Abstract
AIMS: Idiopathic pulmonary fibrosis (IPF) is a severe lung disease with mean survival of 2.5 years. Delay in diagnosis and treatment probably decreases a survival in IPF. Our study aims to investigate influence of time from the first symptoms to diagnosis on the survival.
METHODS: The study was retrospective using data from EMPIRE registry (European Multipartner IPF registry). 566 patients were stratified according to duration of symptoms preceding the diagnosis of IPF: in earlier diagnosed group (ED) the diagnosis was stated within 1 year from onset of symptoms (383 patients), and in the later diagnosed (LD) group symptoms lasted longer than 1 year (183 patients). The survival from the diagnosis up to 84 months was evaluated by Kaplan-Meier curve and statistical significance tested by Log Rank test. The vital capacity (VC) initial values and VC decline in 6, 12, and 18 months of follow-up were evaluated and compared between both groups using t-test. P value less than 0.05 was considered statistically significant.
RESULTS: The Kaplan-Meier survival curves showed better survival in ED compared to LD patients. The median survival in ED was 63.1 and in the LD 43.9 months (p=0.018). VC at the time of diagnosis was higher in ED (82.9% of PV in ED vs 75.8% of PV in LD; p=0.008). There was no difference in rate of VC decline.
CONCLUSIONS: Our results show that earlier diagnosis and treatment may increase survival in IPF. Early diagnosis and treatment does matter in prognosis of IPF patients and thus maximal effort should be generated to get the patients diagnosed and treated as early as possible.
The study was supported by grant NT 13433-4/2012, IGA MZCR.
- Copyright ©the authors 2016
