Multiple Hepatic Aneurysm and Dry Gangrene of Fingertips in Eosinophilic Granulomatosis with Polyangiitis (EGPA)

As EGPA is a systemic necrotizing vasculitis affecting mainly small-sized arteries, clinical features of medium-sized vasculitis was very rare in EGPA. Here we present that multiple hepatic aneurysms and distal gangrene presented in the same patient with EGPA. The angiography was performed because of hepatic subcapsular hematoma. The patient treated for EGPA presented to the emergency room with abrupt squeezing abdominal pain. She have experienced sudden onset of gangrene in both fingertips one year ago because of arterial thrombosis when her eosinophil count increased up to 6,000 cells/μL. Her absolute eosinophil count in ER was 1,120 cells/μL. The abdomen-pelvis CT demonstrated hepatic subcapsular hematoma in right lobe caused by suspicious aneurysmal rupture. We immediately performed angiography. The celiac angiogram demonstrated multiple sized hepatic aneurysms in both lobe and some of aneurysm in S7 and S8 were huge, even more than 1 cm. The appearance of small aneurysms was string of beads, generally observed in polyarteritis nodosa. Given the clinical situations, the emergency embolization was done. This patient has been treated with relatively high dose steroid, azathioprine and cyclophosphamide. After mepolizumab started, eosinophil count showed stable state with near zero percentage. Small aneurysm and gangrene can occur in EGPA. Deconstruction of vessel associated with vasculitis can be active when eosinophil count is even below 1,500 cells/μL. If medium sized arteries is suspected to be involved in EPGA with distal ischemia, thorough control of eosinophilia with several immunosuppression might be needed to prevent from the fatal complication.