Case Report: sustained five-year remission in eosinophilic granulomatosis with polyangiitis with intestinal perforation after surgery and rituximab-based therapy without glucocorticoid escalation

Résumé fourni par la source

Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic necrotizing vasculitis characterized by eosinophilic infiltration and granuloma formation, affecting multiple organs. Gastrointestinal (GI) involvement is relatively uncommon and it typically presents with nonspecific symptoms, such as abdominal pain or diarrhea; in contrast, ulceration and intestinal perforation are rare, but potentially life-threatening complications that often require surgical intervention. The standard treatment for severe GI EGPA includes high-dose glucocorticoids combined with cyclophosphamide or rituximab (RTX). However, the perioperative escalation of glucocorticoids is generally avoided owing to the increased risk of postoperative complications. We report a case with a 5-year follow-up of EGPA resistant to multiple immunosuppressive agents with severe GI involvement, including intestinal perforation and multiple jejunal ulcers, which was successfully treated with RTX for postoperative remission induction and long-term maintenance therapy without any prednisolone escalation. The prednisolone dose was gradually reduced from 17.5 mg/day to 1 mg/day over two years without any escalation, and a sustained remission was achieved throughout the course. This case suggests that RTX may represent a viable therapeutic option for severe or treatment-resistant EGPA in cases with GI involvement when glucocorticoid escalation is undesirable or unsafe, such as in the perioperative setting.

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