Idiopathic Pulmonary Fibrosis as a Challenge for the Polish Healthcare System

• Autorzy: Marcin Kwiecień , Monika Homa , Małgorzata Budasz-Świderska , Marcin Kaczor
• Języki publikacji: EN

Abstrakty

EN

Idiopathic pulmonary fibrosis (IPF) is a rare disease characterised by age-dependent incidence, unclear aetiology and progressive course. Given the complexity of the diagnostic pathway and limited therapeutic options, and according to the fact that the Polish society is ageing, healthcare and social security expenditure are considerable and likely to rise. Until recently, there was no effective treatment for patients with IPF. After diagnosis only procedures that relieve the symptoms of the disease and treat its complications or lung transplantation were offered. In recent years a huge interest in this clinical entity has been seen. This resulted in the introduction of new drugs, whose efficacy has been confirmed in reliable clinical trials and whose slow the progression of the disease and improve survival of patients. The first such drug was pirfenidone; however such treatment is not available in Poland. Furthermore, due to the inadequacy of Polish legislative reimbursement solutions, which do not take into account the specific nature of rare disease, there are not used any favourable solution during the assessment of public funding of orphan drugs. This is contrary to European consensuses on an egalitarian approach to the use of orphan drugs. It is necessary to develop standards of care for IPF patients, including systemic solutions and the inclusion of the drugs used in the treatment of IPF on the reimbursement list. In long term this will reduce the financial burden associated with the disease, and thus reduce the direct and indirect costs resulting from its prevalence (original abstract)

Słowa kluczowe

EN

Health care system; Medications

PL

System ochrony zdrowia; Leki

• Czasopismo: Journal of Health Policy and Outcomes Research
• Rocznik: 2015
• Numer: nr 2
• Strony: 6--17

Twórcy

autor

Marcin Kwiecień

• Aestimo s.c., Kraków, Poland

autor

Monika Homa

• Aestimo s.c., Kraków, Poland

autor

Małgorzata Budasz-Świderska

• Roche Polska Sp. z o.o., Warsaw, Poland

autor

Marcin Kaczor

• Aestimo s.c., Kraków, Poland ; Uniwersytet Jegielloński Collegium Medicum, Kraków, Poland

