A 73 year old white man presented with life threatening hypokalaemic paraly
sis requiring admission to an intensive care unit. Biochemical investigatio
ns showed severe hypokalaemia with hyperchloraemic metabolic acidosis, a sp
ot urine pH of 6.5, and a positive urinary anion gap, establishing the diag
nosis of distal renal tubular acidosis. Autoimmune tests revealed Sjogren s
yndrome as the underlying cause of the distal renal tubular acidosis. Full
recovery followed potassium and alkali replacement. This dramatic presentat
ion of Sjogren syndrome has not previously been reported in an elderly man.