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补体成分,特别是遗传性C_2和C_4缺乏的人易患SLE样综合征.既往曾报道C_9缺乏者一般是健康的,本文作者报道1例完全性C_9缺乏(C_9D)者可能系患原发性干燥综合征(SS)的病例.患者日本女性,48岁,眼干、口干、发热、关节痛、体重下降2个月入院.体检发现低热(37.6℃),双侧颈部和腹股沟淋巴结肿大,肝稍大,脾未扪及.既往有关节痛史.实验室检查:血红蛋白10.5g,白细胞2800,血小板29.1万.肝肾功能、尿常规、血清电解质均正常.LE细胞、抗核抗体、类风湿因子、抗天然DNA、Sm、RNP、Ro/SS-A、La/SS-B抗体均阴性.胸部X线摄片、心电图均正常.橡皮糖试验(当唾液流率应用口香糖作为刺激物刺激时),涎腺造影及唇腺活检均正常.颈、
Complement components, especially those with hereditary C_2 and C_4 deficiency, are predisposed to SLE-like syndromes. Previously reported C_9 deficiency was generally healthy, and the authors report that one patient with complete C_9 deficiency (C_9D) may have primary Sickness Syndrome (SS) Cases Japanese women, aged 48, with dry eyes, dry mouth, fever, joint pain, and weight loss, were admitted to the hospital for 2 months and were found to have low fever (37.6 ° C), bilateral neck and inguinal lymph nodes Large, large liver, spleen not palpable. Previous history of joint pain. Laboratory tests: hemoglobin 10.5g, white blood cells 2800, 291,000 platelets. Liver and kidney function, urine routine, serum electrolytes were normal. ELE cells, anti-nuclear antibodies , Rheumatoid factor, anti-natural DNA, Sm, RNP, Ro / SS-A and La / SS-B antibodies were all negative. Chest X-ray and electrocardiogram were normal. Gum test (when chewing gum was used as stimulus When stimulated), salivary gland angiography and labial gland biopsy were normal. Neck,