Hamzi Amine Mohamed, Alayoud Ahmed, El Kabbaj Driss, Benyahia Mohamed
Department of Nephrology, Dialysis and Transplantation, Military Hospital Mohammed V, Rabat, Morocco.
Arab J Nephrol Transplant. 2014 Jan;7(1):37-40.
Rare cases of association between lupus nephritis (LN) and minimal changes nephrotic syndrome (MCNS) were described. Some authors suggest that this association, taking into account the low prevalence of both diseases, may not be a simple coincidence. Several pathophysiological hypotheses have been proposed to explain this association, including a potential central role of T lymphocytes.
We describe the case of a 21 years-old female patient who initially presented with isolated nephrotic range proteinuria. She had no evidence of systemic involvement and Immunological tests were negative, including anti-neutrophil antibodies (ANA) and anti-double-stranded DNA antibodies (Anti-dsDNA). Renal biopsy showed normal glomeruli under light microscopy and no significant deposits were found in immunofluorescence studies. She was diagnosed to have MCNS and responded to a short course of steroids. She remained in remission for three years, after which she presented again with nephrotic-range proteinuria accompanied by clinical signs of systemic involvement. During her second presentation, she fulfilled the diagnostic criteria of systemic lupus erythematosus (SLE) and another kidney biopsy showed class-V lupus nephritis. She was treated with pulse steroids followed by oral prednisolone and mycophenolate mofetil, with good clinical response.
This case indicates that relapses of MCNS should be carefully investigated in the right setting to avoid missing a systemic disease such as SLE.
已有狼疮性肾炎(LN)与微小病变肾病综合征(MCNS)关联的罕见病例报道。一些作者认为,鉴于这两种疾病的低患病率,这种关联可能并非简单的巧合。已经提出了几种病理生理假说来解释这种关联,包括T淋巴细胞可能发挥的核心作用。
我们描述了一名21岁女性患者的病例,该患者最初表现为单纯的肾病范围蛋白尿。她没有全身受累的证据,免疫学检查均为阴性,包括抗中性粒细胞抗体(ANA)和抗双链DNA抗体(抗dsDNA)。肾活检光镜下肾小球正常,免疫荧光检查未发现明显沉积物。她被诊断为MCNS,并对短期使用类固醇治疗有反应。她缓解了三年,之后再次出现肾病范围蛋白尿并伴有全身受累的临床症状。在她第二次就诊时,她符合系统性红斑狼疮(SLE)的诊断标准,再次进行的肾活检显示为V级狼疮性肾炎。她接受了脉冲类固醇治疗,随后口服泼尼松龙和霉酚酸酯,临床反应良好。
该病例表明,在适当的情况下,应对MCNS的复发进行仔细调查,以避免漏诊诸如SLE等全身性疾病。