CHEST Guidelines-Pirfenidone-Induced-Sarcoid-Like-Reaction

Pirfenidone-Induced-Sarcoid-Like-Reaction_chest

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This article discusses a novel complication, a sarcoid-like reaction, linked to the use of pirfenidone, a medication approved to slow the progression of idiopathic pulmonary fibrosis (IPF). The case report involves a 65-year-old male with IPF who started on pirfenidone in April 2015. The patient initially showed no signs of sarcoidosis or lymphadenopathy. However, after commencing pirfenidone, he developed diffuse hypermetabolic mediastinal and abdominal lymphadenopathy, a feature commonly associated with sarcoidosis. This led to further evaluation, including an endobronchial ultrasound-guided transbronchial needle aspiration and a mediastinoscopy with excisional biopsy. Findings revealed benign lymph node tissue with non-necrotizing granulomas, indicating a sarcoid-like reaction. The diagnosis of actual sarcoidosis could not be entirely ruled out, though it was considered unlikely due to the timing with pirfenidone therapy.<br /><br />Pirfenidone was discontinued, and lung transplantation was pursued. Post-discontinuation CT scans indicated stable lymphadenopathy, strengthening the suspicion that the lymphadenopathy was a reaction to pirfenidone. The patient's successful bilateral lung transplantation further confirmed this theory, as post-transplant pathology continued to show non-necrotizing granulomas, and later scans revealed resolved lymphadenopathy.<br /><br />This case highlights the need for clinicians to consider a potential sarcoid-like reaction in patients undergoing treatment with pirfenidone who develop hypermetabolic lymphadenopathy. Tissue biopsy is recommended for accurate diagnosis, suggesting an awareness of this complication is essential to differentiate between sarcoidosis, an inciting medication reaction, and other differential diagnoses. The detailed and multidisciplinary evaluation approach adopted in this case underscores the necessity for comprehensive patient management in complex presentations.

Keywords

pirfenidone

sarcoid-like reaction

idiopathic pulmonary fibrosis

lymphadenopathy

non-necrotizing granulomas

lung transplantation

hypermetabolic

biopsy

sarcoidosis

patient management