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

Abstract

Introduction: Currently, only two drugs have been shown to modify the inevitable natural history of idiopathic pulmonary fibrosis (IPF). Changes in the reimbursement policy for antifibrotic drugs in Poland have led to the availability of pirfenidone from January 2017 and nintedanib from March 2018 for the treatment of Polish patients with IPF. This study aimed to evaluate the possible changes and shortcomings in the clinical practice standards in IPF in the era of access to antifibrotic therapy in Poland. Material and methods: A real-world data survey was performed among physicians attending the Polish Respiratory Society Congress held in May 2018. The present survey was a follow-up to the previous survey undertaken in 2016, before the availability of antifibrotics in Poland. Results: A total of 99 physicians participated in the survey, among which 80% were pulmonologists. The majority of participants (83%) represented hospital-based clinicians and most of them (93%) were involved in interstitial lung diseases (ILD) management. As many as 63% of the respondents elaborate the final diagnosis of IPF working with the expert radiologist routinely, 47% do that in the cooperation with other pulmonologists, and if a biopsy was performed 39% discuss its results with the expert pathologist. Bronchoalveolar lavage (BAL) and surgical lung biopsy (SLB) would never be recommended in the differential diagnosis of IPF by 9% and 16% of the respondents, respectively. Corticosteroids (CS) or a combination of CS and immunosuppressants (IS) is still recommended by 22% of participants. Proton pump inhibitors (PPI) in the case of symptomatic GERD are prescribed by 44% of the respondents, and 12% prescribe PPI regardless of GERD symptoms. Pirfenidone is used by 70%, and nintedanib by 48% of the respondents. Only 39% of the respondents refer patients with IPF to professional rehabilitation centers. Conclusions: The level of cooperation between pulmonologists and other specialists in the diagnostic workup of IPF is unsatisfactory. IPF treatment practices in the era of access to effective drugs in Poland require immediate improvement. There is an urgent need to develop the local Polish practical guidelines to improve the management of IPF.

A multicentre retrospective observational study on Polish experience of pirfenidone therapy in patients with idiopathic pulmonary fibrosis: the PolExPIR study

“…Recently, changes in the reimbursement policy for antifibrotics in Poland have led to the wider availability of pirfenidone since January 2017 for the treatment of Polish patients with IPF [34]. The drug has become available in the frame of therapeutic program refunded by the NHF for patients with the mild-to-moderate disease based on the inclusion criteria similar to those used for the phase III pirfenidone RCT, namely the ASCEND study [5].…”

Double Lung Transplantation for Idiopathic Pulmonary Fibrosis in a Patient with a History of Liver Transplantation and Prolonged Journey for Disease-Specific Antifibrotic Therapy

“…Both national Polish and European findings confirm the urgent need for improvement in the area of ILD diagnosis. Multidisciplinary discussion (MDD), involving at minimum a pulmonologist and a radiologist with expertise in the differential diagnosis of ILD, is required to ensure an accurate diagnosis [ 12 , 21 ]. MDD is critical for the diagnosis of IPF and other ILD, especially when the HRCT patterns and clinical workup do not provide a clear diagnosis [ 22 ].…”

“…In our case, the first ILD workup performed at the local respiratory center was a reason for an initial delay of an accurate IPF diagnosis. A recent survey undertaken in a representative group of Polish pulmonologists showed that only 63% of them routinely work with an ILD expert radiologist for the differential diagnosis of ILD [ 21 ]. In the authors' opinion, the formation of experienced multidisciplinary teams in Polish respiratory centers involved in ILD diagnosis and management should become a priority as long as IPF diagnostic standards are considered.…”

A survival analysis of idiopathic pulmonary fibrosis in the context of antifibrotic therapy in Saudi Arabia

“…[ 1 2 ] It is characterized by nonproductive cough and exertional breathlessness. [ 2 3 4 ] The median survival of patients with IPF has been reported to range from 2.5 to 3.5 years. [ 2 5 ]…”