Efficacy of concurrent treatments in idiopathic pulmonary fibrosis patients with a rapid progression of respiratory failure: an analysis of a national administrative database in Japan

• Published in: BMC pulmonary medicine Vol. 16; no. 1; p. 91
• Main Authors: Oda, Keishi, Yatera, Kazuhiro, Fujino, Yoshihisa, Ishimoto, Hiroshi, Nakao, Hiroyuki, Hanaka, Tetsuya, Ogoshi, Takaaki, Kido, Takashi, Fushimi, Kiyohide, Matsuda, Shinya, Mukae, Hiroshi
• Format: Journal Article
• Language: English
• Published: England BioMed Central Ltd 08.06.2016; BioMed Central
• Subjects: Adrenal Cortex Hormones - administration & dosage; Age; Aged; Aged, 80 and over; Artificial respiration; Bronchoscopy; Care and treatment; Classification; Co-trimoxazole; Confidence intervals; Consent; Corticosteroids; Data analysis; Databases, Factual; Disease Progression; Dosage and administration; Ethics; Female; Hospitals; Humans; Idiopathic Pulmonary Fibrosis - complications; Intensive Care Units; Japan; Kaplan-Meier Estimate; Logistic Models; Macrolides - administration & dosage; Male; Medical prognosis; Middle Aged; Mortality; Multivariate Analysis; Patient outcomes; Patients; Prognosis; Pulmonary fibrosis; Pulmonology; Respiration, Artificial; Respiratory Insufficiency - mortality; Respiratory Insufficiency - therapy; Retrospective Studies; Studies; Substance abuse treatment; Survival Rate; Treatment Outcome; Trimethoprim, Sulfamethoxazole Drug Combination - administration & dosage; Ventilators; Japan; Corticosteroid; Co-trimoxazole; Acute exacerbation of idiopathic pulmonary fibrosis; Acute respiratory failure; Nationwide database; Macrolide; Mechanical ventilation
• ISSN: 1471-2466; 1471-2466
• DOI: 10.1186/s12890-016-0253-x

Some IPF patients show a rapid progression of respiratory failure. Most patients are treated with high-dose corticosteroids. However, no large clinical studies have investigated the prognosis or efficacy of combined treatments including high-dose corticosteroids in IPF patients with a rapid progression of respiratory failure. We enrolled IPF patients who received mechanical ventilation and high-dose corticosteroids between April 2010 and March 2013. Records were extracted from a Japanese nationwide inpatient database. We conducted a retrospective epidemiologic and prognostic analysis. Two hundred nine patients receiving an average of 12.8 days of ventilatory support were enrolled. There were 138 (66 %) fatal cases; the median survival was 21 days. The short-term (within 30 days) and long-term (within 90 days) survival rates were 44.6 and 24.6 %, respectively. The average monthly admission rate among the IPF patients with the rapid progression of respiratory failure in the winter was significantly higher than that in spring (p = 0.018). Survival did not differ to a statistically significant extent in the different geographic areas of Japan. Survivors were significantly younger (p = 0.002) with higher rates of mild dyspnea on admission (p = 0.012), they more frequently underwent bronchoscopy (p < 0.001), and received anticoagulants (p = 0.027), co-trimoxazole (p < 0.001) and macrolide (p = 0.02) more frequently than non-survivors. A multivariate logistic analysis demonstrated that two factors were significantly associated with a poor prognosis: >80 years of age (OR = 2.94, 95 % Cl 1.044-8.303; p = 0.041) and the intravenous administration of high-dose cyclophosphamide (OR = 3.17, 95 % Cl 1.101-9.148; p = 0.033). Undergoing bronchoscopy during intubation (OR = 0.25, 95 % Cl 0.079-0.798; p = 0.019) and the administration of co-trimoxazole (OR = 0.28, 95 % Cl 0.132-0.607; p = 0.001) and macrolides (OR = 0.37, 95 % Cl 0.155-0.867; p = 0.033) were significantly associated with a good prognosis. The dosage of co-trimoxazole significantly correlated with survival. Co-trimoxazole and macrolides may be a good addition to high-dose corticosteroids in the treatment of IPF patients with a rapid progression of respiratory failure.