Abstract
BackgroundPatients with fibrotic hypersensitivity pneumonitis (HP) show variable clinical courses, and some experience rapid deterioration (RD), including acute exacerbation (AE). However, little is known about AE in fibrotic HP. Here, we retrospectively examined the incidence, risk factors, and outcomes of AE in fibrotic HP.MethodsThe incidence rates of AE were calculated in 101 patients with biopsy-proven HP. AE was defined as the worsening of dyspnoea within 30 days, with new bilateral lung infiltration and no evidence of infection or other causes of dyspnoea.ResultsDuring follow-up (median: 30 months), 18 (17.8%) patients experienced AE. The 1, 3, and 5 year incidence rates of AE were 6.0, 13.6, and 22.8%, respectively. Lower diffusing capacity of the lung for carbon monoxide (DLCO) and a radiologic usual interstitial pneumonia (UIP)-like pattern were risk factors for AE. In-hospital mortality after AE was 44.4%. Median survival from diagnosis was significantly shorter in patients with AE (26.0 months) than in those with no-AE RD (55.0 months; p = 0.008) or no RD (not reached; p < 0.001). AE remained a significant predictor of all-cause mortality (hazard ratio, 8.641; 95% confidence interval, 3.388–22.040; p < 0.001) after adjustment for age, body mass index, lung function, lymphocyte levels in bronchoalveolar lavage fluid, and the presence of a UIP-like pattern.ConclusionsAE was not uncommon among patients with fibrotic HP and significantly affected prognosis. A lower DLCO value and radiologic UIP-like pattern at diagnosis were associated with the development AE in patients with fibrotic HP.
Highlights
Hypersensitivity pneumonitis (HP) is an interstitial lung disease (ILD) that results from inhalation of organic antigens in susceptible individuals [1]
In their retrospective study involving 100 patients with chronic bird fancier’s lung, Miyazaki et al found a high mortality rate following acute exacerbation (AE) (12 of 14 patients, 85.7%) [18]. These results demonstrate detrimental outcomes, it remains challenging to determine how AE affects patient prognosis in fibrotic hypersensitivity pneumonitis (HP) owing to the limited amount of data and knowledge on the incidence and risk factors of AE [15, 18]
In this study, we evaluated the incidence, risk factors, and outcomes of AE in patients with fibrotic HP
Summary
Hypersensitivity pneumonitis (HP) is an interstitial lung disease (ILD) that results from inhalation of organic antigens in susceptible individuals [1]. Four patients developed AE, which resulted in respiratory failure requiring mechanical ventilation; three of them died and one had to undergo lung transplantation In their retrospective study involving 100 patients with chronic bird fancier’s lung, Miyazaki et al found a high mortality rate following AE (12 of 14 patients, 85.7%) [18]. These results demonstrate detrimental outcomes, it remains challenging to determine how AE affects patient prognosis in fibrotic HP owing to the limited amount of data and knowledge on the incidence and risk factors of AE [15, 18]. We retrospectively examined the incidence, risk factors, and outcomes of AE in fibrotic HP
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