Sandhya Pulukool, Danda Debashish, Rajaratnam Simon, Thomas Nihal
Department of Clinical Immunology and Rheumatology, Christian Medical College and Hospital, Vellore-632004, India.
Department of Endocrinology, Diabetes and Metabolism, Christian Medical College and Hospital, Vellore-632004, India.
Open Rheumatol J. 2014 Dec 19;8:103-9. doi: 10.2174/1874312901408010103. eCollection 2014.
To study the profile of Renal Tubular Acidosis (RTA) in Asian Indian patients with Primary Sjögren's Syndrome (pSS).
The Electronic medical records of patients with a diagnosis of pSS seen between 2003 and 2010 at our tertiary care teaching hospital were screened for RTA. Clinical features, immunological profile, acid-base balance and electrolyte status, 25-hydroxyvitamin D (25(OH) D3) levels, histopathological changes in minor salivary gland biopsy samples and radiological findings were retrieved. RTA was diagnosed in cases of hyperchloremic metabolic acidosis with urinary pH values higher than 5.5. Those with known features suggestive of RTA including hypokalemic paralysis, hyperchloremia and nephrocalcinosis without acidosis were defined as incomplete RTA.
Of the 380 patients with clinically suspected pSS, 25 had RTA. The median age was 32 (18-60) years. Nineteen patients had complete RTA. Six had incomplete RTA. Only 10 patients (40%) had symptoms related to RTA at presentation. Sixteen patients (64%) had present or past history of hypokalemic paralysis. Pseudofractures were seen in 7 patients and an additional 2 had subclinical radiological osteomalacia. Majority of the patients (61.2%) had a normal 25(OH) D3 level. Those with osteomalacia had significantly lower serum phosphate, blood ph and higher alkaline phosphatase. Serum calcium and 25(OH) D3 levels were not significantly different between patients with osteomalacia and those without.
Most patients were asymptomatic for RTA inspite of clinically overt and elicitable features. Skeletal manifestation was a common finding in patients with Sjögren and RTA, despite normal levels of 25 (OH) D3 in a majority.
研究亚洲印度裔原发性干燥综合征（pSS）患者的肾小管酸中毒（RTA）情况。
筛查2003年至2010年期间在我们的三级护理教学医院就诊且诊断为pSS的患者的电子病历，以确定是否存在RTA。收集临床特征、免疫指标、酸碱平衡和电解质状态、25-羟维生素D（25(OH) D3）水平、小唾液腺活检样本的组织病理学变化以及影像学检查结果。当患者出现高氯性代谢性酸中毒且尿pH值高于5.5时诊断为RTA。那些具有提示RTA的已知特征（包括低钾性麻痹、高氯血症和无酸中毒的肾钙质沉着症）的患者被定义为不完全RTA。
在380例临床疑似pSS的患者中，25例患有RTA。中位年龄为32（18 - 60）岁。19例患者患有完全性RTA。6例患有不完全性RTA。仅10例患者（40%）在就诊时出现与RTA相关的症状。16例患者（64%）有低钾性麻痹的现病史或既往史。7例患者出现假性骨折，另外2例有亚临床影像学表现的骨软化症。大多数患者（61.2%）的25(OH) D3水平正常。患有骨软化症的患者血清磷酸盐、血pH值显著降低，碱性磷酸酶升高。骨软化症患者与无骨软化症患者的血清钙和25(OH) D3水平无显著差异。
尽管有明显的临床特征且可引出相关表现，但大多数RTA患者无症状。骨骼表现是干燥综合征合并RTA患者的常见发现，尽管大多数患者的25(OH) D3水平正常。
