A clinical episode known as an acute exacerbation (AE), which can be fatal, can occur throughout the course of idiopathic pulmonary fibrosis (IPF). Other than IPF, interstitial lung disease (ILD) has also been linked in studies to AE. Antineutrophil cytoplasmic antibody (ANCA)-associated ILD (ANCA-ILD) patients’ prevalence, clinical characteristics, AE risk factors, and leading causes of mortality, however, have not been thoroughly documented.

A retrospective analysis was done on the data of 304 IPF patients and 54 ANCA-ILD patients. In ANCA-ILD patients, researchers determined the incidence of AE, post-AE prognoses, risk factors for AE, and the leading reasons for mortality. They also contrasted the results from IPF and ANCA-ILD.

Around 84 (27.6%) IPF patients and 14 (25.9%) ANCA-ILD patients had AE. In patients with ANCA-ILD and IPF, the median survival times (MSTs) following an AE were 35.5 and 60 days, respectively (P = 0.588, log-rank test). In a multivariate analysis, serum C-reactive protein (CRP) [O.R. 2.202 (95% CI 1.037, 4.674), P < 0.01] and the percentage of projected forced vital capacity (%FVC) [O.R. 0.750 (95% CI 0.570, 0.986), P < 0.01]  were shown to be independent risk variables for AE. The most common reason for mortality in ANCA-ILD and IPF patients was AE.

Patients with ANCA-ILD may experience AE, which occurs more frequently in this condition than in IPF. The most common reason for mortality in ANCA-ILD patients was AE. In ANCA-ILD, a low %FVC and a high serum CRP level were independent predictors of AE.

Reference: resmedjournal.com/article/S0954-6111(22)00257-8/fulltext