Abstract
Sjögren's syndrome (SS) is an autoimmune disorder with glandular and extra glandular manifestations. The extra glandular manifestations include renal symptoms, primarily tubulointerstitial nephritis (TIN), while the glandular component involves the lymphocytic infiltration of exocrine glands. We describe the case of a 28-year-old woman who experienced two bouts of sub-acute onset recurrent flaccid quadriparesis in four months. Following the initial onset of quadriparesis, additional symptoms emerged, including dysphagia, dry mouth, and dry eyes, which raised the suspicion of underlying SS. Examination revealed a positive urine anion gap suggestive of distal renal tubular acidosis (DRTA), high urine pH, and severe hypokalemic hyperchloremic metabolic acidosis. The Schirmer test was positive after five minutes, and an additional workup confirmed positive anti-Ro and anti-La antibodies. A tissue biopsy collected from the sublingual salivary gland indicated lymphocytic infiltration, acinar atrophy, ductal dilatation, and epimyoepithelial cell islands on histological analysis, confirming SS to be the underlying cause of the symptoms. We stress in our conclusion that SS can also initially manifest without the classic symptoms of sicca and that it should be taken into consideration in cases of recurrent hypokalemic flaccid quadriparesis in the context of DRTA.