Sjögren renal tubular acidosis — SCE Rheumatology MCQ

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HardConnective Tissue DiseasesSjögren renal tubular acidosisSCE Rheumatology

A 46-year-old woman with primary Sjögren disease presents with muscle weakness and recurrent renal stones. Blood tests show potassium 2.8 mmol/L, bicarbonate 16 mmol/L and chloride 112 mmol/L. Urine pH is 6.8 despite systemic acidosis. Creatinine is normal. What is the most likely diagnosis?

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Correct answer: E — Distal renal tubular acidosis

Explanation lettering: B = shown as A · C = shown as B · D = shown as C · A = shown as D

Distal renal tubular acidosis is best because Sjögren disease can cause distal renal tubular acidosis with hypokalaemic normal-anion-gap acidosis and inappropriately alkaline urine. A is less suitable because primary hyperaldosteronism causes hypertension and metabolic alkalosis; B is less suitable because diabetic ketoacidosis causes high-anion-gap acidosis and ketones; C is less suitable because proximal RTA usually has bicarbonaturia with other proximal tubular defects; D is less suitable because Gitelman syndrome causes metabolic alkalosis with hypomagnesaemia. Clinical pearl: renal tubular disease is a classic extraglandular Sjögren complication.

Reference: BSR Sjögren disease guideline 2023