强免疫抑制治疗抗GBM肾炎最初100%新月形病变的成功治疗

Q4 Medicine
Atthaphong Phongphithakchai, Suntornwit Praditau-Krit, P. Dandecha, Ussanee Boonsrirat, Poowadon Wetwittayakhlung
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引用次数: 0

摘要

抗肾小球基底膜肾炎是一种罕见的肾小球疾病,由靶向肾脏毛细血管床的自身抗体引起。该病的临床表现是一种变异性肾病综合征,可迅速发展为肾小球肾炎。治疗结果取决于首次诊断时的预测因素。我们报告了一例58岁的男性,他没有潜在的疾病,表现为明显的腹胀和急性肾损伤。入院前,他没有慢性肾脏疾病的证据,但实验室检查显示显微镜下血尿(每高倍视野红细胞30-50)、蛋白尿(2.9 g/d)和肾功能衰竭(血清肌酐610µmol/L)与快速进行性肾小球肾炎相容;因此,进行了肾活检。病理显示100%新月体肾小球肾炎,肾小球基底膜处IgG呈线性沉积。最初的血清抗GBM滴度为105.59 RU/mL。该患者有不良的肾脏预后因素作为治疗反应。在与患者讨论了治疗方案后,由于其良好的基线功能状态,我们决定进行强化免疫抑制治疗和血浆置换。患者获得部分缓解,不依赖透析。总之,尽管100%新月体和血清肌酐≥600µmol/L的肾预后较差,但接受治疗的患者具有良好的生存状态,并且没有成为透析依赖性患者。然而,免疫抑制治疗应在仔细监测感染的同时进行,以避免感染相关的发病率和死亡率。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Successful treatment with intense immunosuppressive therapy in an initially 100% crescentic lesion of anti-GBM nephritis
Anti-glomerular basement membrane (anti-GBM) nephritis is uncommon glomerular disease caused by autoantibodies targeting the capillary beds of the kidney. The clinical presentation of the disease is a variable nephritic syndrome, rapidly progressing to glomerulonephritis. Treatment outcomes are dependent on predictors at first diagnosis. We presented a case of 58-year-old man who did not have underlying disease presented with marked abdominal distension and acute kidney injury. He had no evidence of chronic renal disease before admission however, laboratory test showed microscopic haematuria (RBC 30-50 per high-powered field), proteinuria (2.9 g/d), and renal failure (serum creatinine 610 µmol/L) compatible with rapidly progressive glomerulonephritis; hence, a renal biopsy was conducted. The pathology showed 100% crescentic glomerulonephritis with IgG deposits in a linear pattern at the GBM. The initial serum anti-GBM titre was 105.59 RU/mL. This patient had poor renal prognosis factors for treatment response. After a discussion regarding treatment option with the patient, we decided to give intensive immunosuppressive therapy and plasmapheresis due to his good baseline functional status. The patient achieved partial remission and is not dialysis dependent. In conclusion, despite a poor renal prognosis with 100% crescents and serum creatinine ≥ 600 µmol/L, the treated patient had a good survival status and did not become dialysis-dependent. However, immunosuppressive treatment should be performed along with careful monitoring for infection to avoid infection-related morbidity and mortality.
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来源期刊
Journal of Nephropathology
Journal of Nephropathology Medicine-Nephrology
CiteScore
1.30
自引率
0.00%
发文量
35
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