Primary Sjogren’s syndrome is an autoimmune disorder primarily involving the exocrine glands but it can have a varied presentation. It can also present without classically described sicca symptoms. One-third of the patients experience extra-glandular involvement in the form of nonspecific arthralgia, myalgia, arthritis, parenchymal involvement of renal parenchyma, lung, and rarely nerves. Renal involvement in primary Sjogren’s can present with type 1 renal tubular acidosis (RTA) and is associated with a normal anion gap and hypokalemia. We, hereby, report a case of primary Sjogren’s syndrome presenting with type 1 RTA with hypokalemic quadriparesis as the first presenting feature.
Different Stages of Primary Sjögren’s Syndrome Involving Lymphotoxin and Type 1 IFN
