Progressive Allograft Dysfunction Following Lung Transplantation: First Experience with Pirfenidone – A Case Report

Background: Chronic lung allograft dysfunction (CLAD) is one of the major factors limiting graft function after lung transplantation (LuTx). No effective medical treatment is available but animal experiments with pirfenidone have shown some promise. The aim of this case report was to evaluate pirfenidone in a LuTx recipient with progressive CLAD.

Methods: Descriptive data analysis was performed prospectively based on lung function testing, functional outcome and quality of life valuation.

Results: Our case is a 56-year-old female LuTx recipient who underwent LuTx in 2009 due to idiopathic pulmonary fibrosis accompanied by pulmonary hypertension. In 2011 the patient was diagnosed with bronchiolitis obliterans syndrome (BOS) stage 1 (A0/B0). As the patient rapidly progressed to BOS stage 2 and a therapy with azithromycine, montelucast and i.v. steroids as well as a fundoplication were unsuccessful we started a pirfenidone treatment in October 2011. Forced Expiratory Volume in 1 Second (FEV1), Forced Vital Capacity (FVC) and Total Lung Capacity (TLC) before the start of pirfenidone were 1,09 l (51% pred.), 2,21 l (84% pred.) and 4,24 l (95% pred.), respectively. Follow-up pulmonary function tests after three months revealed a FEV1 of 1,26 l (59% pred.), a FVC of 2,23 l (85% pred.) and a TLC of 4,49 l (101% pred.) as well as after six months a FEV1 of 1,30 l (61% pred.), a FVC of 2,54 l (100% pred.) and a TLC of 4,53 l (102% pred.), correspondingly. The distance covered in 6 minutes before the treatment was 510 m and during therapy after three months 590 m and after six months 580 m, respectively. According to the Short Form-36 Health Questionnaire the patient was found to have a good quality of life throughout the conduct of the treatment. Laboratory evaluations including monthly renal- and liver function tests, CMV- and infection screening as well as tacrolimus blood levels showed no change during the administration of pirfenidone.

Conclusion: Pirfenidone was well tolerated and the patient continued the treatment on stable dosage and remained on BOS stage 2 under therapy. Our case suggests that the use of pirfenidone after LuTx is safe. The therapeutic potential of pirfenidone in LuTx recipients should be investigated in future controlled trials.