Ram Rapur, Swarnalatha Gudithi, Dakshinamurty Kaligotla Venkata
Department of Nephrology, Sri Venkateswara Institute of Medical Sciences, Tirupati, India.
Am J Nephrol. 2014;40(2):123-30. doi: 10.1159/000365199. Epub 2014 Aug 20.
The exact frequency of distal and proximal renal tubular acidosis (RTA) in Sjögren's syndrome is unknown. Other features of Sjögren's syndrome like polyuria, glomerular manifestations, familial occurrence and pregnancy are not widely reported. The aim was to prospectively study the clinical features and outcome of distal and proximal RTA in Sjögren's syndrome and also report on other renal manifestations of Sjögren's syndrome.
The present study is a prospective consecutive case series of patients who presented with a history suggestive of RTA and Sjögren's syndrome. All patients were followed for 1 year. The diagnosis of RTA was by fractional excretion of bicarbonate. The diagnosis of Sjögren's syndrome was according to the American-European classification system [modified by Tzioufas and Voulgarelis: Best Pract Res Clin Rheumatol 2007;21:989-1010].
The total number of RTA patients diagnosed during this period was 149. Sjögren's syndrome accounted for 34.8% (52 of 149) of RTA patients. The important symptoms and laboratory parameters were oral and ocular symptoms in 23 (44.2%), dental caries in 12 (23%), body pains in 47 (90.3%), mean serum pH 7.202 ± 0.03, mean serum bicarbonate, 14.03 ± 1.66 mmol/l, and mean urine pH, 7.125 ± 0.54. There were 30 (57.6%) patients with distal RTA and 22 (42.3%) patients with proximal RTA.
The clinical implication of the present study is that RTA is a common feature of Sjögren's syndrome. It may be missed if the presentation is not due to oral and ocular symptoms. The present study is also the only one with a 1-year follow-up.
干燥综合征中远端和近端肾小管酸中毒(RTA)的确切发生率尚不清楚。干燥综合征的其他特征,如多尿、肾小球表现、家族性发病和妊娠等,尚未得到广泛报道。本研究旨在前瞻性地研究干燥综合征中远端和近端RTA的临床特征及预后,并报告干燥综合征的其他肾脏表现。
本研究是一项前瞻性连续病例系列研究,研究对象为有RTA病史且疑似干燥综合征的患者。所有患者均随访1年。RTA的诊断依据是碳酸氢盐排泄分数。干燥综合征的诊断依据美国-欧洲分类系统[由齐奥法斯和武尔加雷利斯修改:《最佳实践与临床风湿病学》2007年;21:989-1010]。
在此期间诊断出的RTA患者总数为149例。干燥综合征占RTA患者的34.8%(149例中的52例)。重要症状和实验室参数包括:23例(44.2%)有口腔和眼部症状,12例(23%)有龋齿,47例(90.3%)有全身疼痛,平均血清pH值为7.202±0.03,平均血清碳酸氢盐为14.03±1.66 mmol/L,平均尿pH值为7.125±0.54。有30例(57.6%)患者为远端RTA,22例(42.3%)患者为近端RTA。
本研究的临床意义在于,RTA是干燥综合征的常见特征。如果临床表现不是由口腔和眼部症状引起,可能会漏诊。本研究也是唯一一项进行了1年随访的研究。