulmonary fibrosis associated with scleroderma (PF-SSc) has been reported to be significantly better than that of IPF. Because the nonspecific interstitial pneumonia-pattern (NSIP), a newly defined subgroup of idiopathic interstitial pneumonias (IIP), has better prognosis than the usual interstitial pneumonia pattern (UIP), we postulated that NSIP may occur more frequently than UIP in patients with scleroderma who develop fibrosis.

Method: We reviewed the pathologic, radiologic and clinical outcomes in 19 patients with PF-SSc. Two pulmonary pathologists reclassified the histopathology of surgical lung biopsies (SLBx) and consensus diagnosis was achieved in all patients.

Results: Thirteen patients had NSIP, five had UIP, and remained one showed only nondiagnostic honeycombing. No significant difference was noted in the initial pulmonary function test (PFT), bronchoalveolar lavage (BAL) findings, or other clinical parameters between UIP and NSIP groups. Comparison of the clinical outcome of 12 patients who were followed for more than 12 months (mean: 34.5 +/- 26.0 months) suggested a better prognosis for NSIP than UIP. Five of the eight NSIP patients improved and three were stable, whereas in patients with UIP, one worsened and three were stable.

Conclusion: NSIP seems to be the major histopathologic pattern in patients with PF-SSc.

Publication types

  • Comparative Study

MeSH terms

  • Adult
  • Biopsy / methods
  • Bronchoalveolar Lavage Fluid / cytology
  • Female
  • Humans
  • Lung Diseases, Interstitial / etiology
  • Lung Diseases, Interstitial / pathology
  • Male
  • Middle Aged
  • Pulmonary Fibrosis / classification
  • Pulmonary Fibrosis / diagnostic imaging
  • Pulmonary Fibrosis / etiology
  • Pulmonary Fibrosis / pathology*
  • Radiography, Thoracic
  • Respiratory Function Tests
  • Retrospective Studies
  • Scleroderma, Systemic / complications
  • Scleroderma, Systemic / pathology*
  • Treatment Outcome