원위부 신세뇨관성 산증과 급성 췌장염이 동반된 Sjogren증후군

Distal renal tubular acidosis is classified into primary and secondary. Sjogren Syndrome is the most common cause of secondary distal RTA. We experienced a 51 year-old female patient who had many manifestations of primary Sjogren syndrome which showes Distal renal tubular acidosis, acute pancreatitis, acute renal failure, possibly nephrogenic diabetes insipidus, but We found other atypical find-ings including ANA negative serology, high anion gap metabo1ic acidosis.