Sussman G L, Rivera V J, Kohler P F
Ann Intern Med. 1983 Jul;99(1):32-5. doi: 10.7326/0003-4819-99-1-32.
A 34-year-old man had polar extremes of B cell dysfunction: systemic lupus erythematosus evolving into common variable hypogammaglobulinemia. He presented in 1974 with seizures and six other criteria for systemic lupus erythematosus; his antinuclear antibody titer was 1:1024 and IgG level, 2870 mg/dL. After 5 months of immunosuppressive treatment, a 79% decrease in serum IgG and 95% decrease in IgA levels occurred and manifestations of systemic lupus erythematosus disappeared. Six years later, he developed panhypogammaglobulinemia, had recurrent sinopulmonary infections, and showed nodular lymphoid hyperplasia on rectal biopsy. For comparison, serum immunoglobulin concentrations were measured serially in 13 other patients with systemic lupus erythematosus. Three developed severe depressions of these levels, two with IgG levels less than 300 mg/dL and one with an IgA level of 8 mg/dL. These decreases were transient, related to treatment, and not associated with infections. Daily high-dose prednisone therapy (60 mg/d) rather than treatment with cytotoxic drugs correlated with decreased immunoglobulin concentrations.
一名34岁男性出现了B细胞功能障碍的两极情况：系统性红斑狼疮演变为常见变异型低丙种球蛋白血症。他于1974年因癫痫发作及其他六项系统性红斑狼疮标准症状就诊；其抗核抗体滴度为1:1024，IgG水平为2870mg/dL。经过5个月的免疫抑制治疗后，血清IgG水平下降了79%，IgA水平下降了95%，系统性红斑狼疮的症状消失。六年后，他出现了全丙种球蛋白低下血症，反复发生鼻窦肺部感染，直肠活检显示有结节性淋巴样增生。作为对照，对另外13名系统性红斑狼疮患者连续测量血清免疫球蛋白浓度。其中三人出现了这些水平的严重下降，两人的IgG水平低于300mg/dL，一人的IgA水平为8mg/dL。这些下降是短暂的，与治疗有关，且与感染无关。每日大剂量泼尼松治疗（60mg/d）而非细胞毒性药物治疗与免疫球蛋白浓度降低相关。
