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Antineutrophil cytoplasmic antibody-associated crescentic glomerulonephritis with membranous nephropathy treated using thiamazole

机译:噻唑治疗抗中性粒细胞胞浆抗体相关的新月型肾小球肾炎合并膜性肾病

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A 21-year-old woman with nephrotic syndrome was referred to our hospital. She had congenital diaphragmatic hernia, hypoxic ischemic encephalopathy, and mental retardation, and had been treated for hyperthyroidism with thiamazole in another hospital. Serum creatinine was 37.8 μmol/L and antineutrophil cytoplasmic antibody against myeloperoxidase (MPO-ANCA) was 39 EU. Urinalyses were 3+ for proteins and 3+ for occult blood. A renal biopsy was performed. An examination using light microscopy (LM) revealed necrotizing glomerulonephritis with crescent formation. Immunofluorescence microscopy showed granular staining with immunoglobulin G and complement component 3 along the capillary walls. Electron microscopy (EM) disclosed subepithelial dense deposits. A renal biopsy suggested necrotizing glomerulonephritis with membranous nephropathy (MN) in stages I or II. Since many cases of drug-induced ANCA-associated glomerulonephritis (AAG) have been reported, we stopped thiamazole and treated with corticosteroid. The MPO-ANCA titer became negative 49 days after the initiation of treatment. Two years after the first treatment, the MPO-ANCA titer became elevated again and was 82 EU. The patient was administered cyclophosphamide and prednisone. However, the MPO-ANCA titer did not decrease. A renal biopsy was performed again 3 years after the first renal biopsy. LM revealed no crescentic formation but demonstrated spike formations along the glomerular basement membrane. EM also disclosed subepithelial dense deposits, but less than the first biopsy. The renal biopsy suggested MN in stages II or III. AAG was regarded as inactive after corticosteroid treatment. Therefore, ciclosporin administration was started. In conclusion, we experienced a rare case of AAG complicated with MN. The histopathologic results showed that immunosuppressive therapy seemed to be effective in treating crescentic glomerulonephritis; furthermore, it reduced proteinuria but could not reduce the MPO-ANCA titer.
机译:一名21岁的肾病综合征妇女被转诊至我院。她患有先天性diaphragm肌疝,缺氧缺血性脑病和智力低下,并在另一家医院接受了噻唑治疗甲亢。血清肌酐为37.8μmol/ L,抗髓过氧化物酶(MPO-ANCA)的抗中性粒细胞胞浆抗体为39EU。尿液分析的蛋白质为3+,隐血的为3+。进行肾脏活检。使用光学显微镜(LM)的检查显示坏死性肾小球肾炎伴新月形形成。免疫荧光显微镜检查显示,沿毛细血管壁用免疫球蛋白G和补体成分3进行了颗粒染色。电子显微镜(EM)揭示了上皮下致密沉积物。肾脏活检提示I或II期坏死性肾小球肾炎合并膜性肾病(MN)。由于已经报道了许多药物引起的ANCA相关性肾小球肾炎(AAG),因此我们停止了噻唑并应用皮质类固醇治疗。开始治疗后49天,MPO-ANCA滴度变为阴性。首次治疗两年后,MPO-ANCA滴度再次升高,为82欧盟。病人被给予环磷酰胺和泼尼松。但是,MPO-ANCA滴度没有降低。第一次肾脏活检后3年再次进行肾脏活检。 LM显示没有新月形形成,但沿肾小球基底膜显示出尖峰形成。 EM还披露了上皮下密集的沉积物,但少于第一次活检。肾活检提示MN为II或III期。皮质类固醇治疗后,AAG被认为是无活性的。因此,开始使用环孢菌素。总之,我们经历了AAG并发MN的罕见情况。组织病理学结果表明,免疫抑制疗法似乎对治疗新月型肾小球肾炎有效;此外,它降低了蛋白尿,但不能降低MPO-ANCA滴度。

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