The European IPF registry offers important insights into the characteristics, care management, and outcomes of idiopathic pulmonary fibrosis (IPF) patients in Europe, a study reports.
Data from the registry showed that the most common symptoms reported by IPF patients are breathing difficulties, fatigue, and a dry cough, according to the study, which was led by researchers in Germany.
The registry also showed that the use of antifibrotic therapies is successfully increasing the survival rate of this patient population.
The study, “The European IPF registry (eurIPFreg): baseline characteristics and survival of patients with idiopathic pulmonary fibrosis,” was published in the journal Respiratory Research.
IPF primarily impacts the elderly, and the progression of the disease can be rather rapid and variable. It is therefore essential to identify risk factors that can help in the early diagnosis of IPF and treatments that have a positive influence on clinical outcomes.
To address this, the European IPF Network set up the IPF registry (NCT02951416), a multicenter online registry linked with the European Biobank. The registry is funded by the European Commission’s FP7 program.
The European IPF registry is a growing database of IPF patients and physicians documenting disease symptoms, medication history, and disease state. Its purpose is to offer a better understanding of IPF, study the progression of the disease and risk factors, and find better treatment options.
In the study, researchers evaluated data from 525 IPF patients, of whom 73.7% were male, at the time of recruitment (baseline data) in the registry, between November 2009 and December 2016. The mean age of participants at the time of recruitment was 68.1. Of the participants, 18.64% had a family history of IPF or other interstitial lung disease — conditions that cause scarring of the lungs.
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Primary reasons why patients sought medical advice were a gradual inability to breath, or insidious dyspnea, (90.1%), fatigue (69.2%), and dry coughing (53.2%).
Researchers found that the proportion of patients who underwent a surgical lung biopsy dropped from 32% in 2009 to 8% in 2016. The team attributed this decrease to the availability and increased use of less invasive techniques, such as cryobiopsy.
Forced vital capacity (FVC) and diffusing capacity of the lungs for carbon monoxide (DLCO) were used to assess the lung function of participants. FVC assesses the patient’s breathing ability, while DLCO assesses the ability of the lungs to transfer oxygen through the blood.
Baseline value of mean FVC was 68.4% of the predicted value, indicating a reduced ability to breathe. A total of 135 patients received long-term oxygen treatment.
Similarly, the mean DLCO value was 42.1% of the predicted value, indicating impaired lung function. For comparison, a DLCO value of more than 75% is considered normal.
According to the researchers, comorbidities, or coexisting conditions, were common in IPF patients, the most frequently reported being arterial hypertension (32.2%), followed by gastroesophageal reflux (27.9%). Pulmonary hypertension was reported in 16.8% of the patients.
They also found changes in medication choices after the approval in Europe of antifibrotic medications, specifically Boehringer Ingelheim‘s Ofev (nintedanib) in 2015 and Genentech‘s Esbriet (pirfenidone) in 2011, which significantly improved patient survival.
Prior to antifibrotics, treatments of choice included immunosuppressants, steroids, and N-acetylcysteine.
The median survival rate of patients taking antifibrotics was 123.1 months, compared with 68.3 months on any other medications.
According to the team, pneumonia with sepsis and multi-organ failure were the main causes of death in these patients, followed by heart failure due to pulmonary hypertension.
“The data reflect changes in the diagnostic and therapeutic approach in IPF in the last 10 years, supporting the important role of large real-world data registries to document and scrutinize changes in IPF management,” the researchers concluded.
They also emphasized the importance of setting up patient registries: “A real-life data registry significantly complements data from randomized controlled trials, typically comprising a wide range of severity, progression and co-morbidities.”