Abstract
BACKGROUNDEosinophilic granulomatosis with polyangiitis (EGPA) is a systemic small-vessel vasculitis characterized by the presence of asthma and eosinophilia. Because cerebral aneurysm formation induced by EGPA is a rare occurrence, there is no established treatment strategy for this condition.OBSERVATIONSA 67-year-old female who was diagnosed with idiopathic eosinophilia 3 months ago developed de novo fusiform aneurysms in the left vertebral, left internal carotid, and bilateral superficial temporal arteries, as noted during a regular follow-up examination of a convexity meningioma. Pathological examination of the resected superficial temporal artery revealed eosinophilic granulomas, which led to the diagnosis of EGPA, as well as EGPA-induced aneurysm formation. As the partially thrombosed vertebral artery fusiform aneurysm enlarged, the compression of the medulla oblongata occurred despite intensive immunosuppressive therapy for 1 year. The patient underwent flow diversion therapy administered using the pipeline embolization device, resulting in complete disappearance of the aneurysm.LESSONSConsidering that the entire circumference of the aneurysmal wall is affected by necrotizing vasculitis, flow diverter therapy would be a reasonable and efficient approach for the treatment of EGPA-related aneurysms in cases in which the patient is nonresponsive to immunosuppressants.
Highlights
Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic small-vessel vasculitis characterized by the presence of asthma and eosinophilia
We searched the relevant literature in Medline database using the following keywords: cerebral aneurysm, eosinophilic granulomatosis with polyangiitis, and/or Churg-Strauss syndrome
According to our literature review, 44.8 years was the mean patient age at the time of aneurysmal detection, including ruptured and unruptured aneurysms, which suggests that cerebral aneurysms accompanied by EGPA are more common in younger patients
Summary
Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic small-vessel vasculitis characterized by the presence of asthma and eosinophilia. Because cerebral aneurysm formation induced by EGPA is a rare occurrence, there is no established treatment strategy for this condition. LESSONS Considering that the entire circumference of the aneurysmal wall is affected by necrotizing vasculitis, flow diverter therapy would be a reasonable and efficient approach for the treatment of EGPA-related aneurysms in cases in which the patient is nonresponsive to immunosuppressants. KEYWORDS eosinophilic granulomatosis with polyangiitis; Churg-Strauss syndrome; vasculitis; fusiform aneurysm; flow diverter. Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a rare systemic antineutrophil cytoplasmic antibody-associated vasculitis accompanied by asthma and eosinophilia.[1]. It is characterized by eosinophilic necrotizing inflammation involving small-sized vessels. We report a case of EGPA-related cerebral aneurysm treated successfully with flow diverter therapy, and present some of the characteristics and treatment of EGPA-related cerebral aneurysms with a literature review
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