척-스트라우스증후군(eosinophilic granulomatosis with polyangiitis) 환자에서 성공적으로 수행한 심장이식 1예
Heart transplantation in a patient with eosinophilic granulomatosis with polyangiitis known as Churg-Strauss syndrome
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초록
Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome, is a rare systemic necrotizing vasculitis affecting small- to medium-sized vessels. EGPA is associated with severe asthma and eosinophilia. The most frequently involved organs are skin and peripheral nerves; however, EGPA may involve other organs, such as the gastrointestinal tract, kidney, and heart. Antineutrophil cytoplasm antibodies (ANCAs)-related abnormal immune reactions are known to be associated with EGPA, but only 30%–40% of patients have a positive marker of ANCA. ANCA-negative patients are at higher risk of cardiac involvement than ANCA-positive patients. Cardiac involvement is one of the leading causes of mortality and could be resistant to conventional treatment. Early treatment with steroid plus cyclophosphamide is important because it could give chances of restoration of cardiac function. For patients undergoing heart transplantation, we should consider the severity of cardiac disease and the presence of systemic diseases, including vasculitis. Here, we report a case of a 25-year-old EGPA patient with cardiac involvement who eventually received heart transplantation for progressive heart failure, although treated with systemic corticosteroid with cyclophosphamide. EGPA patients undergoing heart transplantion are rarely reported worldwide, and this is the first case report in Korea.
Abstract
Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome, is a rare systemic necrotizing vasculitis affecting small- to medium-sized vessels. EGPA is associated with severe asthma and eosinophilia. The most frequently involved organs are skin and peripheral nerves; however, EGPA may involve other organs, such as the gastrointestinal tract, kidney, and heart. Antineutrophil cytoplasm antibodies (ANCAs)-related abnormal immune reactions are known to be associated with EGPA, but only 30%–40% of patients have a positive marker of ANCA. ANCA-negative patients are at higher risk of cardiac involvement than ANCA-positive patients. Cardiac involvement is one of the leading causes of mortality and could be resistant to conventional treatment. Early treatment with steroid plus cyclophosphamide is important because it could give chances of restoration of cardiac function. For patients undergoing heart transplantation, we should consider the severity of cardiac disease and the presence of systemic diseases, including vasculitis. Here, we report a case of a 25-year-old EGPA patient with cardiac involvement who eventually received heart transplantation for progressive heart failure, although treated with systemic corticosteroid with cyclophosphamide. EGPA patients undergoing heart transplantion are rarely reported worldwide, and this is the first case report in Korea.
참고문헌 (18)
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