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

Dong Soon Kim, Bin Yoo, Jin Sung Lee, Eun Kyung Kim, Chae Man Lim, Sang Do Lee, Younsuck Koh, Woo Sung Kim, Won Dong Kim, Thomas V. Colby, Masanori Kitiaichi

Research output: Contribution to journalArticlepeer-review

175 Scopus citations


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 pneumoniapattern (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 non-diagnostic 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.

Original languageEnglish (US)
Pages (from-to)121-127
Number of pages7
JournalSarcoidosis Vasculitis and Diffuse Lung Diseases
Issue number2
StatePublished - 2002


  • HRCT
  • Nonspecific interstitial pneumonia (NSIP)
  • Scleroderma
  • Surgical lung biopsy
  • Usual interstitial pneumonia (UIP)

ASJC Scopus subject areas

  • Internal Medicine
  • Immunology and Allergy
  • Pulmonary and Respiratory Medicine


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