Hypokalemic Periodic Paralysis as Initial Manifestation of Primary Sjogrens Syndrome: A Case Report

Sjogrens syndrome (SS) is common autoimmune diseases manifesting with various glandular and extra glandular features which are difficult to evaluate and manage. Hypokalemic flaccid paralysis is the rare extra glandular clinical manifestation of SS. We present a case of acute flaccid paralysis which on evaluation turned out to be primary sjogrens syndrome. We present a case of 34 year old young female with no comorbidities presented with acute flaccid paralysis. Her evaluation reveals severe hypokalemia with K+ -1.7, metabolic acidosis with pH -7.23, urinary pH -> 6.5. An initial diagnosis of hypokalemic periodic paralysis secondary to distal renal tubular acidosis (dRTA) is made. She was later found to have sjogrens syndrome on the basis of positive ANA and Anti Ro (SS -A) antibody. Also Ultrasound abdomen reveals nephrocalcinosis which is commonly associated with dRTA. She was managed with potassium supplements, sodium bicarbonate and immunomodulators. Primary sjogrens 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 complications and better quality of life.