BACKGROUND: The prognosis of idiopathic pulmonary fibrosis (IPF) can be predicted by the gender, age, and physiology (GAP) index. However, antifibrotic therapy (i.e., nintedanib and pirfenidone) may improve survival. AIMS: This study aimed to compare the outcomes of antifibrotic-treated IPF with the survival predicted by the GAP index. METHODS: A retrospective cohort study was conducted from March 2014 to January 2020. The electronic health-care records of all IPF patients treated with nintedanib or pirfenidone were reviewed. Besides standard demographic and mortality data, the variables required to calculate the GAP index were also extracted. RESULTS: Eighty-one patients (male 55, 68%; age 71.4 ± 10.2 years) with IPF received antifibrotic therapy (nintedanib 44.4%; pirfenidone 55.6%; mean follow-up 35 ± 16.5 months). Cumulative mortality (whole cohort 3 years 12%; 4 years 26%; 5 years 33%) was significantly less than predicted by the GAP index. CONCLUSIONS: The survival of antifibrotic-treated IPF is better than predicted by the GAP index. Novel systems for prognostication are required. The survival benefit from pirfenidone and nintedanib seem similar overall.
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a chronic progressive age-related lung disease causing relentless fibrosis of the lung parenchyma. Currently, pirfenidone and nintedanib are the two antifibrotic drugs, approved for the treatment of IPF. Both are shown to slow progression by preserving lung functions from rapid decline compared to a placebo. We are reporting a real-life patient experience using these two antifibrotic medications (AFMs) in our tertiary care hospital. METHODS: A retrospective cohort study was conducted for all IPF cases diagnosed in multidisciplinary meetings between 2015 and 2020 at KAMC, Riyadh (Saudi Arabia). We are reporting patients' demographics, lung function, survival, tolerance, side effects, or death in patients taking AFMs. RESULTS: A total of 81 cases were identified. The majority of patients aged 67 years (68%) were men with a median age of 68 years. Late presentation, severe disease, and definite usual interstitial pneumonia patterns were reported in 60% of our patients. The average number of hospital admissions before starting treatment was 1 (range: 0–3) in the nintedanib group and 1.4 (range: 1.2–5) in the pirfenidone group. There was an increase in the number of hospital admissions in the group started on pirfenidone 1.7 (range: 1.9–8) compared to nintedanib 0.5 (range: 0–3), P = 0.001. The observed mortality outcome in this cohort was 4 (11%) and 12 (27%) for nintedanib and pirfenidone, respectively. The predominant side effects were gastrointestinal symptoms for both the groups 18 (22%). CONCLUSIONS: Pirfenidone and nintedanib are the available approved antifibrotic agents used for many years to treat IPF patients. Real-life data showed better tolerability than reported in the West, good compliance, and a manageable side effect profile in this group of elderly and severe IPF patients.
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