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No large-scale studies have reported on the clinical course of acute exacerbation of fibrosing interstitial lung disease (AE-FILD). We aimed to elucidate the clinical course of AE-FILD. We retrospectively included patients admitted with AE-FILD diagnosis. The clinical courses of AE-FILD were classified as consistent improvement after response to treatment (responder group); no response to treatment (non-responder group); improvement after treatment followed by worsening respiratory status not attributable to AE-FILD (complication group); and improvement after treatment followed by worsening respiratory status attributable to AE-FILD relapse (second-attack group). Among 340 patients, 123 died within 90 days, 265 responded, and 75 were refractory to initial treatment. Among patients who responded to treatment, 191, 26, 46, and 2 belonged to the responder, complication, second-attack, and unknown groups, respectively. Multivariate analysis revealed that high lactate dehydrogenase, high C-reactive protein, and low PaO2/FiO2 were associated with 90-day mortality (odds ratio [OR] 2.27, p = 0.002; OR 2.01, p = 0.008; and OR 1.70, p = 0.042, respectively). Of patients who responded to treatment, a second attack was strongly associated with 90-day mortality (OR 18.54, p |
