Abstract

Background and aims: We assessed disease characteristics and outcomes in patients with PF in Sweden.

Methods: This retrospective longitudinal study included patients with ICD-10 J84.1 from the Swedish National Patient Register in 2001–2015 (Figure). Electronic medical record data from Stockholm and Uppsala were used to refine for idiopathic PF (IPF) to form a subcohort (Figure).

Results: In the PF cohort (n=17,247) and IPF subcohort (n=291), mean (SD) age at diagnosis was 74.6 (10.5) and 70.6 (9.0) years, respectively; 62.5% and 70.1% of patients were male. For death from any cause and from PF, respectively, 5-year survival was 30% and 70% in the PF cohort and 60% and 78% in the IPF subcohort. Death from any cause or from PF within 1 year of PF diagnosis was associated with male sex and higher age at diagnosis. Lung transplant and oxygen-use rates, respectively, were 0.4% and 11.0% in the PF cohort and 0.7% and 61.9% in the IPF subcohort. Rate of hospitalisation in pulmonary units in the first year after diagnosis was 13.4% in the PF cohort and 16.5% in the IPF subcohort. The most common comorbid diagnoses within 1 year of PF diagnosis were ischaemic heart disease, congestive heart failure and dyspnoea in the PF cohort and cough, dyspnoea and ischaemic heart disease in the IPF subcohort.

Conclusions: Prognosis was poor and comorbidity burden was substantial in the cohort of patients with PF and subcohort with IPF.