Bibliografia

• Rowińska-Zakrzewska E, Bestry I. Idiopatyczne włóknienie płuc. Interna Szczeklika. Gajewski P. (ed.), Medycyna Praktyczna, Krakow 2015;729-734
• Orphanet Report Series. Rare disease collection. List of rare diseases and synonyms: Listed in alphabetical order. ​Available from: http://www.orpha.net/orphacom/cahiers/docs/GB/List_of_rare_diseases_in_alphabetical_order.pdf
• Demkow U. Immunopathogenesis of idiopathic pulmonary fibrosis. Pneumonol Alergol Pol. 2014; 82(1):55-60; doi: 10.5603/PiAP.2014.0009
• Wolters PJ, Collard HR, Jones KD. Pathogenesis of Idiopathic Pulmonary Fibrosis. Annu Rev Pathol. 2014; 9:157-179; doi: 10.1146/annurev-pathol-012513-104706
• Belkin A, Swigris JJ. Health-related quality of life in idiopathic pulmonary fibrosis: where are we now? Curr Opin Pulm Med. 2013; 19(5):474-9; doi: 10.1097/MCP.0b013e328363f479
• Swigris JJ, Kuschner WG, Jacobs SS, Wilson SR, Gould MK. Health-related quality of life in patients with idiopathic pulmonary fibrosis: a systematic review. Thorax. 2005; 60(7):588-94; doi:10.1136/thx.2004.035220
• Bellaye PS, Kolb M. Why do patients get idiopathic pulmonary fibrosis? Current concepts in the pathogenesis of pulmonary fibrosis. BMC Med. 2015; 13:176; doi: 10.1186/s12916-015-0412-6
• Tzouvelekis A, Bonella F, Spagnolo P. Update on therapeutic management of idiopathic pulmonary fibrosis. Ther Clin Risk Manag. 2015; 11:359-70; doi: 10.2147/TCRM.S69716. eCollection 2015
• Lee AS, Mira-Avendano I, Ryu JH, Daniels CE. The burden of idiopathic pulmonary fibrosis: an unmet public health need. Respir Med. 2014; 108(7):955-67; doi: 10.1016/j.rmed.2014.03.015
• Collard HR, Ward AJ, Lanes S, Cortney Hayflinger D, Rosenberg DM, Hunsche E. Burden of illness in idiopathic pulmonary fibrosis. J Med Econ. 2012; 15(5):829-35; doi: 10.3111/13696998.2012.680553
• European Respiratory Society. European Lung White Book. Part C. Chapter 22. Interstitial lung disease. ​Available from: http://www.erswhitebook.org/chapters/interstitial-lung-diseases/
• Sergew A, Brown KK. Advances in the treatment of idiopathic pulmonary fibrosis. Expert Opin Emerg Drugs. 2015; 20(4):1-17; doi: 10.1517/14728214.2015.1102886
• Spagnolo P, Rossi G, Cavazza A. Pathogenesis of idiopathic pulmonary fibrosis and its clinical implications. Expert Rev. Clin. Immunol. 2014; 10(8):1005-1017; doi:10.1586/1744666X.2014.91705
• Demographic profile of the elderly and consequences of the Polish population ageing in light of the forecast for 2014-2050. ​Available from: http://stat.gov.pl/obszary-tematyczne/ludnosc/ludnosc/sytuacja-demograficzna-osob-starszych-i-konsekwencje-starzenia-sie-ludnosci-polski-w-swietle-prognozy-na-lata-2014-2050,18,1.html
• Raghu G, Collard HR, Egan JJ, Martinez FJ, et al. 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; doi:10.1164/rccm.2009-040GL
• Elmufdi F, Henke CA, Perlman DM, Tomic R, Kim HJ. Novel mechanisms and treatment of idiopathic pulmonary fibrosis. Discov Med. 2015 Sep; 20(109):145-53
• Behr J, Kreuter M, Hoeper MM et al. Management of patients with idiopathic pulmonary fibrosis in clinical practice: the INSIGHTS-IPF registry. Eur Respir J. 2015; 46(1):186-96; doi: 10.1183/09031936.00217614
• Valeyre D. Towards a better diagnosis of idiopathic pulmonary fibrosis. Eur Respir Rev. 2011; 20(120):108-13; doi: 10.1183/09059180.00001611
• Spagnolo P, Tonelli R, Cocconcelli E, Stefani A, Richeldi L. Idiopathic pulmonary fibrosis: diagnostic pitfalls and therapeutic challenges. Multidiscip Respir Med. 2012; 7(1):42; doi: 10.1186/2049-6958-7-42
• Ryerson CJ, Donesky D, Pantilat SZ, Collard HR. Dyspnea in idiopathic pulmonary fibrosis: a systematic review. J Pain Symptom Manage. 2012; 43(4):771-82; doi: 10.1016/j.jpainsymman.2011.04.026
• Ziora D. Samoistne śródmiąższowe zapalenia płuc i zapalenia oskrzelików. Wielka Interna. Pulmonologia. Część II. Wyd. 1. Antczak A (ed.). Medical Tribune Polska, Warsaw 2010; 317-321
• Ley B, Harold R. Collard HR, King TE Jr. Clinical Course and Prediction of Survival in Idiopathic Pulmonary Fibrosis Am J Respir Crit Care Med. 2011; 183(4):431-40; doi: 10.1164/rccm.201006-0894CI
• Kim HJ, Perlman D, Tomic R. Natural history of idiopathic pulmonary fibrosis. Respir Med. 2015; 109(6):661-70; doi: 10.1016/j.rmed.2015.02.002
• Papla B. Śródmiąższowe idiopatyczne zapalenie płuc. Pol J Pathol 2010; 1(1):1323
• Hutchinson J, Fogarty A, Hubbard R, McKeever T. Global incidence and mortality of idiopathic pulmonary fibrosis: a systematic review. Eur Respir J. 2015; 46(3):795-806; doi: 10.1183/09031936.00185114
• Xaubet A, Ancochea J, Bollo E, Fernández-Fabrellas E, Franquet T, Molina-Molina M et al. A. Normativa sobre el diagnóstico y tratamiento de la fibrosis pulmonar idiopática. Arch. Bronconeumol. 2013; 49(8):343-353; doi:10.1016/j.arbres.2013.03.011
• Orphanet Report Series. Rare Diseases collection. Prevalence and incidence of rare diseases: Bibliographic data. Prevalence, incidence or number of published cases listed by diseases (in alphabetical order). Number 1. July 2015. ​Available from: http://www.orpha.net/orphacom/cahiers/docs/GB/Prevalence_of_rare_diseases_by_alphabetical_list.pdf
• Caminati A, Madotto F, Cesana G, Conti S, Harari S. Epidemiological studies in idiopathic pulmonary fibrosis: pitfalls in methodologies and data interpretation. Eur Respir Rev. 2015; 24(137):436-44; doi: 10.1183/16000617.0040-2015
• EMPIRE (European MultiPartner IPF Registry). ​Available from: http://empire.registry.cz/
• American Thoracic Society. Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. American Thoracic Society (ATS), and the European Respiratory Society (ERS). Am J Respir Crit Care Med. 2000; 161(2 Pt 1):646-64
• Behr J. Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis. N. Engl. J. Med. 2012; 367(9):869; author reply 870-871; doi:10.1056/NEJMc1207471

Identyfikatory

DOI

10.7365/JHPOR.2015.2.1