关于一例维持性血液透析 5 年后的 EGPA 疑似病例的讨论及相关文献分析:病例报告。

IF 1.3 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Medicine Pub Date : 2024-09-20 DOI:10.1097/MD.0000000000039856
Lingshan Zhao, Chenli Zhang
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引用次数: 0

摘要

理论依据:嗜酸性粒细胞肉芽肿伴多血管炎(EGPA)是一种以外周血和组织坏死性肉芽肿炎症为病理特征,伴有嗜酸性粒细胞增多并浸润中小血管的疾病,是一种与全身性血管炎相关的罕见抗中性粒细胞胞浆抗体(ANCA)家族病。由于发病率低、临床表现多样、早期诊断困难,大多数患者在多器官受损后才被确诊,因此错过了最佳治疗时间,预后较差。据报道,约有25%至30%的EGPA病例伴有肾脏疾病,而关于EGPA并发肾脏损害的研究较少,其中大部分是针对ANCA阳性患者。一般来说,在维持性血液透析中初步诊断出 EGPA 的病例更是少之又少。我们报告了一例维持性血液透析 5 年后被诊断为 EGPA 的患者:患者女性,54 岁,连续维持血液透析 5 年,过去 3 年因反复皮疹住院,最近 2 个月病情加重。既往有支气管哮喘病史,近期经常发作,从外周血可观察到嗜酸性粒细胞增多,从心脏彩超可观察到易患嗜酸性粒细胞性心内膜炎,从血管炎五项检查可观察到 P-ANCA 和 MPO-AB 阳性:患者发病时肾功能不全,维持性血液透析5年,反复肺部感染,合并眼部病变,散在皮疹,P-ANCA阳性,MPO-AB阳性,哮喘存在,嗜酸性粒细胞绝对值1.60×109/L,总分>6分,诊断考虑EGPA:由于多器官受损,患者接受了类固醇和环磷酰胺联合治疗:2天后,患者皮疹颜色较前明显加深,喘息改善,嗜酸性粒细胞恢复正常水平:启示:对于肾衰竭患者,首先应将 ANCA 检测提上日程。同时,透析患者的免疫监测被忽视也告诉我们,对这类患者进行免疫监测对早期诊断 EGPA 具有重要意义。
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A discussion on a suspected case with EGPA after maintenance hemodialysis for 5 years and related literature analysis: A case report.

Rationale: Pathological featuring by necrotizing granulomatous inflammation of peripheral blood and tissues with increased eosinophils infiltrating small and medium vessels, eosinophilic granulomatosis with polyangiitis (EGPA), a family of rare antineutrophil cytoplasmic antibody (ANCA) associated with systemic vasculitis. With low morbidity, diverse clinical manifestations, and difficult early diagnosis, the majority of patients are confirmed after multiple organ damages, thus missing the best treatment time and having a poor prognosis. About 25% to 30% of EGPA cases have been reported to suffer from the renal disease, and there are few studies on EGPA complicated with kidney damage, most of them on ANCA-positive patients. Generally, the initial diagnosis of EGPA on maintenance hemodialysis is even rare. We report a case of a patient with maintenance hemodialysis for 5 years and then was diagnosed with EGPA.

Patient concerns: The female patient, 54-year-old, having maintenance hemodialysis for 5 years consecutively, was hospitalized for the recurring rash in the past 3 years and then exacerbation in the last 2 months. With the previous history of bronchial asthma having attacked frequently recently, it could be observed from peripheral blood that the eosinophils increased, from the cardiac color ultrasound that it was prone to eosinophilic endocarditis, from 5 tests for vasculitis that P-ANCA and MPO-AB were positive.

Diagnoses: The patient's onset is renal dysfunction, with maintenance hemodialysis for 5 years, recurrent lung infections, combined with eye lesions, scattered skin rashes, P-ANCA positive, MPO-AB positive, asthma present, eosinophil absolute value 1.60 × 109/L, total score >6 points, diagnosis considering EGPA.

Interventions: Due to multiple organ damage, the patient received treatment with a combination of steroids and cyclophosphamide.

Outcomes: After 2 days, the patient's rash significantly darkened compared to before, wheezing improved, and eosinophils returned to normal levels.

Lessons: The ANCA test shall be put on the high agenda for patients presenting with kidney failure at first. Meanwhile, the neglected immune monitoring for patients with dialysis tells us that it is of great significance for this kind of patient to have immune monitoring in the early diagnosis of EGPA.

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来源期刊
Medicine
Medicine 医学-医学:内科
CiteScore
2.80
自引率
0.00%
发文量
4342
审稿时长
>12 weeks
期刊介绍: Medicine is now a fully open access journal, providing authors with a distinctive new service offering continuous publication of original research across a broad spectrum of medical scientific disciplines and sub-specialties. As an open access title, Medicine will continue to provide authors with an established, trusted platform for the publication of their work. To ensure the ongoing quality of Medicine’s content, the peer-review process will only accept content that is scientifically, technically and ethically sound, and in compliance with standard reporting guidelines.
期刊最新文献
A case of metachronous triple primary carcinoma complicated with pulmonary tuberculosis: Case report and review. A cross-sectional study of the association between blood cadmium and mortality among adults with myocardial infarction. A discussion on a suspected case with EGPA after maintenance hemodialysis for 5 years and related literature analysis: A case report. A rare case report of endobronchial neurofibroma treated with transbronchial endoscopic resection and literature review. A retrospective comparative case series of efficacy and safety of radiofrequency thermocoagulation versus drug therapy in patients with trigeminal neuralgia: A clinical case report.
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