以消化道出血为主要症状的中性粒细胞胞质抗体相关性血管炎1例报告及文献复习

Anti-neutrophil cytoplasmic antibody-associated vasculitis with gastrointestinal bleeding as the main symptom:a case report and literature review

抗中性粒细胞胞质抗体相关性血管炎(AAV)具有极其广泛的症状,临床医生很难做出快速而正确的诊断。2021年11月11日宜昌市中心人民医院急诊与危重症医学科收治1例36岁男性AAV患者,以胃肠道症状(腹痛、黑便)为主要体征收入急诊重症监护病房(EICU),初步诊断为AAV合并消化道出血(GIH)。经过多次胃镜及肠镜检查均未找到出血点;腹部发射型CT(ECT)提示回肠、升结肠及横结肠弥漫性出血。全院多学科会诊考虑为AAV致消化道小血管病变引起的弥漫性出血,遂采用甲泼尼龙1000 mg/d冲击治疗+环磷酰胺(CTX)0.2 g/d免疫抑制治疗,患者症状迅速缓解并转出EICU。经过17 d住院治疗,患者最终因消化道大出血死亡。结合病例诊疗过程并对相关文献进行系统回顾发现,只有少数AAV患者以胃肠道症状为首发症状,并发GIH的患者更为罕见,此类患者预后较差。本例患者因治疗消化道出血而推迟了使用诱导缓解+免疫抑制剂,这可能是AAV继发GIH危及生命的主要原因。胃肠道出血是血管炎的一种罕见致命性并发症,及时有效的诱导缓解治疗是生存的关键,患者是否应该接受维持治疗、维持治疗的时间及寻找疾病诊断和治疗反应的标志物是进一步研究的方向和挑战。

Anti-neutrophil cytoplasmic antibody-associated vasculitis(AAV)has a wide range of symptoms,and it is difficult for clinicians to make a quick and correct diagnosis.On November 11,2021,a 36-year-old male patient with AAV was admitted to the emergency and critical care department of Yichang Central People's Hospital.He was admitted to the emergency intensive care unit(EICU)with gastrointestinal symptoms(abdominal pain,black stool)as the main physical signs,and was initially diagnosed as AAV with gastrointestinal hemorrhage(GIH).No bleeding point was found after repeated gastroscopy and colonoscopy.Abdominal emission CT(ECT)showed diffuse hemorrhage in the ileum,ascending colon and transverse colon.Multi-disciplinary consultation in the whole hospital considered the diffuse hemorrhage caused by small vascular lesions in the digestive tract caused by AAV.Pulse therapy with methylprednisolone 1000 mg/d and immunosuppressive therapy with cyclophosphamide(CTX)0.2 g/d were administered.The patient's symptoms quickly relieved and transferred out of the EICU.After 17 days of treatment,the patient finally died of massive gastrointestinal bleeding.A systematic review of relevant literatures combined with the case diagnosis and treatment process found that only a minority of AAV patients present with gastrointestinal symptoms as their first symptoms,and patients with GIH were very rare.Such patients had a poor prognosis.This patient delayed the use of induced remission and immunosuppressive agents due to the treatment of gastrointestinal bleeding,which may be the main cause of life-threatening GIH secondary to AAV.Gastrointestinal bleeding is a rare and fatal complication of vasculitis.Timely and effective induction and remission treatment is the key to survival.Whether patients should receive maintenance therapy,the duration of maintenance therapy,and the search for markers of disease diagnosis and treatment response are directions and challenges for further research.