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P12 Pirfenidone post-authorisation safety registry (PASSPORT) – update and concomitant use of N-acetylcysteine and/or corticosteroids
  1. T Maher1,
  2. V Cottin2,
  3. A Azuma3,
  4. L Groves4,
  5. P Hormel4,
  6. M Sköld5,
  7. S Tomassetti6,
  8. D Koschel7
  1. 1Royal Brompton Hospital, London, UK
  2. 2National Reference Center for Rare Pulmonary Disease, Louis Pradel Hospital, Lyon, France
  3. 3Nippon Medical School, Tokyo, Japan
  4. 4Genentech, South San Francisco, CA, USA
  5. 5Karolinska Institute, Stockholm, Sweden
  6. 6GB Morgagni Hospital, Forli, FC, Italy
  7. 7Fachkrankenhaus Coswig, Coswig, Germany


Background PASSPORT is a post-authorisation safety registry for pirfenidone to collect real-world data in EU patients with idiopathic pulmonary fibrosis (IPF). This analysis assessed the safety of pirfenidone as monotherapy and in combination with N-acetylcysteine (NAC) and/or corticosteroids (CS).

Methods 109 EU sites dosed 1006 patients. Safety data were recorded at routine clinic visits for up to 2 years. Pirfenidone-associated adverse drug reactions (ADR) were collected.

Results At baseline, mean±SD age was 70 ± 8.5 years and mean±SD time since IPF diagnosis was 1.6 ± 2.5 years; 80% of patients were male; supplemental oxygen was used by 27% of patients; mean±SD FVC was 2.56 ± 0.78 L; mean±SD% predicted FVC was 66 ± 16% (14% had <50% predicted FVC). The most common comorbidities (>10%) were hypertension, gastroesophageal reflux disease, hypercholesterolemia and coronary artery disease.

At this interim analysis, median time on pirfenidone was 7.6 months and total exposure was 803 patient-years. Overall, 67% of patients had ≥1 ADR, most commonly: nausea, 17%; fatigue, 15%; decreased appetite, 13%; decreased weight, 12%; rash, 10%; and diarrhoea, 9%. Of patients who had an ADR, 55% experienced their first ADR within the first 30 days’ treatment. Around 5% of patients completed 2 years’ treatment, 55% are ongoing, 9% died and 21% discontinued due to pirfenidone-related ADRs (most commonly nausea, rash and decreased weight). 11% discontinued for other reasons.

Patients with FVC <50% had a higher discontinuation rate than other patients (48% vs 39%, respectively). The imbalance was mainly driven by higher rates of death and lung transplantation. The discontinuation rate due to pirfenidone ADRs was similar among patients with FVC <50% and ≥50% (20.3% vs 20.9%, respectively).

62% of patients received pirfenidone alone; 11%, 8% and 8% received pirfenidone plus NAC, CS, or NAC+CS, respectively. The remaining 11% had partial use of NAC and/or steroids. ADR incidence was generally consistent for these subgroups except weight decrease and ALT increase, which occurred more often in the pirfenidone+CS group.

Conclusions In this real-world setting, pirfenidone was generally safe and well tolerated as monotherapy or combined with NAC and/or CS. The rate of discontinuation due to pirfenidone-related ADRs was similar regardless of disease severity.

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