Longitudinal observation of idiopathic pulmonary fibrosis (IPF) patients cohort from one center in Poland

Abstract

<b>Introduction:</b> IPF is a devastating disease with bad prognosis. Disease course is variable and it is difficult to predict which patient will progress rapidly. Several factors are known to predict morbidity and mortality in IPF patients. <b>Aim:</b> To evaluate the cohort of IPF patients followed up in department of respiratory diseases in years 2007 to 2017 regarding GAP index, pulmonary function tests, 6 – minute – walking test distance – saturation product, nt-proBNP serum concentration, and find out the factors predicting mortality. <b>Results:</b> The study group consisted of 102 subjects, 66% were male. Only patients with diagnosis of IPF and no therapeutic intervention were analyzed. Median age at diagnosis was 65 years. Median follow up was 1089 days. 77% had typical UIP pattern on HRCT, 15% had surgical lung biopsy. At baseline, 55 patients were in GAP stage 1, 29 – GAP 2, 9 – GAP 3. 63 patients died during the follow up time. Patients, who died, had lower baseline FVC % pred. (82.6 vs. 90.3, p=0.03), DLco % pred. (38.7 vs. 60.6, p=0.001), distance / saturation product in 6MWT (p=0.05) and higher serum concentration of nt-proBNP (217.5 vs. 81 pg/ml, p=0.009). In the subset of patients with two or more PFT available we analyzed the median annual decrease of FVC% pred., which was 1.13% for survivors and 2.36% for deceased (p=0.14). At baseline, 83% of patients would fulfill inclusion criteria for antifibrotic treatment under current regulations in Poland. During observational period the percentage felt to 62%. <b>Conclusion:</b> Lower baseline FVC, DLco and distance/saturation product and higher concentration of nt-proBNP predicted mortality in IPF patients observed without any treatment.