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Long‐term clinical course and outcome of interstitial pneumonia with autoimmune features
Author(s) -
Kim Ho Cheol,
Lee Joo Hee,
Chae Eun Jin,
Song Joon Seon,
Song Jin Woo
Publication year - 2020
Publication title -
respirology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.857
H-Index - 85
eISSN - 1440-1843
pISSN - 1323-7799
DOI - 10.1111/resp.13665
Subject(s) - medicine , idiopathic interstitial pneumonia , lung , interstitial lung disease
Background and objective Idiopathic interstitial pneumonia (IIP) with autoimmune features that does not fulfil connective tissue disease (CTD) criteria has been recently defined as interstitial pneumonia with autoimmune features (IPAF). However, its long‐term clinical course and outcome are poorly understood. Methods We included consecutive patients diagnosed with IIP ( n = 586) or CTD‐related interstitial lung disease (CTD‐ILD, n = 149). Some patients with IIP were reclassified as IPAF based on recent guidelines. Results The median follow‐up period was 45 months. Among the IIP patients, 109 (18.6%) were reclassified as IPAF. Compared to the non‐IPAF‐IIP group, the IPAF group had slower diffusing capacity of the lung for carbon monoxide (DL CO ) and total lung capacity declines, and more frequent CTD development during follow‐up periods. The prognosis of the IPAF was better than that of the non‐IPAF‐IIP and similar to that of the CTD‐ILD. IPAF was associated with better prognosis in the IIP cohort on univariate but not on multivariate analysis. Usual interstitial pneumonia (UIP) pattern, old age and low DL CO independently predicted mortality in the IPAF group. Conclusion Compared to the non‐IPAF‐IIP group, the IPAF group had slower lung function declines and more frequent CTD development during follow‐up. Although the prognosis of IPAF group was better than that of non‐IPAF‐IIP group and similar to that of CTD‐ILD group, it showed poor prognosis in patients with old age, UIP pattern, and low DL CO .