Roy Alok Kumar, Pal Dilip, Das Sudip, Kar Chinmoy, Maiti Arunasis
Department of Skin, STD and Leprosy, NRS Medical College and Hospital, Kolkata 700014.
J Indian Med Assoc. 2007 Mar;105(3):141, 148.
Systemic lupus erythematosus is a multisystem organ inflammation due to damage of cells and tissues by pathogenic auto-antibodies and immune complexes. The most important factor for the causation of familial systemic lupus erythematosus is genetic on which environmental factor usually coexists. Two brothers of 7 and 3 years of age having childhood systemic lupus erythematosus are reported here because of low incidence and familial occurrence. Both the children fulfilled the American Rheumatology Association (ARA) criterion of systemic lupus erythematosus though anti-dsDNA was negative in both and antinuclear factor was positive only in the younger child. Systemic lupus erythematosus begins in childhood in 20% of adult patients and usually after the age of 8 years but here onset was even at an earlier age. The treatment of the both the siblings included administration of steroids and cyclophosphamide.
系统性红斑狼疮是一种多系统器官炎症，由致病性自身抗体和免疫复合物对细胞和组织造成损害所致。家族性系统性红斑狼疮发病的最重要因素是遗传，通常还并存环境因素。本文报告了两名分别为7岁和3岁的患儿童期系统性红斑狼疮的兄弟，因其发病率低且为家族性发病。两名患儿均符合美国风湿病学会（ARA）系统性红斑狼疮的标准，尽管两人抗双链DNA均为阴性，仅年幼儿童抗核因子为阳性。20%的成年系统性红斑狼疮患者在儿童期起病，通常在8岁以后，但此处发病年龄更早。两名患儿的治疗均包括使用类固醇和环磷酰胺。
