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The major histopathologic pattern of pulmonary fibrosis in scleroderma is nonspecific interstitial pneumonia.
Sarcoidosis Vasc Diffuse Lung Dis. 2002 Jun; 19(2):121-7.SV

Abstract

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.

Authors+Show Affiliations

Division of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan, College of Medicine, Seoul, Korea. dskim@amc.seoul.krNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Comparative Study
Journal Article

Language

eng

PubMed ID

12108451

Citation

Kim, Dong Soon, et al. "The Major Histopathologic Pattern of Pulmonary Fibrosis in Scleroderma Is Nonspecific Interstitial Pneumonia." Sarcoidosis, Vasculitis, and Diffuse Lung Diseases : Official Journal of WASOG, vol. 19, no. 2, 2002, pp. 121-7.
Kim DS, Yoo B, Lee JS, et al. The major histopathologic pattern of pulmonary fibrosis in scleroderma is nonspecific interstitial pneumonia. Sarcoidosis Vasc Diffuse Lung Dis. 2002;19(2):121-7.
Kim, D. S., Yoo, B., Lee, J. S., Kim, E. K., Lim, C. M., Lee, S. D., Koh, Y., Kim, W. S., Kim, W. D., Colby, T. V., & Kitiaichi, M. (2002). The major histopathologic pattern of pulmonary fibrosis in scleroderma is nonspecific interstitial pneumonia. Sarcoidosis, Vasculitis, and Diffuse Lung Diseases : Official Journal of WASOG, 19(2), 121-7.
Kim DS, et al. The Major Histopathologic Pattern of Pulmonary Fibrosis in Scleroderma Is Nonspecific Interstitial Pneumonia. Sarcoidosis Vasc Diffuse Lung Dis. 2002;19(2):121-7. PubMed PMID: 12108451.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - The major histopathologic pattern of pulmonary fibrosis in scleroderma is nonspecific interstitial pneumonia. AU - Kim,Dong Soon, AU - Yoo,Bin, AU - Lee,Jin Sung, AU - Kim,Eun Kyung, AU - Lim,Chae Man, AU - Lee,Sang Do, AU - Koh,Younsuck, AU - Kim,Woo Sung, AU - Kim,Won Dong, AU - Colby,Thomas V, AU - Kitiaichi,Masanori, PY - 2002/7/11/pubmed PY - 2002/12/18/medline PY - 2002/7/11/entrez SP - 121 EP - 7 JF - Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG JO - Sarcoidosis Vasc Diffuse Lung Dis VL - 19 IS - 2 N2 - 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. SN - 1124-0490 UR - https://www.unboundmedicine.com/medline/citation/12108451/The_major_histopathologic_pattern_of_pulmonary_fibrosis_in_scleroderma_is_nonspecific_interstitial_pneumonia_ L2 - http://www.diseaseinfosearch.org/result/6459 DB - PRIME DP - Unbound Medicine ER -