Idiopathic pulmonary fibrosis prognostic factors – analysis of the Czech registry


Authors: Martina Doubková 1;  Michal Uher 2;  Vladimír Bartoš 3;  Martina Šterclová 4;  Ladislav Lacina 5;  Vladimíra Lošťáková 6;  Ilona Binková 1;  Martina Plačková 7;  Monika Žurková 6;  Radka Bittenglová 8;  Jana Pšikalová 9;  Lenka Šišková 10;  Pavlína Lisá 11;  František Petřík 11;  Jaroslav Polák 12;  Vladimír Řihák 10;  Jana Skřičková 1;  Martina Vašáková 4
Authors‘ workplace: Klinika nemocí plicních a TBC LF MU a FN Brno 1;  Plicní oddělení Krajské nemocnice T. Bati Zlín 10;  Pneumologická klinika 2. LF UK a FN Motol v Praze 11;  Dopravní zdravotnictví a. s., Nemocnice s poliklinikou Praha Italská 12;  Institut biostatistiky a analýz LF MU Brno 2;  Plicní klinika LF UK a FN Hradec Králové 3;  Pneumologická klinika 1. LF UK a Thomayerovy nemocnice v Praze 4;  Klinika pneumologie a hrudní chirurgie Nemocnice Na Bulovce v Praze 5;  Klinika plicních nemocí a tuberkulózy LF UP a FN Olomouc 6;  Klinika plicních nemocí a tuberkulózy LF OU a FN Ostrava 7;  Klinika pneumologie a ftizeologie LF UK a FN Plzeň 8;  Pneumologicko-alergologické oddělení Kroměřížské nemocnice 9
Published in: Čas. Lék. čes. 2016; 155: 188-194
Category: Original Article

Overview

Idiopathic pulmonary fibrosis (IPF) is a rare, progressive and usually fatal form of idiopathic interstitial pneumonia. IPF is characterized by failure of alveolar re-epithelization, persistence of fibroblasts, deposition of extracellular matrix, and distortion of lung architecture, which ultimately results in respiratory failure.

We analysed 202 consecutive patients with IPF diagnosed at the Departments of Pulmonary Diseases and Tuberculosis in the Czech Republic, who they were included in the nationwide Czech IPF registry. Our aim was to determine prognostic factors of IPF and outcome of the disease.

There were 129 males and 73 females who were the median age 67 years. IPF was biopsy-proven in 66 (33 %) of patients. Median time from the first symptom to diagnosis was 12 months. Diagnosis was made in 57 patients (28.3 %) within 6 months from the onset of respiratory symptoms. 8 (4 %) patients had an acute exacerbation during the course of the disease.

In uniparametric (univariate) analysis as prognostic factors associated with poorer survival were found: higher age, higher degree dyspnea scores, clubbing fingers, comorbidities (arterial hypertension, osteoporosis), patients without histology biopsy, and bronchoalveolar increased neutrophil count. We found these positive prognostic factors: higher levels of VC (vital capacity), TLC (total lung capacity) and DLCO (diffusing capacity for carbon monooxide).

In multiparametric (multivariate) analysis as prognostic factors associated with mortality were found: higher age, higher degree of dyspnoe score. Increased lymphocytes in bronchoalveolar fluid, higher level of VC a DLCO were associated with better survival. There was no difference in survival of patients by sex, by smoking status. No significant difference in survival rates was found between IPF with and without emphysema, between the extent of fibrosis on HRCT (high resolution computed tomography) of thorax and mortality. Median survival was 51.6 months. 58 (28.7 %) patients died. The most frequent reason of dead was IPF progression with respiratory failure.

Key words:
Idiopathic pulmonary fibrosis; prognosis; treatment


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