Baseline clinical characteristics, comorbidities and prescribed medication in a real-world population of patients with idiopathic pulmonary fibrosis: the PROOF registry

Abstract

IntroductionPROOF (a Prospective Observational Registry to Describe the Disease Course and Outcomes of Idiopathic Pulmonary Fibrosis) is an ongoing, observational registry initiated in 2013 with the aim of collecting real-world data from patients with idiopathic pulmonary fibrosis (IPF). Here, we present comprehensive baseline data, which were collected from patients on registry inclusion.MethodsPatients with IPF were enrolled across eight centres in Belgium and Luxembourg. Baseline data collected included demographics, diagnostic information and clinical characteristics, including lung function and health-related quality of life. Data on comorbidities and prescribed medication were also collected.ResultsA total of 277 patients were enrolled in the PROOF registry. At inclusion, 92.8% and 6.5% of patients had a definite or probable diagnosis of IPF, respectively. Mean per cent predicted forced vital capacity and carbon monoxide diffusing capacity were 80.6% and 46.9%, respectively. Mean St. George’s Respiratory Questionnaire total score was 47.0, and mean Cough-Visual Analogue Scale score was 30.5 mm. The most prevalent comorbidities reported at inclusion were gastrointestinal disorders (50.2%), including gastro-oesophageal reflux disease (47.3%) and metabolism and nutrition disorders (39.7%). At inclusion, 67.2% and 2.2% of patients were prescribed pirfenidone and nintedanib, respectively, with treatment initiated either prior to, or at the time of, inclusion. Medication prescribed concomitantly with pirfenidone included antihypertensives (54.8%), statins (37.1%) and prophylactic antithrombotics/anticoagulants (36.6%).ConclusionThe PROOF registry provides valuable demographic and clinical data from a real-world population of patients with IPF in Belgium and Luxembourg, demonstrating the high burden of comorbidities and prescribed medication in these patients. Longitudinal data from this patient population will be investigated in future analyses.Trial registrationPROOF is registered with the relevant authorities in Belgium and Luxembourg, with registration to Comité National d’Éthique et de Recherché (CNER) N201309/03 – 12 September 2013 and a notification to Comité National de Protection des Données (CNDP).

Disease Severity and Quality of Life in Patients With Idiopathic Pulmonary Fibrosis

“…Baseline QOL patterns in the IPF-PRO registry were largely consistent with those observed in Insights Pulmonary Fibrosis In Clinical Practice (INSIGHTS-IPF), 24 a multicenter German registry; the Australian IPF Registry (a multicenter cohort of 516 patients) 16 ; and a Prospective Observational Registry to Describe the Disease Course and Outcomes of Idiopathic Pulmonary Fibrosis (PROOF) registry, a cohort of 277 patients enrolled at eight centers in Belgium and Luxembourg. 25 Baseline SGRQ scores in the IPF-PRO registry were nearly identical to baseline SGRQ total scores in the Safety and Efficacy of BIBF 1120 at High Dose in Idiopathic Pulmonary Fibrosis Patients (INPULSIS) trials, 26 despite the longer median time since diagnosis of 1.6 years. Disease severity of patients in the INPULSIS trials (mean CPI, 46.1 27 ; 47.2% GAP Stage I 28 ) was also similar to that of the IPF-PRO registry, which may contribute to the comparable QOL observed.…”

S2K-Leitlinie zur Diagnostik der idiopathischen Lungenfibrose

“…Die Prävalenz nimmt mit steigendem Alter zu [7]. Eine familiäre Häufung der IPF wurde in bis 11 % der Krankheitsfälle beobachtet [9,10].…”

“…Umwelteinflüsse, wie z. B. Exposition gegenüber Asbest, Metall-und Holzstäuben, Chemikalien oder Kontakt mit Allergenen, die üblicherweise bei einer exogen allergischen Alveolitis gefunden werden, konnten in bis zu 38 % der IPF-Patienten eruiert werden, ohne dass ein eindeutiger Kausalzusammenhang hergestellt werden konnte [9,14].…”

“…Komorbiditäten sind bei Patienten mit IPF im Allgemeinen häufiger als in der Normalbevölkerung zu finden und prognoserelevant [24,25]. Relevante Komorbiditäten sind kardiovaskuläre und thrombembolische Erkrankungen, gastroösophagealer Reflux, Lungenkarzinom, Depression, Schlaf-bezogene Atmungsstörungen und Diabetes [9,26,27]. Die IPF kann in einem Drit-tel der Fälle von einer COPD oder einem Lungenemphysems begleitet werden, da diese Erkrankungen als Folge des langjährigen Tabakrauchens auftreten [9,26,27].…”

Patients’ and Healthcare Professionals’ Experiences of Idiopathic Pulmonary Fibrosis Treatment with the Pirfenidone 801 mg Tablet Formulation: A Multinational Survey

“…The two formulations have been shown to be bioequivalent [13], but it is possible that the reduced pill burden associated with the 801 mg formulation may have benefits in terms of adherence and QoL in patients with IPF. Real-world evidence has shown that patients with IPF have a high burden of comorbidities and concomitant medications [16][17][18]; therefore, a simplified pirfenidone dosing regimen may help reduce this burden. An increased number of prescribed daily dose units of medication has been shown to be associated with poorer adherence in patients with a range of conditions [19][20][21], and poor adherence to medications has been found to result in reduced QoL and poorer treatment outcomes [19].…”