저자들은 저칼륨혈증 및 신석회증을 각각 주 증상으로 발현된 쇼그렌증후군 동반 원위신세관산증 2예를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

Renal tubular acidosis (RTA) is a syndrome characterized by hyperchloremic metabolic acidosis and an inability to excrete highly acid urine, in which the impaired acid excretion is disproportional to the reduction in the glomerular filtration rate. Distal renal tubular acidosis (dRTA) is frequently associated with immune-mediated disease, including Sjogren’s syndrome. Sjogren’s syndrome is a systemic autoimmune disease that mainly affects exocrine glands, such as the lacrimal and salivary glands, resulting in xerophthalmia and xerostomia. Extraglandular manifestations are frequent and may include renal involvement. Recently, we experienced two cases of renal tubular acidosis in patients with Sjogren’s syndrome. The first patient had lower extremity weakness and hypokalemia and the second had nephrocalcinosis. We discuss the frequency and pathogenesis of dRTA in Sjogren’s syndrome. (Korean J Med 2016;90:248-252)