Diffuse Lung Disease: Fellow Case Report Slide: Diffuse Lung Disease |

Pirfenidone for Treatment of Bleomycin-Induced Lung Toxicity FREE TO VIEW

Suliman Alamro, MD; Zhou Zhang, MD; Anna Selvaggio, MD; Paul Noble, MD
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Cedars-Sinai Medical Center, Los Angeles, CA

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):508A. doi:10.1016/j.chest.2016.08.522
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SESSION TITLE: Fellow Case Report Slide: Diffuse Lung Disease

SESSION TYPE: Affiliate Case Report Slide

PRESENTED ON: Wednesday, October 26, 2016 at 11:00 AM - 12:15 PM

INTRODUCTION: Bleomycin is a chemotherapy agent that is useful to treat a variety of cancers but the major limitation of bleomycin is pulmonary toxicity that can manifest as life threatening interstitial fibrosis in up to 10% of patients.

CASE PRESENTATION: 64 years old male with Hodgkin lymphoma developed cough after receiving eighth cycle of bleomycin chemotherapy. His PFT showed FVC of 72% predicted, TLC of 83% predicted and DLCO of 52% predicted. Chest computed tomography showed peripheral reticular opacities and honeycombing involving the lower lung zones. He was started on prednisone at 80 mg daily in addition to imatinib with symptomatic improvement. However, his symptoms recurred and he was admitted to the hospital where CTPA showed a diffuse and severe interstitial fibrosis and alveolitis most consistent with bleomycin toxicity in addition to left lower lobe pulmonary embolus. A transbronchial biopsy revealed patchy organizing pneumonia and intraalveolar macrophages. The patient was treated with anticoagulation in addition to steroids. At this time, his PFTs showed FVC 55% predicted, TLC 62% predicted and DLCO 27% predicted. Repeat chest CT showed a usual interstitial pneumonia pattern. The decision was made to start pirfenidone.

DISCUSSION: Current treatments of bleomycin induced lung toxicity include discontinuation of bleomycin and administration of glucocorticoid for symptomatic patients. Pirfenidone is a pyridone derivative that has antifibrotic and anti-inflammatory effects which has shown to improve bleomycin-induced pulmonary fibrosis in animals. There is case report in literature which did not find pirfenidone to be beneficial in patients with fulminant bleomycin induced pneumonitis, however, pirfenidone may beneficial when initiated during early course of bleomycin induced lung injury.

CONCLUSIONS: The patient had significant symptom improvement and his repeat PFT after three months of pirfenidone demonstrated FVC 69% predicted, TLC 76% predicted and DLCO 43% predicted. He was titrated off steroids and after six months of continuing pirfenidone therapy, his PFTs showed normal lung volumes with DLCO of 48% predicted.

Reference #1: Bendstrup E, Hyldgaard C, Agerbaek M, Andersen CU, Hilberg O. No effect of pirfenidone treatment in fulminant bleomycin-induced pneumonitis. Respiratory Medicine Case Reports 12 (2014) 47-49.

Reference #2: Lyer SN, Margolin SB, Hyde DM, Giri SN. Lung fibrosis is ameliorated by pirfenidone fed in diet after second dose in a three-dose bleomycin-hamster model. Exp Lung Res 1998; 24:119-132.

DISCLOSURE: The following authors have nothing to disclose: Suliman Alamro, Zhou Zhang, Anna Selvaggio, Paul Noble

Pirfenidone for treatment of bleomycin induced lung toxicity is not current standard of care and is considered investigational at this time.




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