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以系膜区免疫球蛋白G沉积为主的原发性肾小球肾炎。

Primary glomerulonephritis with predominant mesangial Immunoglobulin G deposits.

作者信息

Kharroubi Melek, Ben Fatma Lilia, Rais Lamia, Jebali Hela, Mami Ikram, Zouaghi Mohamed Karim

出版信息

Tunis Med. 2018 Jul;96(7):442-444.

PMID:30430489
Abstract

BACKGROUND

Glomerular deposits are a major pathologic feature of a wide range of human glomerulonephritis and may be located in the mesangial, subepithelial, and subendothelial regions. Rare cases of primary glomerulonephritis definied by exclusive or predominant mesangial IgG deposits were reported. We reviewed the pathologic findings for the 848 renal biopsies examined in our department between 2007 and 2016, one case of primary mesangial IgG glomerulonephritis (MIG) in the absence of any evidence of systemic lupus erythematous (SLE), of other systemic diseases or of Infections.

REPOTED CASE

Male patient aged 55 years consults for nephrotic syndrome (proteinuria=7g/1.73m2/day; Albuminemia=14g/L) with hematuria.. The serum creatinine concentration was 88 µmol/l, and the creatinine clearance was on MDRD at 82 ml/min/1.73m2. He had a renal biopsy showing the absence of proliferative lesions and the presence of exclusive mesangial IgG deposit. CH50, C3 and C4 levels were normal and antinuclear antibody and anti-DNA antibody assays remained negative during the follow-up period. A full dose corticosteroid was initiated (1mg/Kg/day). The interval from onset of steroid therapy to remission was 2 months. During follow-up; the patient had developed 2 relapsing nephrotic syndrome episods. Then he was switched to Mycophenolate mofetil with remission after 2 months.

CONCLUSION

MIG is a very rare but distinct type of primary glomerulonephritis that is characterized by exclusive or predominant mesangial IgG deposits. Its renal prognosis may be less favorable than previously reported because of the possibility of steroids resistance of the nephrotic syndrome and the recurrence after renal transplantation.

摘要

背景

肾小球沉积物是多种人类肾小球肾炎的主要病理特征,可位于系膜、上皮下和内皮下区域。有报道称,少数原发性肾小球肾炎病例表现为单纯或主要为系膜IgG沉积。我们回顾了2007年至2016年间在我科检查的848例肾活检病理结果,发现1例原发性系膜IgG肾小球肾炎(MIG),无任何系统性红斑狼疮(SLE)、其他系统性疾病或感染的证据。

报告病例

一名55岁男性患者因肾病综合征(蛋白尿=7g/1.73m²/天;白蛋白血症=14g/L)伴血尿前来就诊。血清肌酐浓度为88µmol/l,肌酐清除率根据MDRD公式计算为82ml/min/1.73m²。他接受了肾活检,结果显示无增殖性病变,仅存在系膜IgG沉积。在随访期间,CH50、C3和C4水平正常,抗核抗体和抗DNA抗体检测仍为阴性。开始使用全剂量皮质类固醇(1mg/kg/天)治疗。从开始使用类固醇治疗到缓解的间隔时间为2个月。在随访期间,患者出现了2次肾病综合征复发。随后改用霉酚酸酯,2个月后病情缓解。

结论

MIG是一种非常罕见但独特的原发性肾小球肾炎,其特征为单纯或主要为系膜IgG沉积。由于肾病综合征可能对类固醇耐药以及肾移植后复发,其肾脏预后可能比先前报道的更差。

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