istal renal tubular acidosis (DRTA) is frequently associated with immune-mediated diseases such as systemic lupus erythematosus (SLE), Sjogren's syndrome, idiopathic hypergammaglobulinemia, autoimmune liver diseases, autoimmune thyroid diseases,
renal
transplant rejection, rheumatoid arthritis, and ankylosing spondylitis. We report two cases of distal renal tubular acidosis associated with immune-mediated diseases. The first case was a patient with SLE, who had general weakness, vomiting, and
hypokalemia, and the second case was a patient with Sjogren syndrome, who had quadriparesis, respiratory arrest, and hypokalemia. DRTA was diagnosed with short-term NH4CI loading tests in both patients. In the second case, we performed NaHCO3
test,
which reconfirmed DRTA. The mechanism of DRTA, confirmed by 4 % Na2SO4 loading tests, was a hydrogen ion secretory pump defect in both patients. We discussed the mechanisms of DRTA and the methods that could differentiate the mechanisms, and the
frequencies and the pathogensis of DRTA in SLE and in Sjogren syndrome.
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