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Referring Czech patients with idiopathic pulmonary fibrosis to a transplant center – data from the EMPIRE registry

Publication at First Faculty of Medicine, Second Faculty of Medicine |


Idiopathic pulmonary fibrosis (IPF) remains a fatal disease despite indisputable advances in its management. One of the treatment modalities that may be offered to some IPF patients is lung transplantation.

The present study aimed to assess the adherence of physicians in interstitial lung disease (ILD) centers to the International Society for Heart and Lung Transplantation consensus and to evaluate the potential applicability of the consensus in routine practice, based on clinical practice applied in transplant centers. From the Czech part of the EMPIRE registry, patients diagnosed with IPF between 1999 and December 2021 (n = 1632) who were younger than 65 years of age at the time of diagnosis were retrospectively selected.

From this group of patients, non-smokers and ex-smokers with a body mass index <= 32 kg/m2 (n = 404) were excluded. So were patients with a history of cancer < 5 years from the time of IPF diagnosis, those with alcohol abuse and patients with multiple vascular comorbidities.

The management of each patient was verified in the relevant ILD center. From the database of transplanted patients (1999-12/2021, n = 541), all individuals transplanted for pulmonary fibrosis were selected (n = 186).

Subsequently, the diagnosis of IPF was verified from the patients' medical records (n = 67). The number of candidates eligible for the lung transplant waiting list was 304.

The IPP centers referred 96 patients to a transplant center, of whom 50 % (n = 49) were put on the waiting list. According to data from the EMPIRE registry and IPP centers, 25 referred IPF patients were transplanted; according to the transplant center, the number was 67.

Among Czech IPF patients, there may be a larger pool of potential lung transplantation candidates than has been referred to the transplant center. Given the effect of antifibrotic therapy on slowing down the disease progression, it is rational to individually consider the inclusion of patients older than 65 years.

Unless there is an obvious absolute contraindication for inclusion in the transplant program, it is advisable to have the impact of comorbidities on the eligibility for enrolment in the program assessed by a transplant center physician.