항호중구 세포질 항체 음성인 Churg-Strauss 증후군 1예
A Case Report of Anti-Neutrophil Cytoplasmic Antibodies Negative Churg-Strauss Syndrome
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초록
Churg-Strauss 증후군은 드문 원발성 혈관염으로 알레르기-육아종성 혈관염으로 알려져 있다. 여러 가지 혈관염들은 중복되는 특성이 많기 때문에 감별진단은 임상소견과 검사소견을종합하여 이루어진다. 저자들은 54세 여자 환자로 기관지 천식, 호산구 증가, 다발성 신경병증, 폐침윤, 부비동염 그리고 피부 조직검사상 혈관외 호산구 침윤을 보인 Churg-Strauss 증후군 1예를 보고한다. 환자는 면역혈청검사에서 항호중구 세포질 항체(antineutrophil cytoplasmic antibodies, ANCA)는 음성이었으며, 발열을 동반하고 흉부방사선 검사에서 심비대와 폐부종이 관찰되고 심전도에서 좌심실 비대 소견을 보였다. 환자는 스테로이드 치료로 증상이 빠르게 호전되었다.
Abstract
Churg-Strauss syndrome is a rare primary vasculitis called allergic granulomatosis angiitis. There is a substantial overlap of symptoms or signs between different vasculitides, so that a diagnosis of any vasculitis can be determined by a combination of clinical symptoms and laboratory tests. We report a case of a 54-year-old woman who had bronchial asthma, eosinophilia, peripheral polyneuropathy, pulmonary infiltrate, maxillary sinusitis, and extravascular eosinophilic infiltration on skin biopsy. So she was diagnosed as having Churg-Strauss syndrome, but she was antineutrophil cytoplasmic antibodies-negative on immunologic study. She had fever and showed cardiac involvement that was presented as cardiomegaly and pulmonary edema at chest radiography and left ventricular hypertrophy on electrocardiography. After steroid therapy, general condition and skin manifestation were rapidly improved.
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