Primary sjogren's syndrome with renal tubular acidosis and hypokalemic quadriparesis in middle aged female: A case report

Sjögren’s syndrome is an autoimmune lymphocytic infiltrative disease that leads to chronic inflammatory changes to exocrine glands and extra-glandular systemic organs. It rarely affects children and adolescents. In cases where adolescents are affected, symptoms include xerostomia, xerophthalmia often leads to a missed diagnosis. Consequently, the first presenting sign of Sjögren’s syndrome in adolescents may be heterogeneous, with varying clinical symptoms related to parotitis or systemic organ involvement. Renal involvement occurs in 18–67% of cases, with chronic tubulo-interstitial nephritis being the most frequent pathology which can lead to distal renal tubular acidosis characterized by normal anion gap acidosis with hypokalemia and alkaline urinary pH. Hypokalemic periodic paralysis can be primary or secondary to potassium deficiency which can arise from several causes In our case report, we discuss a 47-year-old female with distal renal tubular acidosis (RTA), who had experienced severe hypokalemic episodes since last 6 month; the patient was eventually diagnosed with Sjögren’s syndrome. She was managed and maintained on potassium and alkali repletion therapy.