Eosinophilic gran with polyangiitis

• Eosinophilic granulomatosis with polyangiitis

• EGPA,

• formerly Churg-Strauss

• Similar to GPA w/ more frequent cardiac involvement,

• a/w asthma and eosinophilia

Epi

rare (incidence 2/million/y); any age (typically 30-40 y); ♂ = ♀; a/w HLA-DRB4

Clinical manifestations of Eosinophilic gran with polyangiitis

• Dx studies: 50% ⊕ ANCA (MPO >PR3), eosinophilia (>1500/uL or 10%, often >60%), biopsy → microgranulomas, fibrinoid necrosis, small artery/vein thromboses w/ eosinophilic infiltrate

Treatment

• high-dose steroids + mepolizumab (anti-IL-5) (if nonsevere) or
• RTX or
• CYC (if severe)
• (Arth Rheum 2021;73:1366);
• mepolizumab for relapse/refractory (NEJM 2017;376:1921)