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

Abstract

Background: 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. Methods: 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]. Results: 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. Conclusions: 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.

A Complicated Pregnancy in an Adult with HNF4A p.R63W-Associated Fanconi Syndrome

“…Interestingly, potassium levels stabilised by 31 weeks, despite infusing increasing amounts of bicarbonate. e phenomenon of rising bicarbonate requirements through gestation has not been previously described in patients with RFS, although it has been noted in other causes of renal tubular acidosis [17,18].…”

Primary Sjogren's syndrome presenting as hypokalemic paralysis

“…Although the involvement of minor salivary gland was present in all patients at the time of presentation, 62% of the patients lacked clinical signs and symptoms of salivary gland involvement. A prospective study[7] showed that RTA is a common feature of SS. In the same study, it was implicated that pSS as a cause of RTA may be missed if the oral and ocular symptoms are absent at presentation.…”

A Case of Kidney Involvement in Primary Sjögren’s Syndrome

“…For example, only 5 out of 48 pSS patients with renal involvement were diagnosed with DI [9], and this number was 7 out of 149 patients in another prospective cohort [10]. Nephrogenic DI is usually reported to be mild [6,11].…”