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Idiopathic pulmonary fibrosis: Does the time for change of diagnostic and therapeutic recommendations come?

Czech version

Authors: Martina Vašáková
Authors‘ workplace: Pneumologická klinika 1. LF UK a Thomayerovy nemocnice, Praha
Published in: Čas. Lék. čes. 2018; 157: 237-243
Category: Review Article

Overview

Idiopathic pulmonary fibrosis (IPF) belongs to the most severe and difficult-to-treat lung diseases, with median survival 2–3 years if not treated. The disease arises in genetically disposed individuals of middle and older age based on repeated alveolar injuries. Fibroproliferative healing of these lesions form the pathogenetic background of the disease, leading to the end-stage pulmonary fibrosis.

Our knowledge of pathogenesis, diagnosis and treatment of IPF increases dynamically since the beginning of new millennium. Progressive breathlessness, crepitus and clubbed fingers are typical for diagnosis of IPF. Radiologic picture of IPF in high resolution CT imaging is characterized by usual interstitial pneumonia, however nearly half of the patients do not have this typical pattern. The careful evaluation of clinical picture, radiologic findings and additional investigations as bronchoalveolar lavage differential cell count and immunologic results, by multidisciplinary team is essential in these cases. In minority of the patients lung biopsy, either surgical or cryobiopsy, is recommended.

Only last 7 years causal treatment of IPF with antifibrotic drugs is available. Nevertheless, antifibrotic treatment only slows down the disease progression. Minor part of the patients with end-stage-diseased is referred for lung transplant. Palliative and symptomatic care is an integral part of IPF treatment.

Key words:

idiopathic pulmonary fibrosis, typical and atypical radiologic findings, cryobiopsy, antifibrotic treatment, lung transplantation, palliative care

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