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

Abstract

Sjögren's syndrome is mainly affects the exocrine glands. Patients usually complain of persistent dryness of the mouth and eyes. However, nonexocrine organs such as the kidneys are often affected in these patients. Distal renal tubular acidosis (dRTA) and interstitiel nephritis are common in Sjögren's syndrome. Nonetheless, severe hypokalemia and paralysis secondary to dRTA are unusual initial manifestation of Sjögren's syndrome. Here, we describe a case of a 48 year old women admitted to the emergency setting with severe hypokalemic paralysis and diagnosed Sjögren's syndrome.

Primary Sjögren's Syndrome First Presenting as Hypokalemic Quadriparesis

“…Thus, adults with apparently idiopathic dRTA should be evaluated for these conditions. 8 Distal RTA occurs in up to 5% of patients with pSS; however, the prevalence of symptomatic dRTA was not assessed in this study. The first case secondary to pSS was reported by Shern and Tu 10 in 1965.…”

“…7 Although diminished H+-ATPase activity is probably the most common defect in dRTA, impaired hydrogen ion secretion may be caused by several defects. 8 dRTA is commonly associated with hypokalemia due to renal wasting. 6 Occasionally, this hypokalemia is sufficiently severe to produce paralysis or respiratory arrest.…”

“…It is unknown whether treatment with steroids has a positive effect on autoimmune dRTA. 8 The clinical presentation of hypokalemic quadriparesis with respiratory failure as the first manifestation of pSS is very atypical. In this article, we tried to emphasize that hypokalemia can precipitate severe muscular weakness, and also that further evaluations are usually needed in order to elucidate the etiology of HMA.…”

Renal Tubular Acidosis (RTA) due to Sjögren’s Syndrome Presenting as Hypokalemic Quadriparesis - A Case Report

“…Nonetheless patients with Sjögren's syndrome rarely present with severe hypokalemia or paralysis secondary to DRTA. 1 Although renal tubular acidosis with hypokalemia associated with Sjögren's syndrome has been reported earlier but hypokalemic quadriparesis as the initial manifestation of the disease are rare. 2 Primary form involves the exocrine glands, with or without systemic involvement; there is also a secondary form, which is associated with other autoimmune diseases (rheumatoid arthritis, lupus erythematosus, scleroderma, inflammatory vascular and connective tissue diseases, etc.).…”

Complicated pregnancies in inherited distal renal tubular acidosis: importance of acid-base balance

“…Other major complications include severe hypokalemia resulting in paralysis, rhabdomyolysis or life-threatening arrhythmia 2,3 . Therapy includes lifetime treatment with potassium citrate and/or sodium bicarbonate to prevent growth retardation in children, kidney stone formation, and progression of nephrocalcinosis.…”