An Open-label Study With Pirfenidone on Chronic Hypersensitivity Pneumonitis |
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| Affiliation: | 1. Inflammation, Repair, and Development Section, National Heart and Lung Institute, Imperial College London, London, UK;2. National Institute for Health Research Respiratory Clinical Research Facility, Royal Brompton Hospital, London, UK;3. Department of Respiratory Medicine, Royal Prince Alfred Hospital, Sydney, NSW, Australia;4. Medical School, University of Sydney, Sydney, NSW, Australia;5. University of Colorado School of Medicine, Denver, CO, USA;6. Bristol-Myers Squibb, Princeton, NJ, USA;7. Center for Interstitial and Rare Lung Diseases, Department of Pulmonology and Critical Care Medicine, Thoraxklinik, University of Heidelberg, Heidelberg, Germany;8. Divison of Pulmonary, Allergy, and Critical Care Medicine, Columbia University Medical Center, New York, NY, USA;9. Regeneron Pharmaceuticals, New York, NY, USA;10. Respiratory Department, Unit of Interstitial Lung Diseases, University Hospital of Bellvitge Institute for Biomedical Research (IDIBELL), Barcelona, Spain;11. Centro de Investigación Biomédica en Red Enfermedades Respiratorias (CIBERES), Madrid, Spain;12. F Hoffmann-La Roche, Basel, Switzerland;13. F Hoffmann-La Roche, Athens, Greece;14. National Reference Coordinating Center for Rare Pulmonary Diseases, Louis Pradel Hospital, Lyon, France;15. Hospices Civils de Lyon, Université Claude Bernard Lyon 1, Lyon, France;1. First Academic Department of Pneumonology, Hospital for Diseases of the Chest, “Sotiria”, Medical School, University of Athens, Greece;2. Division of Immunology, Biomedical Sciences Research Center “Alexander Fleming”, Athens, Greece;3. 5th Respiratory Department, Hospital for Diseases of the Chest, “Sotiria”, Athens, Greece;4. University Hospital of Alexandroupolis, Department of Pneumonology, Democritus University of Thrace, Greece;1. Department of Respiratory Medicine and Allergy, Tosei General Hospital, 160 Nishioiwake-cho, Seto, Aichi, 489-8642, Japan;2. Department of Radiology, Kinki Central Hospital of Mutual Aid Association of Public Health Teachers, Itami, Japan;3. Department of Laboratory of Pathology, Nagasaki University Hospital, Nagasaki, Japan;1. Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico;2. Programa de Maestría y Doctorado en Ciencias Médicas, Facultad de Medicina, Universidad Nacional Autónoma de México, Mexico;3. Hospital Universitario de Caracas, Universidad Central de Venezuela, Caracas, Venezuela;4. Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico;1. Unidad de Medicina Experimental, Facultad de Medicina, Universidad Nacional Autónoma de México, Ciudad de México, Mexico;2. Servicio Clínico de enfermedades del Intersticio del Pulmón y Reumatología Instituto Nacional de Enfermedades Respiratorias, “Ismael Cosío Villegas”, Ciudad de México, Mexico;3. Instituto Nacional de Pediatría, Laboratorio de Inmunogenética Molecular, Departamento de Análisis Clínicos y Estudios Especiales, México, DF, Mexico;4. Departamento de Biologia Celular e Molecular e Bioagentes Patogênicos, Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, São Paulo, Brazil;5. Departamento de Microbiología y Parasitología, Facultad de Medicina, Universidad Nacional Autónoma de México. Ciudad de México, Mexico;6. Servicio de Anatomía Patológica, Unidad de Citopatología. Hospital General de México “Dr. Eduardo Liceaga”, Ciudad de México, Mexico |
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| Abstract: | BackgroundChronic hypersensitivity pneumonitis (cHP) represents a severe lung disease often evolving to fibrosis with the subsequent destruction of the lung parenchyma. There are no approved therapies with confirmed efficacy to deal with this disease.MethodsWe performed an open-label, proof of concept study, to evaluate the efficacy and safety of pirfenidone added to immunosuppressive drugs on the treatment of cHP. We included 22 patients assigned to two groups: Group 1, nine patients that received prednisone plus azathioprine and Group 2, thirteen patients, received prednisone plus azathioprine and pirfenidone (ClinicalTrials.gov identifier NCT02496182). There were no significant imbalances in clinically relevant baseline characteristics between two study groups.ResultsAfter 1 year of treatment, inclusion of pirfenidone was not associated with improved forced vital capacity (primary end-point). A not significant tendency to show higher improvement of diffusion capacity of the lung for carbon monoxide (DLCO) was observed in the group receiving pirfenidone (p = 0.06). Likewise, a significant improvement in the total score on the SGRQ was found in the group 2 (p = 0.02) without differences in other two questionnaires related to quality of life (ATAQ-IPF and EQ-5D-3L). HRCT showed a decrease of the ground glass attenuation without changes in the fibrotic lesions and without differences between both groups.ConclusionsThese findings suggest that the addition of pirfenidone to the anti-inflammatory treatment in patients with chronic HP may improve the outcome with acceptable safety profile. However, prospective randomized double-blind, placebo-controlled trials in largest cohorts are needed to validate its efficacy. |
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| Keywords: | Hypersensitivity pneumonitis Pulmonary fibrosis Pirfenidone Neumonitis por hipersensibilidad Fibrosis pulmonar Pirfenidona |
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