Systemic necrotizing vasculitis presenting as gangrene combined with diabetes insipi-dus:a case report

1 临床资料患者,男性,64 岁,主因"多饮、多尿,伴烦渴半年,肢端疼痛、变黑3 个月"于2014 年2 月17 日入院. 半年前饮水量和尿量由1~2L/d增至6~7L/d,伴烦渴,饮水后可缓解. 3 个月前出现四肢肢端剧烈疼痛,伴感觉减退,局部皮温降低,此后肢端逐渐变黑,部分有破溃、流脓,双手遇冷水变白变红,无口腔、外阴溃疡,无关节肿痛,无光过敏、皮疹,无咳嗽、咯血.SUMMARY The male patient reported here presented as gangrene and central diabetes insipidus ( CDI) , who had characteristics of vasculitis. The patient complained about polydipsia and polyuria half a year ago, and then developed tingling, pain and blackish discoloration of some fingers and toes 3 month ago. He also had Raynaud' s phenomenon. After admission, his laboratory examination showed the rise of erythrocyte sedimentation rate, C-reactive protein, immunoglobulin,β2-glycoproteinⅠand the activi-ty of rheumatoid factors, lupus anticoagulant test. his pituitary gland showed loss of posterior signal on magnetic resonance imaging. In addition, his vasopressin test was active. However, there was no suffi-cient evidence to diagnose any specific disease;as a consequence the patient was diagnosed as idiopathic systemic necrotizing vasculitis ( SNV) . For SNV, the patient was treated with glucocorticoid 40 mg/d and impact therapy of cyclophosphamide 0. 4 g every 2 weeks. He also received symptomatic treatment for gangrene and CDI. Cutaneous involvement leading to gangrene was widely reported in SNV, however pi-tuitary involvement in SNV leading to CDI was rare. The prognosis of this patient was poor.PubMed中文核心期刊要目总览(PKU)中国科技核心期刊(ISTIC)中国科学引文数据库(CSCD)61028-10304