Ascer K, Walker J A, Lief P D, Barland P, Bank N
Am J Med. 1983 Jan;74(1):83-9. doi: 10.1016/0002-9343(83)91123-3.
We are reporting findings in 13 patients who presented with glomerulonephritis without evidence of systemic disease, but who were found to have positive antinuclear antibody results and immunoglobulin and/or complement deposits at the dermal-epidermal junction of normal skin not exposed to light. There was no evidence of other organ involvement, and serologic tests for systemic lupus erythematosus (SLE) gave negative results. The renal disease is characterized by severe proteinuria, focal or diffuse proliferative glomerular lesions on biopsy, with variable patterns of immunoglobulin deposits. No clinical manifestations or serologic results typical of SLE have developed during prolonged observation. HLA phenotyping carried out in eight of the 13 patients revealed DR2 or DR3 alloantigens or both in seven of the eight patients, an incidence similar to that in patients with overt SLE. Because of the specificity of the skin biopsy immunofluorescence, the similarity of HLA-DR antigens, and a favorable response of the renal disease to therapy, we believe that these patients have a variant of SLE.
我们报告了13例患者的研究结果，这些患者表现为无全身性疾病证据的肾小球肾炎，但发现其抗核抗体结果呈阳性，且在未暴露于光的正常皮肤的真皮 - 表皮交界处有免疫球蛋白和/或补体沉积。没有其他器官受累的证据，系统性红斑狼疮（SLE）的血清学检查结果为阴性。肾脏疾病的特征是严重蛋白尿，活检显示局灶性或弥漫性增生性肾小球病变，免疫球蛋白沉积模式多样。在长期观察期间，未出现SLE典型的临床表现或血清学结果。13例患者中的8例进行的HLA表型分析显示，8例患者中有7例存在DR2或DR3同种抗原或两者皆有，这一发生率与显性SLE患者相似。由于皮肤活检免疫荧光的特异性、HLA - DR抗原的相似性以及肾脏疾病对治疗的良好反应，我们认为这些患者患有SLE的一种变体。
