Morel P, Peltier A P, Allegre P, Civatte J
Nouv Presse Med. 1978;7(27):2377-80.
This study involved 10 patients having, in direct immunofluorescence, with covered healthy skin, a speckled IgG (+/-IgM) staining of the epidermal nuclei. The chief clinical signs seen and their respective percentages were: Raynaud's syndrome (80%), arthralgia (80%), diffuse or localised alopoecia (60%), muscular disease (40%), swollen fingers (40%), sclerodactylie (20%), cutaneous sclerosis extending beyond the extremities (30%), cutaneous signs of lupus erythematosus (30%), renal involvement (10%). In nine cases out of ten there were circulating anti-ENA antibodies at high levels, divided into anti-RNP antibodies (7/10), anti-Sm antibodies (1/10) and anti-RNP and anti-Sm antibodies (1/10). Diagnoses were divided into: lupus erythematosus (3/10), systemic scleroderma (3/10), Sharp's mixed connective tissue disease (MCTD) (3/10) and non-classified connective tissue disease (1/10). The combination of speckled staining of epidermal nuclei and circulating anti-ENA antibodies cannot be considered to be specific of any particular type of connective tissue disease and the prognosis of the disease does not appear to differ from that of the usual prognosis of connective tissue disease with anti-ENA antibodies.
本研究纳入了10例患者,其直接免疫荧光显示,覆盖健康皮肤的表皮细胞核有斑点状IgG(±IgM)染色。观察到的主要临床体征及其各自的百分比为:雷诺综合征(80%)、关节痛(80%)、弥漫性或局限性脱发(60%)、肌肉疾病(40%)、手指肿胀(40%)、指硬皮病(20%)、累及肢体以外部位的皮肤硬化(30%)、红斑狼疮皮肤表现(30%)、肾脏受累(10%)。十例中有九例存在高水平的循环抗ENA抗体,分为抗RNP抗体(7/10)、抗Sm抗体(1/10)以及抗RNP和抗Sm抗体(1/10)。诊断分为:红斑狼疮(3/10)、系统性硬化症(3/10)、夏普混合性结缔组织病(MCTD)(3/10)和未分类的结缔组织病(1/10)。表皮细胞核斑点状染色与循环抗ENA抗体的组合不能被认为是任何特定类型结缔组织病的特异性表现,且该疾病的预后似乎与伴有抗ENA抗体的结缔组织病的通常预后并无差异。