The quality of life of people with idiopathic pulmonary fibrosis (IPF) is strongly associated with clinical changes in lung function, comorbidities, disease duration, and overall clinical course of the disease, a study shows.
The study, “The clinical course of idiopathic pulmonary fibrosis and its association to quality of life over time: longitudinal data from the INSIGHTS-IPF registry,” was published in the journal Respiratory Research.
IPF is a severe progressive respiratory disease that can have a major impact on the lives of those who have the disease or care for affected people.
Available therapies aim to prevent the progression of the disease, manage its symptoms, and prolong patients’ lives. But it remains poorly understood which are the best strategies to improve IPF patients’ health-related quality of life and fulfill psychosocial needs.
German researchers conducted a nationwide study to gain better insights on the clinical outcome and overall effect of IPF throughout time in a real-life setting. The INSIGHTS-IPF registry (NCT01695408) was launched in November 2012, and is still recruiting adult IPF patients across 19 pulmonary specialist centers in Germany.
Of 879 patients enrolled in the study, 424 provided information about their quality of life when they first registered and after at least one follow-up visit. These patients’ mean age was 68.7 years, and 76.9% were male. Most patients (77.6%) had one or more comorbidities.
All participants completed the New York Heart Association (NYHA) functional status assessment and a range of quality-of-life questionnaires, including the World Health Organization-5 Well-Being Index (WHO-5) and the EuroQol five-dimensional questionnaire (EQ-5D).
Analysis of the patients’ quality of life over time showed a significant increase in respiratory function scores every 6 months, indicating a poorer quality of life compared to baseline. EQ-5D pain scores and WHO-5 scores also significantly decreased across time, indicating a progressively worse quality of life.
Women showed significantly poorer respiratory function scores during follow-up compared to men.
The more impaired the lung function was at enrollment, the higher the respiratory scores, as determined by the St. George’s Respiratory Questionnaire (SGRQ), during follow-up. Higher SGRQ scores correspond to worse quality of life.
During a 3-year follow-up period, 26.7% of the patients in the registry died (113 people). Changes in any of the quality of life outcome scores — SGRQ, EQ-5D, or WHO-5 — all were predictive of mortality.
Patients with a greater increase in SGRQ total score since enrollment and those with more comorbidities had a 1.03- and 2.4-fold higher mortality risk, respectively.
In the last follow-up visit, patients who died had significantly worse SGRQ scores, as well as significantly lower EQ-5D and WHO-5 scores, compared to surviving patients. In general, lower quality of life was significantly associated with increased risk of mortality.
Further analysis revealed that hospitalizations also had an adverse impact on patients’ quality of life, with significantly higher SGRQ scores at the subsequent follow-up visit compared to those reported at the visit prior to hospitalization. The worst scores were reported in patients hospitalized for acute exacerbations.
Overall, the findings of the “INSIGHTS-IPF registry demonstrate a close relationship between quality of life and clinically meaningful changes in lung function, comorbidities, disease duration, and clinical course of IPF, including hospitalization and mortality,” researchers stated.
The study data reveal a “longitudinal decline in quality of life” in the IPF population. Pharmacological and non-pharmacological strategies to promote an increased lifespan, with particular focus on pulmonary rehabilitation and comorbidities prevention and treatment, may increase quality of life in IPF patients, the team suggested.