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A6572 - Successful Use of Pirfenidone in a Case of Progressive Restrictive Allograft Dysfunction After Lung Transplantation
Author Block: J. Roddy1, A. L. Anton2, A. J. Zane1, K. Shah1; 1Internal Medicine, Medical College of Wisconsin, Milwaukee, WI, United States, 2Pulmonary Medicine, Medical College of Wiscons/Clement Zablocki VAMC, Milwaukee, WI, United States.
Pirfenidone is an antifibrotic agent, FDA approved for the treatment of idiopathic pulmonary fibrosis (IPF). The evidence for use of pirfenidone in patients with restrictive allograft dysfunction is limited to animal studies and few case reports. In addition, the duration of treatment and length of follow-up were rather limited. Restrictive allograft dysfunction is a proposed subtype of chronic lung allograph dysfunction (CLAD) mediated by fibrogenesis resulting in a progressive restrictive ventilatory defect. We present a case of a 47-year-old patient with IPF diagnosed via an open lung biopsy who underwent a single lung transplantation. His post-transplantation course was characterized by an initial relative clinical and radiographic stability for the first 5 years followed by gradual worsening of symptoms and increased oxygen requirements to as high as 6 L of oxygen. His serial spirometry continued to decline to as low as FVC 2.18. Serial CT scans showed progressive fibrosis in his native lung as well as scar changes in the transplanted lung. The observed deterioration was likely attributable to CLAD with restrictive allograft dysfunction as well as worsening IPF in the native lung. Patient was deemed not to be a surgical candidate for re-transplantation. Given the progressive worsening despite the immunosuppressant therapy as well as medical optimization, a pirfenidone trial was offered to the patient. The rationale of pirfenidone initiation was based on dual role in the treatment of IPF in the native lung as well as potential beneficial role in restrictive-type CLAD. Side effects were monitored closely, and the main side effect observed was severe nausea. Within the first three months of use, he claimed no further decline in his respiratory symptoms. Serial PFT’s every three months and CT scans every 6-9 months confirmed clinical stability. Currently our patient has been on pirfenidone for over eighteen months. His overall clinical and radiographic course and oxygen requirements have been stable. This report adds to the body of evidence supporting the use of pirfenidone in patients with progressively restrictive CLAD, highlighting the need for further research to confirm pirfenidone’s beneficial effect in this patient population.