Abstract :
Sjogren’s syndrome (SS) is common autoimmune diseases manifesting with various glandular and extra glandular features which are difficult to evaluate and manage. Hypokalemic flaccid paralysis may be the rare extra glandular clinical manifestation of SS. We present a case of acute flaccid paralysis which on evaluation turned out to be primary sjogren’s syndrome (pSS). We present a case of 30 year old young woman with no known comorbidities presented with acute flaccid paralysis. Her evaluation reveals severe hypokalemia with K -1.6 meq/l, metabolic acidosis with pH-7.28 and urinary pH ?6.5. An initial diagnosis of hypokalemic periodic paralysis (HPP) secondary to distal renal tubular acidosis (dRTA) was made. She was later found to have sjogren’s syndrome on the basis of positive ANA & Anti-Ro (SS-A) Antibody and salivary gland biopsy. She was managed with potassium supplements, sodium bicarbonate and immunomodulators. Primary Sjogren’s syndrome usually associated with distal renal tubular acidosis which may cause hypokalemia leading to HPP, rarely which may be the initial presenting symptom in young women as in our case. Early diagnosis and treatment of these patients would lead to less complication and better quality of life.
Keyword :
Hypokalemic periodic paralysis, Sjogrens syndrome, Distal renal tubular acidosis.