【摘要】 目的探讨原发性干燥综合征、肾小管酸中毒并发骨软化症的临床特点和治疗效果。方法回顾性分析天津医科大学总医院内分泌科2003—2008年收治的20例原发性干燥综合征、肾小管酸中毒并发骨软化症患者的临床资料。结果 20例患者均有龋齿、乏力、骨痛,15例患者有口干症状,10例患者有眼干症状。依据临床表现、骨X线和骨密度检查结果,20例患者分为严重骨病组(10例)和轻度骨病组(10例)。对比两组风湿免疫指标:严重骨病组SSA阳性率、SSB阳性率及球蛋白水平高于轻度骨病组,但差异无统计学意义(P>0.05);严重骨病组ANA、类风湿因子和γ球蛋白水平高于轻度骨病组,差异有统计学意义(P<0.05)。肾脏和肌肉组织活检均可见淋巴细胞浸润和免疫复合物沉积。治疗后无力、骨痛显著缓解。结论继发于原发性干燥综合征、肾小管酸中毒的骨软化症,除了传统的纠酸补钙治疗外,仍需积极的免疫抑制治疗和补充足量的活性维生素D。更多还原

【Abstract】 Objective To summarize the clinical manifestations and treatment of osteomalacia in patients with primary Sjgren′s Syndrom and renal tubular acidosis.Methods The clinical data of 20 patients suffered from this disease and admitted in our hospital from 2003 to 2008 were analyzed retrospectively.Results All these 20 patients experienced decayed tooth,tiredness,and boneache.Among them 15 patients had had thirsty feelings and 10 suffered from scheroma.According to the clinical manifestations,bone X-ray findings,and bone mineral density,these 20 patients were divided into severe bone disease group(n=10) and mild bone disease group(n=10).The comparison of rheumatic immunological index between these two groups concluded that the positive rates of SSA and SSB and the concentration of globulin in severe group were higher than those in mild group,although the difference was not statistically significant(P>0.05).Antinuclear antibody,rheumatoid factor,andγglobulin in severe group were significantly higher than those in mild group(P<0.05).Infiltration of lympocytes and deposition of immunoglobulin complexes were found both in kidney and muscular biopsy tissues.Tiredness and boneache were resolved after treatment.Conclusion For osteomalacia caused by the combination of primary Sjgren′s syndrome and renal tubular acidosis,active immunosuppressive treatment and supplementation of large-dose vitamin D in active form may be feasible in addition to conventional acidity correction and calcium supplementation,.更多还原