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三例系统性红斑狼疮合并微小病变型肾病综合征病例报告

[Three cases presenting with systemic lupus erythematosus and minimal change nephrotic syndrome].

作者信息

Matsumura N, Dohi K, Shiiki H, Morita H, Yamada H, Fujimoto J, Kanauchi M, Hanatani M, Ishikawa H

出版信息

Nihon Jinzo Gakkai Shi. 1989 Sep;31(9):991-9.

PMID:2585838
Abstract

Three cases presenting with systemic lupus erythematosus (SLE) and minimal change nephrotic syndrome (MCNS) are reported in this paper. All cases were female; they abruptly developed nephrotic syndrome at the age of 30, 11 and 23 years, respectively. In Case 1, the diagnosis of SLE was based on fever, butterfly rash, Raynaud's phenomenon, leukopenia, lymphopenia, hypocomplementemia, a high titer of anti-DNA antibodies, positive DNA and LE test, and the presence of anti-nuclear antibodies (speckled pattern). In Case 2, the diagnosis was based on butterfly rash, central nervous system involvement, lymphopenia, hypocomplementemia, a positive LE cell phenomenon, a high titer of anti-DNA antibodies and a positive DNA test. In Case 3, the diagnosis was based on photosensitivity, alopecia, lymphopenia, hypocomplementemia, a high titer of anti-DNA antibodies, a positive DNA test and a positive LE cell phenomenon. In these three cases, initial symptoms were puffy face and pretibial edema which occurred suddenly. These symptoms disappeared completely after either corticosteroid therapy or a combination therapy using corticosteroids and immunosuppressive drugs. These patients took a favorable course and no aggravation was noted in the findings of urinalysis and renal functions. In two of these cases, the diagnostic criteria for SLE were satisfied, but the remaining patient fulfilled only three criteria except for renal disorder. In each of these cases, minor glomerular abnormalities were disclosed by renal histology. It seems likely that SLE was complicated by MCNS in these cases. From these cases, it is suggested that there is a possibility of immunological abnormalities associated with SLE and MCNS.

摘要

本文报告了3例系统性红斑狼疮(SLE)合并微小病变肾病综合征(MCNS)的病例。所有病例均为女性,分别于30岁、11岁和23岁突然出现肾病综合征。病例1中,SLE的诊断依据为发热、蝶形红斑、雷诺现象、白细胞减少、淋巴细胞减少、补体降低、抗DNA抗体高滴度、DNA和狼疮细胞试验阳性以及抗核抗体(斑点型)阳性。病例2中,诊断依据为蝶形红斑、中枢神经系统受累、淋巴细胞减少、补体降低、狼疮细胞现象阳性、抗DNA抗体高滴度和DNA试验阳性。病例3中,诊断依据为光敏性、脱发、淋巴细胞减少、补体降低、抗DNA抗体高滴度、DNA试验阳性和狼疮细胞现象阳性。在这3例中,最初的症状为面部浮肿和胫前水肿,均突然出现。这些症状在使用糖皮质激素治疗或糖皮质激素与免疫抑制药物联合治疗后完全消失。这些患者病情进展良好,尿常规和肾功能检查结果未出现恶化。其中2例符合SLE的诊断标准,但其余1例除肾脏疾病外仅满足3条标准。在每例病例中,肾脏组织学检查均显示轻微的肾小球异常。这些病例似乎是SLE合并MCNS。从这些病例来看,提示存在与SLE和MCNS相关的免疫异常可能性。

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