Idiopathic pulmonary fibrosis. Can we always diagnose and treat it right?

Czech version

Authors: Martina Vašáková
Authors‘ workplace: Pneumologická klinika 1. LF UK a Thomayerovy nemocnice Praha
Published in: Vnitř Lék 2017; 63(11): 796-801
Category: Reviews

Idiopatická plicní fibróza (IPF) je závažné plicní onemocnění charakterizované progresivní fibrotizací plicní tkáně na podkladě patologického vzoru hojení alveolárních lézí. Onemocnění vzniká u jedinců středního a staršího věku, kteří nesou genetickou dispozici k fibroproliferaci. Klinický obraz není jednotný. Pacienti mohou mít rychle progredující onemocnění, případně mohou mít závažné akutní exacerbace, ale mohou mít i relativně benigní průběh nemoci. Problémy v diagnostice činí netypické radiologické obrazy nemoci a nemožnost provést plicní biopsii, případně nediagnostická plicní biopsie. Kauzální léčba této smrtící nemoci je dostupná od roku 2011, nicméně nevede k vyléčení, ale pouze ke zpomalení poklesu plicních funkcí a prodloužení přežití.

Overview

Idiopathic pulmonary fibrosis (IPF) is a severe pulmonary disease characterized by fibrotisation of lung tissue based on pathological healing of alveolar lesions. The disease is initiated in individuals of middle and older age who have a genetical disposition to fibroproliferation. Clinical presentation is not uniform. The patients can have a rapidly progressive disease, eventually severe acute exacerbations, however some of them can have relatively benign course of the disease. Problems in diagnosis are usually caused by atypical radiologic findings and no or nondiagnostic lung biopsy. Causal treatment of this life-threatening disease is available since 2011, nevertheless this treatment is not curative but only slows down declination of lung function and prolongation of survival. Thus it is important to identify the patients with IPF and start treatment early. Section for interstitial lung diseases of the Czech pneumologic and Phtiseologic Society founded the registry of idiopathic pulmonary fibrosis patients (EMPIRE), which has been then adopted by other European and Asian countries and become the biggest registry of this disease in the world. The registry can offer an answer on many questions about this disease in real world and thus is helpful for further knowledge of idiopathic pulmonary fibrosis included the atypical manifestations.

Key words:
atypical clinical and radiologic manifestation – idiopathic pulmonary fibrosis – registry EMPIRE

Sources

1. Kolek V, Kašák V, Vašáková M. Pneumologie. 2. vyd. Maxdorf: Praha 2014: 261–264. ISBN 978–80–7345–387–9.

2. Nalysnyk L, Cid-Ruzafa J, Rotella P et al. Incidence and prevalence of idiopathic pulmonary fibrosis: review of the literature. Eur Respir Rev 2012; 21(126): 355–361. Dostupné z DOI: <http://dx.doi.org/10.1183/09059180.00002512>.

3. Selman M, Pardo A. Revealing the pathogenic and aging-related mechanisms of the enigmatic idiopathic pulmonary fibrosis. An integral model. Am J Respir Crit Care Med 2014; 189(10): 1161–1172. Dostupné z DOI: <http://dx.doi.org/10.1164/rccm.201312–2221PP>.

4. Borensztajn K, Crestani B, Kolb M. Idiopathic pulmonary fibrosis: from epithelial injury to biomarkers-insights from the bench side. Respiration 2013; 86(6): 441–452. Dostupné z DOI: <http://dx.doi.org/10.1159/000357598>.

5. Raghu G, Collard HR, Egan JJ et al. On behalf of the ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosis. An official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management. Am J Respir Crit Care Med 2011; 183(6): 788–824. Dostupné z DOI: <http://dx.doi.org/10.1164/rccm.2009–040GL>.

6. Wells AU. Managing diagnostic procedures in idiopathic pulmonary fibrosis. Eur Respir Rev 2013; 22(128): 158–162. Dostupné z DOI: <http://dx.doi.org/10.1183/09059180.00001213>.

7. Gotway MB, Freemer MM, King TE Jr. Challenges in pulmonary fibrosis. The use of high resolution CT scanning of the lung for the evaluation of patients with idiopathic interstitial pneumonias. Thorax 2007; 62(6): 546–553.

8. Lynch DA, Godwin DJ, Safrin S et al. High-resolution computed tomography in idiopathic pulmonary fibrosis: diagnosis and prognosis. Am J Respir Crit Care Med 2005; 172(4): 488–493.

9. Smith M, Daluzro M, Panse P et al. Usual interstitial pneumonia-pattern fibrosis in surgical lung biopsies. Clinical, radiological and histopathological clues to aetiology. J Clin Pathol 2013; 66(10): 896–903. Dostupné z DOI: <http://dx.doi.org/10.1136/jclinpath-2013–201442>.

10. Fishbein MC. Diagnosis: to biopsy or not to biopsy: assessing the role of surgical lung biopsy in the diagnosis of idiopathic pulmonary fibrosis. Chest 2005; 128(5 Suppl 1): 520S-525S.

11. Flaherty KR, King TE, Raghu G et al. Idiopathic interstitial pneumonia. What is the effect of a multidisciplinary approach to diagnosis? Am J Respir Crit Care Med 2004; 170(8): 904–910.

12. Mura M, Porretta MA, Bargagli E et al. Predicting survival in newly diagnosed idiopathic pulmonary fibrosis: a 3-year prospective study. Eur Respir J 2012; 40(1): 101–109. Dostupné z DOI: <http://dx.doi.org/10.1183/09031936.00106011>.

13. Vašáková M et al. Moderní farmakoterapie v pneumologii. 2. ed. Maxdorf: Praha 2016: 236–243. ISBN 978–80–7345–506–4.

14. King TE Jr, Bradforf WB, Catsro-Bernardini S et al. [ASCEND Study Group]. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med 2014; 370(22): 2083–2092. Dostupné z DOI: <http://dx.doi.org/10.1056/NEJMoa1402582>.

15. Richeldi L, du Bois RM, Raghu et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med 2014; 370(22): 2071–2082. Dostupné z DOI: <http://dx.doi.org/10.1056/NEJMoa1402584>.

16. Swigris JJ, Brown KK, Make BJ et al. Pulmonary rehabilitation in idiopathic pulmonary fibrosis: A call for continued investigation. Resp Med 2008; 102(12): 1675–1680. Dostupné z DOI: <http://dx.doi.org/10.1016/j.rmed.2008.08.014>.