PT  - JOURNAL ARTICLE
AU  - Karen Ho
AU  - Pouneh Dokouhaki
AU  - Mark McIsaac
AU  - Bhanu Prasad
TI  - Renal tubular acidosis as the initial presentation of Sjögren’s syndrome
AID  - 10.1136/bcr-2019-230402
DP  - 2019 Aug 01
TA  - BMJ Case Reports
PG  - e230402
VI  - 12
IP  - 8
4099  - http://casereports.bmj.com/content/12/8/e230402.short
4100  - http://casereports.bmj.com/content/12/8/e230402.full
SO  - BMJ Case Reports2019 Aug 01; 12
AB  - We present a 44-year-old female with an initial presentation with distal renal tubular acidosis (RTA) after she presented with hypokalaemia and normal anion gap acidosis. Three years following the diagnosis, she presented with progressive renal impairment. In the absence of any clinical, biochemical and radiological clues, she underwent a renal biopsy which showed severe tubulitis secondary to lymphocytic infiltration. Serological investigations subsequently revealed positive anti-nuclear, anti-Sjögren’s syndrome related antigen A (SS-A), and anti-Sjögren’s syndrome related antigen B (SS-B) antibodies, supporting the diagnosis of Sjögren’s syndrome. This case is unique in that distal RTA was the presenting clinical manifestation of Sjögren’s syndrome. We hope that a consideration for Sjögren’s syndrome is made in patients with seemingly idiopathic RTA.