A Case of IgG4-Related Disease Developing During Long-Term High-Dose Intravenous Epoprostenol Therapy in a Patient With Idiopathic Pulmonary Arterial Hypertension
Takashi Inao, Kazuhiko Nakayama, Shintaro Yasui, Nanami Shite, Eriko Shiraki, Chiho Ohbayashi, Kojiro Otsuka, Hiroyuki Onishi, Soichiro Ohta, Masanori Iwahashi
https://doi.org/10.1002/pul2.70262
Abstract
Long-term intravenous (IV) epoprostenol is a key therapy for pulmonary arterial hypertension (PAH); however, rare immune-mediated conditions occurring during therapy remain incompletely characterised. We report a 34-year-old man with idiopathic PAH who developed low-grade fever and bilateral pulmonary infiltrates after 11 years of IV epoprostenol. Despite empirical antibiotics, his condition persisted, and a lip biopsy confirmed IgG4-related disease (IgG4-RD). Systemic corticosteroids were initiated, and the patient was transitioned from IV epoprostenol to a combination of IV and inhaled treprostinil; IgG4-RD has remained in remission to date, with normalisation of serum IgG4. This case highlights a practical, minimally invasive diagnostic approach for suspected IgG4-RD in severe PAH and demonstrates the feasibility of prostacyclin modification to sustain PAH control while reducing immunosuppression.
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