Smit Patel

ABSTRACT We present a case of 26 year old female with a presentation of hypokalemia and distal renal tubular acidosis and normal anion gap acidosis. All basic blood investigations were normal and Anti Nuclear Antibody screening was done which was grade IV positive. Subsequently ANA profile was done which was positive was Ssa/Ro 60k Ab titer, Ssa/Ro 52kd Ab titer and Ssb Ab titer. Thyroid profile showed TSH 0.33 and T3 and T4 were 3.1 and 4.9 respectively. Urine anion gap was positive and urine pH was 6.8. The patient was diagnosed as Sjogren syndrome with hyperthyroidism and type 1 Renal Tubular Acidosis. This case is unique as the initial presentation of this patient was RTA and the patient had hyperthyroidism. Most patients of Sjogren have hypothyroidism and not hyperthyroidism. Keywords: Sjogren syndrome, hypothyroidism, renal tubular acidosis, ANA screen