【摘要】 目的 分析抗中性粒细胞胞浆抗体(ANCA)相关性小血管炎(AAV)继发血栓性血小板减少性紫癜(TTP)的临床特点。方法 对9例AAV继发TTP患者的一般情况、临床表现、辅助检查、治疗与转归进行综合分析,包括北京大学人民医院1例及文献报道8例。结果 9例患者中,男1例、女8例;确诊年龄为17~77岁,65岁以上7例。其中显微镜下多血管炎(MPA)7例,肉芽肿性多血管炎1例,未明确分类1例。①临床表现:3例患者出现发热;4例有神经系统症状,其中意识障碍3例,外周神经系统受累2例;所有患者均有肾脏病变,其中7例表现为急进性肾小球肾炎、1例为急性肾损伤、1例为慢性肾小球肾炎。5例患者有肺部病变,其中3例为弥漫性肺泡出血,2例为双侧胸腔积液;3例患者具有典型的TTP五联征(发热、血小板减少、微血管病性溶血性贫血、神经系统症状和肾脏损害),1例患者具有三联征(血小板减少、微血管病性溶血性贫血和神经系统症状)。②辅助检查:8例患者髓过氧化物酶-ANCA或p-ANCA阳性,1例患者c-ANCA阳性;ANCA数值均大于100,滴度均高于1:320。3例患者的血小板计数(PLT)<50×10~9/L,其中2例PLT<20×10~9/L;2例PLT为50~100×10~9/L;4例PLT>100×10~9/L。1例患者血红蛋白<60 g/L,2例血红蛋白为60~90 g/L,3例血红蛋白>90 g/L。4例患者检测血管性血友病因子裂解酶,均低于正常值。③治疗:7例患者采用血浆置换治疗,余2例采用血浆输注。8例患者采用甲泼尼龙冲击治疗,余1例仅采用口服泼尼松。5例使用环磷酰胺,1例使用硫唑嘌呤。1例难治性患者使用利妥昔单抗。1例应用丙种球蛋白。④转归:6例患者存活,2例死亡,1例自动出院。结论 AAV继发TTP极为罕见,老年女性多见,原发病多为MPA。患者较少出现典型的TTP五联征或三联征,且AAV易将TTP症状掩盖,延误诊断。患者多有肺、肾损伤,TTP会加重AAV相关的肺、肾损伤。血浆置换是首选治疗,同时予激素冲击治疗,肺、肾损伤严重者可加用免疫抑制剂。AAV继发TTP是AAV高度活动的表现,预后不良。更多还原

【Abstract】 Objective To analyze clinical features of thrombotic thrombocytopenic purpura(TTP) secondary to antineutrophil cytoplasmic antibody(ANCA) associated vasculitis(AAV).Methods Nine cases of TTP secondary to AAV were summarized(general information,clinical manifestations,treatments and outcomes),including 1 case from Peking University People’s Hospital and 8 cases from previous literatures.Results There were 1 male and 8 female patients,17-77 years old,7 patients were over 65;including 7 cases of microscopic polyangiitis(MPA),1 case of granulomatous polyangiitis and 1 case without specific type.①Clinical manifestations;3 patients had fever;4 patients had neurological symptoms,3 of them had consciousness disorder and 2 of them had peripheral nervous system disorders;all patients had renal injury,7 patients had rapidly progressive glomerulonephritis,1 patient had acute kidney injury and 1 patient had chronic glomerulonephritis;5 patients had pulmonary injury,3 of them had diffuse alveolar hemorrhage and 2 of them had bilateral pleural effusion.Five typical syndromes of TTP(fever,thrombocytopenia,microangiopathic hemolytic anemia,neurological symptoms and renal injury) occurred in 3 patients;triad syndromes(thrombocytopenia,microangiopathic hemolytic anemia and neurological symptoms) occurred in 1 patient.②Results of auxiliary examinations;8 patients had positive myeloperoxidase-ANCA or p-ANCA and 1 patient had positive c-ANCA,ANCA values were over 100,ANCA titers were over 1:320;3 patients had platelet count(PLT) <50 × 10~9/L,2 patients had PLT <20 × 10~9/L,2 patients had PLT 50-100 × 10~9/L,4 patients had PLT > 100 × 10~9/L;1 patient had hemoglobin < 60 g/L,2 patients had hemoglobin 60-90 g/L,3 patients had hemoglobin >90 g/L;4 patients were detected von Willebrand factor lyase ADAMTS13,the results were below normal range.③Treatments;7 patients had plasma exchange and 2 patients had plasma infusion;8 patients had methylprednisolone pulse therapy and 1 patient took prednisone orally;5 patients had cyclophosphamide and 1 patient had azathioprine;1 refractory patient was treated with rituximab;1 patient had gamma globulin.④Outcomes;6 patients survived,2 patients dead,1 patient abandoned treatment and discharged.Conclusions TTP secondary to AAV is rare and elderly females are more.MPA is the most common primary disease of AAV.Typical TTP five syndromes and triad syndromes are infrequent and AAV associated symptoms may cover TTP.Patients often have pulmonary and renal injury.Plasma exchange is the first preferred therapy and pulse-dosage steroid is also recommended;immunosuppressive agents should be used in patients with severe pulmonary or renal injury.TTP secondary to AAV has poor prognosis.更多还原