Merola Joseph F, Chang Caroline A, Sanchez Miguel R, Prystowsky Stephen D
Harvard Medical School, Brigham and Women's Hospital, Boston, MA, USA.
J Drugs Dermatol. 2011 Dec;10(12):1413-20.
The aim was to assess the level of systemic involvement and character of renal disease in patients with chronic cutaneous lupus erythematosus of the discoid lupus variety (hereafter referred to as 'discoid lupus') and features of systemic lupus erythematosus (SLE). Clinical confusion with other types of cutaneous lupus erythematosus complicates interpretation of some previously reported studies.
Over three years, sixteen patients met the diagnostic criteria of discoid lupus, positive anti-nuclear-antibody, and at least one extracutaneous manifestation.
Most patients (14/16) were female, between 26 to 66 years old. Arthritis was the most common extracutaneous manifestation followed by Raynaud's phenomenon. The anti-nuclear-antibody was speckled in ten patients with titers ranging from 1:40 to 1:1280 IU/mL. Elevated levels of double-stranded-DNA in low titers were found in four patients, anti-Smith-antibody in four; anti-Sjogren-syndrome-A-antibody in seven, and anti-ribonucleoprotein-antibody in seven. Renal function markers were transiently high in some patients but normalized over time. Hematuria and/or proteinuria were present at some time in seven patients. The highest BUN and creatinine levels were 42 mg/dL and 1.5 mg/dL, respectively. One patient had membranous glomerulonephropathy class 5; however, discoid lupus developed well after the onset of renal disease during a time when renal function had returned to normal.
Our observational data supports previous reports suggesting that patients with active discoid lupus rarely have progressive renal insufficiency. The mechanism for the development of discoid lupus may involve an immunologic mechanism that differs from that which produces severe organ involvement, especially advanced immune-complex-mediated renal disease. Patients with discoid lupus rarely have sustained high levels of antibodies to double-stranded-DNA. Discoid lupus appears to be a marker for a more benign lupus course. This clinical observation lays the groundwork for a larger prospective, longitudinal cohort study for further validation.
旨在评估盘状红斑狼疮型慢性皮肤红斑狼疮（以下简称“盘状狼疮”）患者的全身受累程度、肾脏疾病特征以及系统性红斑狼疮（SLE）的特征。与其他类型皮肤红斑狼疮的临床混淆使一些先前报道的研究的解读变得复杂。
在三年多的时间里，16名患者符合盘状狼疮、抗核抗体阳性以及至少一种皮肤外表现的诊断标准。
大多数患者（14/16）为女性，年龄在26至66岁之间。关节炎是最常见的皮肤外表现，其次是雷诺现象。10名患者的抗核抗体呈斑点状，滴度范围为1:40至1:1280 IU/mL。4名患者低滴度的双链DNA水平升高，4名患者抗史密斯抗体阳性；7名患者抗干燥综合征A抗体阳性，7名患者抗核糖核蛋白抗体阳性。一些患者的肾功能指标曾短暂升高，但随时间推移恢复正常。7名患者在某些时候出现血尿和/或蛋白尿。最高血尿素氮和肌酐水平分别为42 mg/dL和1.5 mg/dL。1名患者患有5级膜性肾小球肾炎；然而，盘状狼疮在肾病发作且肾功能恢复正常后的一段时间才充分发展。
我们的观察数据支持先前的报道，即活动性盘状狼疮患者很少有进行性肾功能不全。盘状狼疮的发病机制可能涉及一种与导致严重器官受累，尤其是晚期免疫复合物介导的肾脏疾病的机制不同的免疫机制。盘状狼疮患者很少有持续高水平的双链DNA抗体。盘状狼疮似乎是狼疮病程更良性的一个标志。这一临床观察为更大规模的前瞻性纵向队列研究奠定了基础，以便进一步验证。
