Sjogren's syndrome (SS) is an autoimmune disease affecting multiple exocrine glands such as salivary and lacrimal glands, with focal lymphocytic infiltration. The disease predominantly affects women. The classic presentation is xerostomia and keratoconjunctivitis. In adult populations, tubulointerstitial nephritis, which can manifest as renal tubular acidosis (RTA), is the most prevalent renal manifestation of SS. However, in pediatric populations, RTA only occurs in 7.1-19.2% of cases with renal potassium wasting or hypokalemic paralysis. Here, we describe a case of SS manifesting with hypokalemic paralysis with underlying distal renal tubular acidosis (dRTA).
Keywords: autoimmune disease; distal renal tubular acidosis; recurrent hypokalemia; renal tubular defect; severe hypokalemia-induced paralysis; sjogrens syndrome.
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