Abstract
Background: PASSPORT is a post-authorization safety registry for pirfenidone to collect “real-world” data in EU patients with idiopathic pulmonary fibrosis (IPF).
Objective: s: 1) Assess the safety of pirfenidone as monotherapy and with N-acetylcysteine (NAC) and/or corticosteroids (CS); 2) examine country-specific differences.
Methods: 109 EU sites dosed 1006 patients; largest enrolling countries were Germany (N=451), France (N=214) and UK (N=183). Safety data were recorded at routine clinic visits for up to 2 yrs. Adverse drug reactions (ADR: noxious, unintended drug response) were collected.
Results: At baseline, mean±SD age was 70±8.5 yr and time since IPF diagnosis 1.6±2.5 yr; 80% were male; supplemental O2 was used by 27%; mean±SD FVC was 2.56±0.78 L; mean±SD % predicted FVC was 66±16% (15% had %FVC< 50%). Most common comorbidities (>10%) were hypertension, GERD, hypercholesterolemia and coronary artery disease.
At this interim analysis, median time on pirfenidone was 7.6 mo; total exposure was 803 PY. 71% of patients received pirfenidone alone; 12%, 9% and 8% also received NAC, CS, and NAC+CS, respectively. ADR incidence was generally consistent for these subgroups except weight decrease which occurred more often in the pirfenidone+CS group (21.1% vs 9.8%-11.8%). Two-thirds had ≥1 ADR; most common were nausea, 17%; fatigue, 15%; decreased appetite, 13%; decreased weight, 12%; rash, 10%; diarrhea, 9%.
Patient characteristics and ADRs were similar in the 3 largest enrolling countries.
Conclusion: In this real-world setting, pirfenidone was generally safe and well tolerated as monotherapy or combined with NAC and/or CS. Characteristics and safety profile of German, French and UK patients were similar.
- Copyright ©ERS 2015