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Sarcoid-Like Reaction in Patient on Pirfenidone for IPF

Imaging findings

Bulky, metabolically active lymphadenopathy is observed in the mediastinum, upper abdomen, and supraclavicular regions, superimposed on fibrosing interstitial lung disease (UIP/IPF pattern). Excisional biopsy revealed non-necrotizing granulomas.

Key takeaways

The development of widespread granulomatous lymphadenopathy in a patient with IPF on pirfenidone, despite pirfenidone not being a known cause of sarcoid-like reactions, poses a diagnostic challenge. It raises questions about whether it's an unrecognized drug effect, true sarcoidosis, or an unmasking of an underlying immune response.

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