C Magnus Sköld1, Lisen Arnheim-Dahlström2, Karen Bartley3, Christer Janson4, Klaus-Uwe Kirchgaessler5, Aaron Levine6, Giovanni Ferrara7. 1. Karolinska University Hospital, Stockholm, Sweden; Department of Medicine Solna, Karolinska Institutet, Stockholm, Sweden. 2. IQVIA, Solna, Sweden; Department of Medical Epidemiology and Biostatistics, Karolinska Institutet, Stockholm, Sweden. 3. Genentech, Inc., South San Francisco, CA, USA. 4. Department of Medical Sciences: Respiratory, Allergy and Sleep Research, Uppsala University, Uppsala, Sweden. 5. F. Hoffmann-La Roche, Ltd., Basel, Switzerland. 6. IQVIA, Solna, Sweden. 7. Karolinska University Hospital, Stockholm, Sweden; Department of Medicine Solna, Karolinska Institutet, Stockholm, Sweden. Electronic address: Giovanni.ferrara@ki.se.
Abstract
BACKGROUND: For patients with idiopathic pulmonary fibrosis (IPF), there is limited real-world data on patient journey and treatment patterns. AIM: To explore predictors of early diagnosis and treatment initiation, and treatment patterns in IPF patients using linked data from Swedish registers and electronic medical records (EMRs). POPULATION: A national cohort (C1) of 17,247 pulmonary fibrosis patients (ICD-10 code J84.1; no competing diagnosis) diagnosed between 2001 and 2015, and an EMR-based regional subset (C2) comprising 1755 IPF patients diagnosed between 2004 and 2017. The time from early disease symptoms to diagnosis, use of anti-fibrotic medications, time from diagnosis to initiation of anti-fibrotic treatment, and adherence, persistence and treatment length with pirfenidone were explored in these patients. RESULTS: In C1, the median time to diagnosis from the first symptoms dyspnoea, cough and fatigue were 307, 563 and 639 days, respectively. Glucocorticoids were the most frequently prescribed medication. Less than 10% of patients undergoing or initiating treatment, used pirfenidone or nintedanib. Males had a higher probability of initiating anti-fibrotic treatment than females within a year of diagnosis. One-year persistence in pirfenidone patients was 42% in C1 and 25% in C2. CONCLUSION: Diagnosis of pulmonary fibrosis was delayed in patients with cough and fatigue, which are early symptoms of IPF. This, and lower than expected utilisation of anti-fibrotic medications, suggests missed opportunities for early disease diagnosis and treatment. The high rate of treatment discontinuation underscores the importance of supporting and guiding patients to persist with their medications to ensure an accrual benefit of treatment.
BACKGROUND: For patients with idiopathic pulmonary fibrosis (IPF), there is limited real-world data on patient journey and treatment patterns. AIM: To explore predictors of early diagnosis and treatment initiation, and treatment patterns in IPF patients using linked data from Swedish registers and electronic medical records (EMRs). POPULATION: A national cohort (C1) of 17,247 pulmonary fibrosispatients (ICD-10 code J84.1; no competing diagnosis) diagnosed between 2001 and 2015, and an EMR-based regional subset (C2) comprising 1755 IPF patients diagnosed between 2004 and 2017. The time from early disease symptoms to diagnosis, use of anti-fibrotic medications, time from diagnosis to initiation of anti-fibrotic treatment, and adherence, persistence and treatment length with pirfenidone were explored in these patients. RESULTS: In C1, the median time to diagnosis from the first symptoms dyspnoea, cough and fatigue were 307, 563 and 639 days, respectively. Glucocorticoids were the most frequently prescribed medication. Less than 10% of patients undergoing or initiating treatment, used pirfenidone or nintedanib. Males had a higher probability of initiating anti-fibrotic treatment than females within a year of diagnosis. One-year persistence in pirfenidonepatients was 42% in C1 and 25% in C2. CONCLUSION: Diagnosis of pulmonary fibrosis was delayed in patients with cough and fatigue, which are early symptoms of IPF. This, and lower than expected utilisation of anti-fibrotic medications, suggests missed opportunities for early disease diagnosis and treatment. The high rate of treatment discontinuation underscores the importance of supporting and guiding patients to persist with their medications to ensure an accrual benefit of treatment.
Authors: David Rintell; Dena Heath; Florencia Braga Mendendez; Elizabeth Cross; Theodore Cross; Vincent Knobel; Bruno Gagnon; Cameron Turtle; Alan Cohen; Edward Kalmykov; Jonathan Fox Journal: Orphanet J Rare Dis Date: 2021-02-08 Impact factor: 4.123
Authors: Katarzyna Górska; Marta Maskey-Warzęchowska; Małgorzata Barnaś; Adam Białas; Adam Barczyk; Hanna Jagielska-Len; Ewa Jassem; Aleksander Kania; Katarzyna Lewandowska; Sebastian Majewski; Magdalena M Martusewicz-Boros; Wojciech J Piotrowski; Alicja Siemińska; Krzysztof Sładek; Małgorzata Sobiecka; Marzena Trzaska-Sobczak; Witold Tomkowski; Beata Żołnowska; Rafał Krenke Journal: Ther Adv Chronic Dis Date: 2022-08-22 Impact factor: 4.970