Multiple hepatic aneurysms and dry gangrene of fingertips in eosinophilic granulomatosis with polyangiitis: a case repor
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Allergy, Asthma & Clinical Immunology Open Access
CASE REPORT
Multiple hepatic aneurysms and dry gangrene of fingertips in eosinophilic granulomatosis with polyangiitis: a case report Eunsil Koh1, Noeul Kang1, Jin‑Young Lee3, Duk‑Kyung Kim1, Young Soo Do2, Byung‑Jae Lee1 and Dong‑Chull Choi1*
Abstract Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic necrotizing vasculitis mainly affecting small-sized arteries. Involvement of medium-sized vessels is very rare in EGPA. Here we present the case of a patient with EGPA who showed multiple hepatic aneurysms and distal gangrene. Case presentation: A known EGPA patient visited to the emergency room (ER) with abrupt squeezing abdominal pain. She had suffered from gangrene in the fingertips of both hands for 1 year because of arterial thrombosis associated with hypereosinophilia. However, her absolute eosinophil count in the ER was 1120 cells/µL. An abdomenpelvis CT demonstrated subcapsular hematoma in the right hepatic lobe. A celiac angiogram demonstrated multiple sized aneurysms in both hepatic lobes and some aneurysms in S7 and S8 were huge, more than 1 cm in size. The shape of the small aneurysms resembled a string of beads, as in polyarteritis nodosa. Given the clinical situation, emergency embolization was performed. Before this patient visited to the ER, she had been treated with a high dose of systemic corticosteroid, azathioprine, and cyclophosphamide. After addition of mepolizumab, the eosinophil count remained stable state with a near zero percentage of total white blood cell count. Conclusions: Aneurysm and gangrene resulting from the involvement of medium-sized vessels can occur in EGPA. Destruction of vessels might occur even if eosinophil count is below 1500 cells/µL. If involvement of medium-sized arteries is suspected, thorough investigation to identify the involved organs and prompt management are needed to prevent fatal complications. Keywords: Eosinophilic granulomatosis with polyangiitis, EGPA, Aneurysm, Gangrene, Mepolizumab Background Eosinophilic granulomatosis with polyangiitis (EGPA), formerly named the Churg-Strauss syndrome, is a rare systemic necrotizing vasculitis with eosinophilia. EGPA affects small- to medium-sized blood vessels of various organs [1]. Vasculitis is classified by the size of the predominantly affected vessel, and characteristic *Correspondence: [email protected] 1 Departments of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea Full list of author information is available at the end of the article
manifestations vary depending on the classification of vasculitis [2]. Gangrene or aneurysms, known to be clinical features of medium-sized vasculitis, scarcely present in ANCA-associated vasculitis of small sized vasculitis [3, 4]. Blood eosinophil count of EGPA patients is usually about 5000 cells/µL (reference value
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