Overall drug treatment of idiopathic pulmonary fibrosis patients from national registries - a real-world study from Finland.

BACKGROUND: Currently, two disease-modifying antifibrotic drugs are indicated for the treatment of idiopathic pulmonary fibrosis. The objective of this study was to analyse antifibrotic and overall prescription medication use of IPF patients in the real world. METHODS: Data was collected from the FinnishIPF registry and the Registry of the Social Insurance Institution of Finland (SII). Purchases of all prescription medicines were assessed. The frequency, the initiation interval, the duration, and the breaks of the antifibrotic treatments were defined. The association between the prescription of antifibrotic therapy and different patient-related clinical parameters was studied. Accordingly, the relationships between the delay in starting therapy and patient-related variables were analysed. RESULTS: Of the 263 IPF patients, 132 (50.2%) had started antifibrotic treatment during the study period 2011-2018. The mean interval from the diagnosis to the first purchase was 367 (SD 429) days. The antifibrotic drug was switched in 14% of patients. Discontinuation of therapy occurred most commonly during the first year of the treatment. The one-year persistence was 77.1% for pirfenidone and 78.9% for nintedanib. A tendency of treating patients under 75 years was noticed. Low forced vital capacity predicted earlier initiation of medication. CONCLUSIONS: The initiation of antifibrotics after diagnosis was slow, probably due to reimbursement limitations. Younger age at diagnosis affected treatment initiation although it is unknown which patients benefit most from these medications. The reasons for discontinuation of the antifibrotic therapy during the first year should be a focus in clinical work and further studies.

Forced Vital Capacity (FVC) decline, mortality and healthcare resource utilization in idiopathic pulmonary fibrosis.

Aim of the study: Potential care implications of antifibrotic reimbursement restrictions were studied by forced vital capacity (FVC) decline, mortality and specialty care related healthcare resource utilization in patients with idiopathic pulmonary fibrosis (IPF). Material and methods: IPF patients were identified from the electronic medical records of the Hospital District of Southwest Finland between 2005 and 2017. Text-mining was used for patient identification to exclude other interstitial lung diseases (ILD) from the cohort. FVC reimbursement restriction (FVC 50-90%) was used for stratification. Results: Out of all patients with ILD, 27% (N = 266) were identified to have IPF. At baseline, 24% presented with FVC>90% and 63% with FVC 50-90% predicted. FVC at diagnosis did not improve during the study period. Median survival decreased by severity from 6.7 years in FVC>90% at baseline to 0.7 years in patient with FVC<50% predicted. In the FVC>90% group, 14% died before a change in FVC category could be noted. Overall, 4.7 million euro worth of specialty care resources were spent on IPF patients. The highest cost driver was inpatient days. Conclusions: IPF is associated with a high burden of disease, and reimbursement restrictions are in conflict with early care. As there are antifibrotic treatment options for IPF patients, early diagnosis is important.

Demographics and survival of patients with idiopathic pulmonary fibrosis in the FinnishIPF registry.

Idiopathic pulmonary fibrosis (IPF) is characterised by unpredictable disease course and poor survival. After the introduction of novel antifibrotic drugs, the prognosis of patients with IPF is probably changing. FinnishIPF, a nationwide registry of carefully characterised patients, was initiated in Finland in 2011. For the data analysis, we included 453 incident IPF patients diagnosed during 2011-2015. In this study, we describe the demographics and prognosis of these real-life patients. The median overall survival time of registered IPF patients was 4.5 years. The transplant-free survival at 1, 2, 3, 4 and 5 years was 95%, 83%, 70%, 58% and 45%, respectively. Smoking did not have any effect on survival. 117 (26%) patients received pirfenidone or nintedanib. Patients who received ≥6 months of treatment had better survival compared with those who did not receive treatment but this difference disappeared after age adjustment. The transplantation rate was 3%. Although IPF is diagnosed in Finland at a older age, the prognosis is better than expected due to a relatively well preserved lung function at diagnosis. Age and pulmonary function were identified as independent predictors of survival in the entire IPF patient population as well as in patients who had received antifibrotic treatment.

Re-evaluation of diagnostic parameters is crucial for obtaining accurate data on idiopathic pulmonary fibrosis.

BACKGROUND: The FinnishIPF registry is a prospective, longitudinal national registry study on the epidemiology of idiopathic pulmonary fibrosis (IPF). It was designed to describe the characteristics, management and prognosis of prevalent and incident IPF patients. The study was initiated in 2012. METHODS: We present here results limited to five university hospitals. Patients with IPF were screened from hospital registries using ICD-10 diagnosis codes J84.1 and J84.9. All patients who gave informed consent were included and evaluated using novel diagnostic criteria. Point prevalence on the 31(st) of December in 2012 was calculated using the reported population in each university hospital city as the denominator. RESULTS: Patients with ICD-10 codes J84.1 and J84.9 yielded a heterogeneous group - on the basis of patient records assessed by pulmonologists only 20-30 % of the cases were IPF. After clinical, radiological and histological re-evaluation 111 of 123 (90 %) of patients fulfilled the clinical criteria of IPF. The estimated prevalence of IPF was 8.6 cases/100 000. 60.4 % were men. Forty four percent of the patients were never-smokers. At diagnosis, the patients' mean age was 73.5 years and mean FVC was 80.4 % and DLCO 57.3 % of predicted. CONCLUSIONS: Our results suggest that hospital registries are inaccurate for epidemiological studies unless patients are carefully re-evaluated. IPF is diagnosed in Finland at a stage when lung function is still quite well preserved. Smoking in patients with IPF was less common than in previous reports.

Idiopathic pulmonary fibrosis--a systematic review on methodology for the collection of epidemiological data.

BACKGROUND: Recent studies suggest that the incidence of idiopathic pulmonary fibrosis (IPF) is rising. Accurate epidemiological data on IPF, however, are sparse and the results of previous studies are contradictory. This study was undertaken to gain insight into the various methods used in the epidemiological research of IPF, and to get accurate and comparable data on these different methodologies. METHODS: A systematic database search was performed in order to identify all epidemiological studies on IPF after the previous guidelines for diagnosis and treatment were published in 2000. Medline (via Pubmed), Science Sitation Index (via Web of Science) and Embase databases were searched for original epidemiological articles published in English in international peer-reviewed journals starting from 2001. After pre-screening and a full-text review, 13 articles were accepted for data abstraction. RESULTS: Three different methodologies of epidemiological studies were most commonly used, namely: (1) national registry databases, (2) questionnaire-based studies, and (3) analysis of the health care system's own registry databases. The overall prevalence and incidence of IPF varied in these studies between 0.5-27.9/100,000 and 0.22-8.8/100,000, respectively. According to four studies the mortality and incidence of IPF are rising. CONCLUSIONS: We conclude that there are numerous ways to execute epidemiological research in the field of IPF. This review offers the possibility to compare the different methodologies that have been used, and this information could form a basis for future studies investigating the prevalence and incidence of IPF.