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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。
Objective To investigate the clinical features and treatment of primary Sjogren’s syndrome, renal tubular acidosis complicated with osteomalacia. Methods The clinical data of 20 patients with primary Sjogren’s syndrome and tubuloacidosis complicated with osteomalacia treated in Department of Endocrinology, Tianjin Medical University General Hospital from 2003 to 2008 were retrospectively analyzed. Results Twenty patients had dental caries, fatigue and bone pain. Fifteen patients had dry mouth symptoms and ten patients had dry eye symptoms. According to the clinical manifestations, bone X-ray and bone density test results, 20 patients were divided into severe bone disease group (10 cases) and mild osteoarthritis group (10 cases). Compared with the two groups of rheumatoid immune indicators: serious skeletal disease group SSA positive rate, SSB positive rate and globulin levels were higher than mild skeletal disease group, but the difference was not statistically significant (P> 0.05); severe osteoporosis group ANA, rheumatoid Factor and γ-globulin levels were higher than those in mild osteopathy group, the difference was statistically significant (P <0.05). Kidney and muscle biopsy showed lymphocyte infiltration and immune complex deposition. After treatment weakness, significant relief of bone pain. Conclusions Osteomalacia secondary to primary Sjogren’s syndrome and renal tubular acidosis require active immunosuppressive therapy and sufficient vitamin D supplementation in addition to the traditional acid-replacement therapy.