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Renal tubular acidosis as the initial presentation of Sjögren’s syndrome
  1. Karen Ho1,
  2. Pouneh Dokouhaki1,
  3. Mark McIsaac1 and
  4. Bhanu Prasad2
  1. 1 University of Saskatchewan College of Medicine, Saskatoon, Canada
  2. 2 Regina General Hospital, Regina, Canada
  1. Correspondence to Dr Bhanu Prasad, bprasad{at}


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.

  • renal system
  • acute renal failure
  • sjogren’s syndrome

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  • Contributors KH wrote the initial draft. PD contributed to the pathology images, MM assisted with the drafts and BP wrote the final version. All the authors have read the final version.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Patient consent for publication Obtained.

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