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


METHODS
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.


CONCLUSIONS
NSIP seems to be the major histopathologic pattern in patients with PF-SSc.
Kim
Dong Soon
DS
Division of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan, College of Medicine, Seoul, Korea. dskim@amc.seoul.kr
Yoo
Bin
B
Lee
Jin Sung
JS
Kim
Eun Kyung
EK
Lim
Chae Man
CM
Lee
Sang Do
SD
Koh
Younsuck
Y
Kim
Woo Sung
WS
Kim
Won Dong
WD
Colby
Thomas V
TV
Kitiaichi
Masanori
M
D000328:Adult; D001706:Biopsy / Q000379:methods; D001992:Bronchoalveolar Lavage Fluid / Q000166:cytology; D005260:Female; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000209:etiology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D011658:Pulmonary Fibrosis / Q000145:classification / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology*; D013902:Radiography, Thoracic; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000150:complications / Q000473:pathology*; D016896:Treatment Outcome