Idiopathic Pulmonary Fibrosis (IPF)―Common Practice in Poland before the “Antifibrotic Drugs Era”

Abstract

Introduction: Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive and debilitating lung disease with a median survival time of 3-5 years. For now, pirfenidone (PIR) and nintedanib (NTB) are the only drugs that can slow down the disease's progression. In Poland, these drugs, although registered for legal use, had not been reimbursed for IPF patients until the end of the year 2016. Aim of the study was to assess what was common practice in terms of diagnosis and treatment in the period before antifibrotic drugs became available for IPF patients in Poland. Material and methods:We performed a survey among participants of two nationwide pulmonological congresses held in 2016. Results: One hundred and fifty physicians took part in the study. Only 55% of respondents would reach their final diagnosis in collaboration with a radiologist. Just 40% of those sending patients for surgical lung biopsy (SLB) would discuss the case directly with a pathologist. 22% would never refer the patient suspected of having IPF for SLB. 85% believed that bronchoalveolar lavage (BAL) may be useful for diagnosis. 41% of respiratory professionals would not use any drug for the treatment of IPF patients. 23% of physicians would prescribe corticosteroids in high doses (CS), either in monotherapy or in combination with other drugs. Only 43% of respondents would use antacid drugs in case of symptomatic gastro-oesophageal reflux disease (GERD), and only 11% would prescribe these drugs regardless of GERD diagnosis. Conclusions: The majority of Polish pulmonologists were not supported by radiologists and pathologists in the diagnostic process. Treatment standards were unsatisfactory, mostly due to a lacking of reimbursement regulations. Further education is necessary to improve management of IPF patients in Poland.

Diagnostic and Treatment Standards in Idiopathic Pulmonary Fibrosis in the Era of Antifibrotic Drugs in Poland: A Real-World Practice Survey

“…The first part of our survey evaluating diagnostic standards confirmed that the clinical workup of Polish professionals for IPF diagnosis has only slightly changed over that time. Compared to the previous survey in which almost 30% of the respondents agreed that BAL is necessary in all cases in the differential diagnosis of IPF [7], in the actual one, only 17% of physicians agreed to the same opinion. The majority of respondents (74%) use BAL only in selected cases of patients.…”

“…The present real-world data survey on the clinical practice standards in IPF diagnosis and treatment in the era of access to the antifibrotic therapy for Polish patients with IPF is a follow-up to the previous survey on the same topic undertaken in 2016, before the availability of antifibrotics in Poland [7]. During two years in between both surveys, the situation has changed for Polish patients with IPF in regards to the access to effective pharmacological therapy.…”

“…Despite the availability of effective pharmacological treatment options for IPF patients in Poland, local evidenced-based diagnostic and therapeutic standards established for Polish IPF patients with regards to the specificity of the national health service system and specific patients' needs and expectations are lacking. The previous survey on common practices of the Polish physicians in the management of IPF, undertaken before the availability of antifibrotic therapy in Poland, pointed out unsatisfactory local diagnostic and therapeutic standards in IPF [7].…”

Guidelines of the Polish Respiratory Society for Diagnosis and Treatment of Idiopathic Pulmonary Fibrosis

“…Many publications recommend extending the team, if possible, to include a rheumatologist, thoracic surgeon (who should take part in assessing indications for SLB and choosing an optimum biopsy site), occupational medicine specialist (potential environmental exposure), cardiologist and other specialists depending on comorbidities, psychologist, ILD nurse as well as palliative care specialist. In spite of unequivocal international guidelines, a survey conducted among Polish pulmonologists before antifibrotic agents were commonly available demonstrated that only 55% of respondents establish IPF diagnosis in cooperation with a radiologist and only 40% of diagnoses in patients following lung biopsy were discussed directly with a pathologist [58]. The final diagnosis should be clearly formulated.…”

“…For instance, we currently have access to unequivocal data documenting harmful effects of glucocorticosteroids and immunosuppressants [67]. Despite this fact, the aforementioned survey demonstrated that in 2016 23% of IPF patients in Poland received glucocorticosteroids [58]. Negative results were obtained also in the ACE-IPF study, which demonstrated detrimental effects of warfarin use [68] in spite of encouraging results from earlier studies [69].…”