• David Bennett, Nicola Lanzarone, Antonella Fossi, Felice Perillo, Elda De Vita, Luca Luzzi, Piero Paladini, Elena Bargagli, Piersante Sestini, and Paola Rottoli.
• Unit of Respiratory Diseases, Department of Medical Sciences, University Hospital of Siena, Siena, Italy - david.btt@gmail.com.
• Panminerva Med. 2020 Sep 1; 62 (3): 143-149.

BackgroundChronic lung allograft dysfunction (CLAD) is still the principal long-term cause of mortality after lung transplant. Animal studies and small case series have proposed pirfenidone, a potent antifibrotic agent registered for idiopathic pulmonary fibrosis (IPF), for treatment of CLAD. The aim of this study was to evaluate the safety profile and potential efficacy of pirfenidone in patients with CLAD.MethodsThe present study concerns a cohort of nine CLAD patients treated with pirfenidone. Pulmonary function tests were performed before and after beginning treatment. Side effects were recorded and survival was analyzed. All data were retrospectively collected.ResultsThe duration of treatment was 408.5±534.8 days. Significant side effects occurred in one case. FEV1 decline reduced from -44.5±40.7 mL/month in the 6 months before therapy to -12.8±34.3 mL/month in the following 6 months. However, data was only available for three patients (three patients died before 6 months of therapy, two patients lacked lung function parameters, one discontinued therapy and one was still in the early months of therapy). Median survival was 686 days. No significant survival differences were observed in relation to CLAD phenotype (BOS, RAS and BOS/RAS). Median survival from the start of pirfenidone therapy was 221 days.ConclusionsOur CLAD patients treated with pirfenidone showed a good safety profile, similarly to that reported for IPF patients. The drug showed potential for stabilizing decline in respiratory function. Further studies are needed in order to draw conclusions about the effectiveness of this therapy.

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