Minimal Change Nephrotic Syndrome in Two Patients with Suspected Systemic Lupus Erythematosus: Association or Co-Incidence

紅斑性狼瘡(SLE)是一種眾所皆知的自體免疫性疾，它可製造各種自體免疫抗體與免疫複合物沉積於身體各個器官而著稱；微細病變(MCD)是以光學顯微鏡下的無明顯變化和沒有免疫複合物沉積為其特色，而這兩個本質上不同之疾病，在其致病機轉上擁有一些相同特色，如：T-淋巴球的調控失常以及其T4/T8淋巴球之比例在疾病活性期較低。在此我們報告以腎病症候群(NS)為表現的兩個病例，其病程中牽引出紅斑性狼瘡和微細腎病變彼此間的關係。第一位表現出符合美國風溼醫學會全身紅斑性狼瘡的診斷標準，不過腎臟切片上呈現微細病變，也發現有管網狀包涵物(tubuloreticular inclusion)之存在；且在追蹤過程中，發現抗核抗體(ANA)的滴定度升高，提高全身紅斑性狼瘡診斷之可能性。這兩個病例接受類固醇治療後，其蛋白尿與下肢水腫也獲得大幅改善。文獻中的狼瘡性腎炎病例，大多數腎病症候群患者病理切片的分類多數為RPS/ISKDC/WHO的第三至五類，但以微細病變為表現者屈指可數。雖然依目前的證據並不太支持微細病變症候群(MCNS)是狼瘡腎炎的另一種變異型，兩者具相關連性，其治療策略（類固醇）跟一般原發性微細病變相同；因此腎病症候群或全身紅斑性狼瘡族群中，仔細探討潛在之原因與以腎切片術作詳細辨別是必要的。

關鍵字

微細病變腎病症候群；全身紅斑性狼瘡；類固醇

並列摘要

Systemic lupus erythematosus (SLE) is characterized by the production of pathogenic autoantibodies and the deposition of immune complexes in various organs, whereas minimal change disease (MCD) is characterized by normal or only mild mesangial cell proliferation without deposition of immune complexes. Although these two diseases are so different, they all share the same characteristics of dysregulation of T-lymphocyte and the appearance of low T4/T8 ratio. In this report, we present two cases who had (suspected) SLE developed minimal change NS (MCNS). Case 1 was diagnosed to have SLE, which fulfilled 5 revised SLE criteria of American College of Rheumatology (ACR), but MCD was shown in renal biopsy. Transient renal insufficiency was also noted. Case 2 had NS as well. Renal pathology revealed MCD and tubuloreticular inclusion. High titer of ANA developed later, and the diagnosis of SLE was highly suspected. Both cases got remission from NS after receiving steroid therapy. Since evidence supports that MCNS might be associated or related with SLE, the therapeutic strategy (corticosteroid) is suggested to treat as primary MCNS. Proper recognition of this entity by renal biopsy should be taken into consideration in SLE patient with NS.

並列關鍵字

Minimal change nephrotic syndrome MCNS ； systemic lupus erythematosus SLE ； corticosteroid

國際替代計量

Minimal Change Nephrotic Syndrome in Two Patients with Suspected Systemic Lupus Erythematosus: Association or Co-Incidence

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