RARE PRESENTATION OF A PRIMARY SJOGREN SYNDROME (A CASE REPORT)

Sjogren syndrome (SS) is a slowly progressive inflammatory disorder that involves the exocrine glands manifesting as keratoconjunctivitis and xerostomia. SS may be a primary disorder or may be secondary to other autoimmune disorders, such as systemic lupus erythematosus, rheumatoid arthritis, scleroderma and biliary cirrhosis. Extraglandular manifestations such as arthralgia, arthritis, Raynauds phenomenon, pulmonary disease, gastrointestinal disease, leukopenia, anemia, lymphadenopathy, neuropathy, vasculitis, renal manifestations and lymphoma etc., may mimic these other autoimmune disorders. The predominant form of renal disease in primary SS is distal renal tubular acidosis (d-RTA) syndrome resulting from tubulointerstitial lymphocytic infiltration. Though overt renal disease of any form is seen in about 4 of primary SS(1), the first presenting symptom as hypokalemic paralysis is very rare. In this case report, we describe a case of a patient with SS in whom the first manifestation of disease was paralysis due to hypokalemia and distal renal tubular acidosis. This type of presentation is rare since only a few cases have been reported in the literature.(2,3)