Horita Y, Nazneen A, Cheng M, Razzaque M S, Namie S, Tadokoro M, Taura K, Miyazaki M, Ozono Y, Kohno S, Harada T, Taguchi T
Second Department of Pathology, Nagasaki University School of Medicine, Japan.
Nihon Jinzo Gakkai Shi. 1997 Oct;39(7):759-64.
A case of systemic lupus erythematosus (SLE) associated with minimal change nephrotic syndrome (MCNS) in a 25-year-old female is described. The patient suddenly manifested butterfly rash and proteinuria was first pointed out on March, 1994. On admission, her skin biopsy indicated SLE. Subsequently, she developed nephrotic syndrome. Urinalysis showed heavy proteinuria (4.1 g/day), with no other abnormalities in the urinary sediment. Immunological examination revealed positive antinuclear antibody at a titer of 1:80 with a speckled pattern. Anti-ssDNA and anti-SS-A antibodies were positive, but other antibodies were negative. Serum complement (CH50) was within the normal range (30.5 U/ml). The renal biopsy showed no apparent cellular proliferation or increase of extracellular matrices in glomeruli by light microscopy. Slight deposition of IgG, IgM, C3 and C1q was focally seen in the mesangium and capillary wall by immunofluorescence. Electron microscopic examination revealed small and scattered dense deposits in the mesangium, subepithelium and subendothelium, associated with diffuse fusion of the foot processes of epithelial cells along the glomerular basement membrane. According to the WHO classification, the histological features were compatible with those of lupus nephritis (LN), class Ib. The patient was treated with PREDNISOLONE, Mizorbine and Dilazep, resulting in the disappearance of proteinuria and a normal serum level of total protein. The association of LN and MCNS is very rare. We also investigated the relationship between the intensity of proteinuria and histological types of 53 cases with LN examined in our laboratory. The cases with heavy proteinuria were mostly classified as WHO-Class IV and Class V. We report here a case of LN associated with MCNS and also review the literatures.
本文描述了一名25岁女性系统性红斑狼疮（SLE）合并微小病变肾病综合征（MCNS）的病例。患者于1994年3月突然出现蝶形皮疹，首次发现蛋白尿。入院时，皮肤活检显示为SLE。随后，她发展为肾病综合征。尿液分析显示大量蛋白尿（4.1g/天），尿沉渣无其他异常。免疫检查发现抗核抗体阳性，滴度为1:80，呈斑点状。抗单链DNA和抗SS-A抗体阳性，但其他抗体阴性。血清补体（CH50）在正常范围内（30.5U/ml）。肾活检光镜下肾小球未见明显细胞增殖或细胞外基质增加。免疫荧光显示IgG、IgM、C3和C1q在系膜和毛细血管壁有局灶性轻度沉积。电镜检查显示系膜、上皮下和内皮下有小而散在的致密沉积物，伴有沿肾小球基底膜上皮细胞足突弥漫性融合。根据世界卫生组织（WHO）分类，组织学特征符合狼疮性肾炎（LN）Ib型。患者接受泼尼松龙、咪唑立宾和地拉嗪治疗，蛋白尿消失，血清总蛋白水平恢复正常。LN与MCNS的关联非常罕见。我们还研究了我们实验室检查的53例LN患者蛋白尿强度与组织学类型之间的关系。大量蛋白尿的病例大多分类为WHO-IV级和V级。我们在此报告一例LN合并MCNS的病例，并对相关文献进行综述。
