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Distal RTA (dRTA) leading to recurrent hypokalemia and paresis as an initial manifestation of primary Sjogren's syndrome (pSS) is well-known but not commonly reported (1), as pSS is one of the secondary causes of dRTA.
Based on the above clinical presentation and the attendant laboratory work, a diagnosis of distal RTA (dRTA) due to primary Sjogren's syndrome was made.
2) RTA is suspected in the presence of hyperchloremic metabolic acidosis (HMA) and it is divided into three categories: proximal RTA, distal RTA (dRTA), and hyperkalemic RTA.
Distal RTA occurs in up to 5% of patients with pSS; however, the prevalence of symptomatic dRTA was not assessed in this study.
Typical findings of type 1 distal RTA (dRTA) include low serum bicarbonate, normal serum anion gap, hyperchloremia and hypokalemia.
We were unable to determine the etiology of RTA in our case, however, owing to the association of distal RTA with autoimmune diseases, a possible autoimmune etiology must always be sought.
1) A frank distal RTA was found in 5% of patients with Sjogren's syndrome.
3) Distal RTA is a clinical syndrome consisting of hypokalemia, hyperchloremic metabolic acidosis, inability to lower urine pH below 5 during acidemia, nephrocalcinosis and nephrolithiasis.
Of the different types of RTA associated with hypokalemia, distal RTA may be due to a defect in proximal tubular reabsorption found in the bicarbonate ion (HC[O.
DISCUSSION: In view of history and investigations, possibility of renal tubular dysfunction secondary to distal RTA was considered.

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