抗中性粒细胞胞浆抗体相关血管炎伴非呼吸道出血的临床分析

Analysis of clinical characteristics of anti-neutrophil cytoplasmic antibody associated vasculitis with non-respiratory tract hemorrhage

目的:探讨抗中性粒细胞胞浆抗体(ANCA)相关血管炎(AAV)伴非呼吸道出血的临床特征、诊疗及预后。方法:回顾性整理我院近5年诊治的47例AAV患者的临床病历资料,分析其中合并非呼吸道出血者的诊治过程。结果:47例AAV中合并非呼吸道出血6例(12.7%),中位年龄58岁。2例发生2处出血,共8处出血;消化道出血2例(4.2%),脑出血3例(6.4%),肾出血1例,腹膜后血肿1例,肌肉血肿1例。6例AAV临床表型为显微镜下多血管炎(MPA)4例,肉芽肿性多血管炎(GPA)2例(脑出血2例,肾出血1例)。6例中5例出血是AAV初诊活动期发生,同时伴急进性肾炎;胸部CT多提示双肺间质改变,但无咯血。1例肌肉血肿发生在血管炎复发期。4例MPOANCA强阳性,1例MPO-ANCA中阳性,1例PR3-ANCA强阳性。5例出血发作前已予激素治疗,临床表现较急骤危重,如头痛、腹痛、血便,常伴血红蛋白快速下降,3例休克血压;血小板计数及凝血指标均正常,仅1例血钙偏低。6例继续激素、环磷酰胺治疗,3例并用静脉免疫球蛋白、血浆置换和血液透析,辅以输血、止血(血管介入栓塞或手术)。6例中2例死亡(脑出血1例,消化道出血1例),4例存活者使用激素、免疫抑制剂诱导缓解,2例并用血浆置换治疗好转、脱离血液透析。结论:AAV合并非呼吸道出血的比例达12.7%;以脑出血和消化道出血为主,与本病导致血管损害相关,也是致命性的并发症。早期识别,积极免疫抑制剂治疗联合血浆置换、静脉免疫球蛋白,并止血处理(血管介入栓塞或手术),部分患者经救治可缓解。

Objective： To summarize the clinical features,diagnosis and prognosis of anti-neutrophil cytoplasmic antibody（ ANCA） associated vasculitis（ AAV） with non-respiratory tract hemorrhage. Methods： A total of 47 cases of AAV treated in our hospital in the recent 5 years were searched,and the diagnosis and treatment of those with non-respiratory bleeding were reviewed. Results： Six patients（ 12. 7%） with non-respiratory hemorrhage were found in 47 patients with AAV. The median age was 58 years. Bleeding occurred in 2 sites in 2 cases and a total of 8 sites of bleeding were seen.There were 2 cases of gastrointestinal bleeding（ 4. 2%）,3 cases of cerebral hemorrhage（ 6. 4%）,1 case of renal hemorrhage,1 case of retroperitoneal hematoma and 1 case of muscle hematoma. The clinical phenotypes of 6 cases of AAV included 4 cases of MPA and 2 cases of GPA（ 2 cases of intracerebral hemorrhage and 1 case of renal hemorrhage）. In 6 cases,haemorrhage occurred in 5 cases during the initial active stage of AAV,accompanied by rapidly progressive glomerulonephritis. Thoracic CT showed interstitial lungs but no hemoptysis. Muscle hematoma occurred in one case during the recurrence of vasculitis. Four cases were strongly positive for MPO-ANCA,1 was moderately positive for MPO-ANCA and 1 was strongly positive for PR3-ANCA. Five bleeding episodes have been treated with glucocorticoid,and the clinical manifestations were sudden and critical,such as headache,abdominal pain and bloody stools,often accompanied by rapid decline in hemoglobin. There were 3 cases of shock blood pressure. The counts of platelets and indexes of coagulation function were normal except one case of slightly low blood calcium level. Six patients were given methylprednisolone combined with cyclophosphamide treatment continuously,and 3 given intravenous gamma globulin infusion and plasma exchange,hemodialysis,combined with hemostatic treatment（ selective transarterial embolization or surgery）. After treatment,2 patients died. Four survivors were administrated immunosuppressive therapy continuously to induce remission,and two patients who were subjected to plasma exchange were not given maintenance hemodialysis any more. Conclusions： The proportion of AAV with non-respiratory hemorrhage is up to 12. 7%,mainly presenting with cerebral hemorrhage and gastrointestinal bleeding,which is related to the vascular damage caused by AAV and also a serious life-threatening complication. By immunosuppressive therapy combined with plasma exchange,intravenous gamma globulin infusion,hemostatic treatment（ selective transarterial embolization or surgery）,some AAV patients with non-respiratory hemorrhage could be rescued and achieve remission.