Renal Tubular Acidosis in Sjögren's Syndrome: A Case Series

Background: The exact frequency of distal and proximal renal tubular acidosis (RTA) in Sjogren's syndrome is unknown. Other features of Sjogren'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 Sjogren's syndrome and also report on other renal manifestations of Sjogren's syndrome. Methods: The present study is a prospective consecutive case series of patients who presented with a history suggestive of RTA and Sjogren's syndrome. All patients were followed for 1 year. The diagnosis of RTA was by fractional excretion of bicarbonate. The diagnosis of Sjogren's syndrome was according to the American-European classification system [modified by Tzioufas and Voulgarelis: Best Pract Res Clin Rheumatol 2007;21:989-1010]. Results: The total number of RTA patients diagnosed during this period was 149. Sjogren'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. Conclusions: The clinical implication of the present study is that RTA is a common feature of Sjogren'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.