43-year-old Caucasian female presented with progressive weakness and dyspnea. She was diagnosed with hypokalemic paralysis from a severe distal renal tubular acidosis (RTA). Immunologic work-up showed a strongly positive ANA of 1:640 and positive antibodies to SSA and SSB. Schirmer's test was normal. Renal involvement in Sjogren's syndrome (SS) is not uncommon and may precede sicca complaints. The pathology in most cases is a tubulointerstitial nephritis causing among other things, distal RTA, and, rarely, hypokalemic paralysis. Treatment consists of potassium repletion, alkali therapy and corticosteroids. Primary SS should be a differential in premenopausal women with acute weakness and hypokalemia.

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