Abstract Background Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare, multisystem autoimmune disorder characterized by adult-onset asthma, peripheral eosinophilia, and small- to medium-vessel vasculitis. Cardiac involvement is a leading cause of mortality in EGPA. A case exhibiting progressive enlargement of coronary aneurysms despite normal peripheral eosinophil counts and treatment consistent with guideline-recommended immunosuppressive therapy is exceedingly rare. Case Summary A 55-year-old woman with a seven-year history of EGPA presented to our institution with acute chest pain. Four years prior to the current presentation, the patient underwent coronary angiography for acute coronary syndrome at another hospital, which revealed coronary ectasia. Consequently, low-dose aspirin was initiated as secondary prevention. Following the diagnosis of EGPA, guideline-directed immunosuppressive therapy was maintained. Throughout the four-year follow-up period, her peripheral eosinophil counts remained within the normal range. An emergent coronary angiography, prompted by a diagnosis of non-ST elevation myocardial infarction, demonstrated progression to a giant coronary aneurysm. Coronary computed tomography angiography also showed thrombus within the dilated coronary artery. Given the likely etiology of coronary thrombosis, anticoagulation therapy with warfarin was initiated in addition to her ongoing aspirin regimen. The patient remained clinically stable during hospitalization, experienced no recurrent chest pain, and was discharged on the fourth day. Discussion We reported a rare case of an EGPA-related coronary artery aneurysm longitudinally monitored through serial coronary angiography. This case demonstrates that EGPA-related coronary aneurysms may progress despite adherence to therapeutic guidelines and persistently normal eosinophil counts, emphasizing the importance of continuous imaging surveillance and individualized management strategies.
Okamoto et al. (Sat,) studied this question.