- In adults, the most common cause is autoimmune disease, eg, Sjögren syndrome or rheumatoid arthritis, or hypercalciuria. In otherwise healthy adults with unexplained distal RTA, diagnosis of Sjögren syndrome should be considered.
- In children, hereditary causes of distal RTA are most common and are due to genetic mutations in the basolateral chloride-bicarbonate exchanger and in the apical hydrogen-ATPase. The autosomal recessive form presents in infancy, whereas the autosomal dominant form typically presents in adolescence or adulthood.
- Both ifosfamide and toluene ingestion can cause distal RTA in children and adults.
Distal renal tubular acidosis
N25.89 – Other disorders resulting from impaired renal tubular function
236461000 – Distal renal tubular acidosis
Differential Diagnosis & Pitfalls
- Chronic diarrhea – Urine pH may be inappropriately elevated in this setting; however, the urine anion gap should be negative.
- Ileal loop urinary diversion
- Bladder-drained pancreas transplant
- Toluene inhalation
Drug Reaction Data