Hypokalemic periodic paralysis in Sjogren’s syndrome secondary to distal renal tubular acidosis

Abstract

We report a 53-year-old Turkish female presented with progressive weakness and mild dyspnea. Laboratory results demonstrated severe hypokalemia with hyperchloremic metabolic acidosis. The urinary anion gap was positive in the presence of acidemia, thus she was diagnosed with hypokalemic paralysis from a severe distal renal tubular acidosis (RTA). Immunologic work-up showed a strongly positive ANA of 1:3,200 and positive antibodies to SSA and SSB. Schirmer's test was abnormal. Autoimmune and other tests revealed Sjögren syndrome as the underlying cause of the distal renal tubular acidosis. 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 could be a differential in women with acute weakness and hypokalemia.

American Society of Nephrology Quiz and Questionnaire 2014

“…In any event, the potassium wasting can be severe and lead to hypokalemic paralysis (36,37). In this patient, the addition of topiramate effectively led to the development of proximal RTA as well as possibly worsening of the distal RTA (due to complementary defects in distal acidification from the drug and from Sjögren syndrome).…”

“…Most of these patients had ocular and dental symptoms consistent with the diagnosis of Sjögren syndrome. Of note, hypokalemia in these patients can be severe and may lead to hypokalemic paralysis (34,36,37).…”

An Unusual Initial Presentation of Sjögren’s Syndrome: Severe Hypokalemic Paralysis Secondary to Distal Renal Tubular Acidosis

“…Severe hypokalemia and paralysis due to dRTA is an unusual initial presentation of Sjögren's syndrome [6,7]. Palkar et al [8] also reported a case of Sjögren's syndrome that presented with acute quadriparesis, bulbar weakness, atrial fibrillation, and ventricular ectopics due to hypokalemic dRTA.…”

Primary Sjögren's Syndrome First Presenting as Hypokalemic Quadriparesis

“…6 Occasionally, this hypokalemia is sufficiently severe to produce paralysis or respiratory arrest. 9 In adults, autoimmune diseases are major causes of dRTA, 3 and this may be its presenting manifestation. Thus, adults with apparently idiopathic dRTA should be evaluated for these conditions.…”