Amano A, Miyagi K, Sakata M, Sakaeyama Y, Sakaida H, Nozawa F, Azuma T
Department of Internal Medicine, Toyoko Hospital, Saint Marianna University, Kanagawa.
Ryumachi. 1993 Jun;33(3):255-9.
A perforation in the small intestine and one and a half syndrome were observed in 45-year-old male with allergic granulomatosis and angiitis (AGA). The patient was first admitted to our hospital in 1988 with complaints of bronchial asthma and an abnormal density on his chest X ray. The diagnosis of PIE syndrome was made based on marked peripheral blood eosinophilia. He was successfully treated with prednisolone and had been well on the maintenance therapy of small doses of it. In May 1990, peripheral neuropathy occurred in his bilateral lower legs and it was followed by nausea and vomiting. The laboratory findings on admission showed leukocytosis (19,300/mm3) with 43% eosinophilia and elevated blood level of IgE (3000 IU/ml). On the tenth day of admission, ileus symptom and subsequently a perforation of the small intestine were observed. The mesenterial specimens obtained during the emergency operation showed the findings compatible with AGA. Remission of AGA was induced by the treatment with 60 mg prednisolone and 100 mg cyclophosphamide per day following methylprednisolone pulse therapy. He was well on maintenance prednisolone until June 1991, when he suddenly developed one and a half syndrome. Although cranial CT scan and MRI showed no abnormal findings, cyclophosphamide therapy was resumed and prednisolone was increased because microangiopathy due to AGA was thought to be the cause of this central nervous system symptom. The one and a half syndrome improved with this therapy. This case suggests that the long term treatment with prednisolone and cyclophosphamide would be indicated in the severe AGA with close monitoring of the symptoms and peripheral blood eosinophilia.
一名45岁患有变应性肉芽肿性血管炎（AGA）的男性患者出现了小肠穿孔和一个半综合征。该患者于1988年首次因支气管哮喘和胸部X线片密度异常入住我院。根据外周血嗜酸性粒细胞显著增多，诊断为PIE综合征。他接受泼尼松龙治疗成功，且一直通过小剂量维持治疗病情良好。1990年5月，他双侧小腿出现周围神经病变，随后出现恶心和呕吐。入院时实验室检查结果显示白细胞增多（19,300/mm³），嗜酸性粒细胞占43%，血IgE水平升高（3000 IU/ml）。入院第10天，观察到肠梗阻症状，随后出现小肠穿孔。急诊手术中获取的肠系膜标本显示的结果符合AGA。在甲泼尼龙冲击治疗后，每天给予60 mg泼尼松龙和100 mg环磷酰胺治疗，诱导AGA缓解。在1991年6月之前，他通过泼尼松龙维持治疗情况良好，当时他突然出现一个半综合征。尽管头颅CT扫描和MRI未显示异常，但考虑到AGA所致的微血管病被认为是该中枢神经系统症状的病因，于是恢复环磷酰胺治疗并增加泼尼松龙剂量。经此治疗，一个半综合征有所改善。该病例提示，对于重症AGA患者，应采用泼尼松龙和环磷酰胺长期治疗，并密切监测症状和外周血嗜酸性粒细胞情况。
