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Clinically Significant Interstitial Lung Disease in Limited Scleroderma: Histopathology, Clinical Features, and Survival

Abstract

Purpose: To evaluate the pathologic patterns, clinical features, and survival among subjects with scleroderma and clinically significant interstitial lung disease (SSc-ILD) evaluated at an ILD center.

Methods: Retrospective cohort study of all SSc patients referred for further evaluation of ILD who underwent surgical lung biopsy. Clinical data were abstracted by review of the medical record and lung biopsies were reviewed and classified according to current pathologic criteria.

Results: All patients presented with significant respiratory symptoms. Twenty-two of 27 subjects had surgical lung biopsy-proven ILD, and 5 had miscellaneous non-ILD patterns. Of those with ILD, 64% (14/22) had a non-specific interstitial pneumonia (NSIP) pathologic pattern (13 fibrotic NSIP, 1 cellular NSIP), and 36% (8/22) had usual interstitial pneumonia (UIP) pattern. Subjects with NSIP were younger (median age 42 yrs vs. 58 yrs; p = 0.003), but no differences in pulmonary physiology (ppFVC in the NSIP group was 52 vs. 65 in the UIP group; p = 0.22; ppDLCO 40 vs. 42; p = 1.0) were noted. All patients had limited skin involvement. The Scl-70 antibody was absent among those assessed (0/10 NSIP and 0/7 UIP). All patients were treated with cytotoxic therapy. Median survival for those with NSIP was 15.3 years (5596 days) compared with 3 years (1084 days) for those with UIP (log-rank test, p=0.07).

Conclusions: In SSc patients with limited cutaneous disease and clinically significant ILD, fibrotic NSIP and UIP are the predominant pathologic patterns. Those with UIP pattern disease had a trend towards shorter survival.

Key Words: Systemic sclerosis • scleroderma • interstitial lung disease • histopathology • survival