ArticlePulmonary circulation2026
A Case of IgG4-Related Disease Developing During Long-Term High-Dose Intravenous Epoprostenol Therapy in a Patient With Idiopathic Pulmonary Arterial Hypertension.
Article in Pulmonary circulation, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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1 citing paper in PubMed.
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Authors and funding
10 authors.
Funding
No grant is acknowledged in the PubMed record.
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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