Abstract

Aims: To evaluate the effectiveness and tolerability of pirfenidone and nintedanib in idiopathic pulmonary fibrosis (IPF) and other fibrotic lung diseases.
Materials and methods: The study was retrospective and monocentric based on patients treated between 2015 and 2020 at the Vall d’Hebron University Hospital, the largest public hospital complex in Catalonia, Spain. Effectiveness and tolerability, including the relative change in forced vital capacity (FVC) assessed at 12 months, were evaluated using data for patients with interstitial lung disease retrieved from a registry and the medical records.
Results: Antifibrotics were administered to 55 patients: 34 (61.8%) with IPF, 14 (25.5%) with progressive pulmonary fibrosis (PPF), 5 (9.1%) with systemic sclerosis-associated interstitial lung disease (SSc-ILD), and 2 (3.6%) with post-COVID-19 fibrosis. Median age was 69.9 years; 78.2% were male. The median duration of nintedanib and pirfenidone treatment at study end was 12.1 (interquartile range (IQR) 6.9 – 22.8) and 27.6 months (IQR 10.4 – 58.7), respectively. Treatment was discontinued due to toxicity in 48.5% of patients treated with nintedanib and 18.2% of those treated with pirfenidone. At 1 year, 62% of patients with IPF, 64% of those with PPF, and all patients with SSc-ILD were still receiving antifibrotic therapy, and the median change from baseline in FVC was –2% (IQR from –7.1 to 7.2), –9.9% (from –25.4 to –1.6), and –3.3% (from –18.7 to 16.3), respectively.
Conclusion: Antifibrotic therapy using pirfenidone and nintedanib in patients with idiopathic pulmonary fibrosis provides significant stabilization of the disease measured using the change in FVC at 12 months. Treatment withdrawal was mostly attributable to poor tolerance to nintedanib.

Author Details

Authors

• Carla Sans-Pola^{1, 2, 3}
• Ricardo Boy¹
• Ana Villar⁴
• Iñigo Ojanguren⁴
• Carmen Pilar Simeón-Aznar⁵
• Alfredo Guillen-Del-Castillo⁵
• Guillem Vancells⁶
• Antònia Agustí^{1, 2, 3}
• Immaculada Danés^{1, 2, 3}

Departments

• ¹ Department of Clinical Pharmacology, Vall d’Hebron University Hospital, Vall d’Hebron Barcelona Hospital Campus,
• ² Department of Pharmacology, Therapeutics and Toxicology, Universitat Autònoma de Barcelona,
• ³ Immunomediated Diseases and Innovative Therapies Research Group, Vall d’Hebron Institut de Recerca (VHIR), Vall d’Hebron University Hospital,
• ⁴ Unit of Interstitial Lung Disease, Respiratory Department,
• ⁵ Unit of Autoimmune Systemic Diseases, Internal Medicine Department, and
• ⁶ Pharmacy Department, Vall d’Hebron University Hospital, Barcelona, Spain

Address

Immaculada Danés, MD
Hospital Universitari, Vall d’Hebron
Passeig Vall d’Hebron, 119
08035 Barcelona
Email: [email protected]

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