Major causes of type 1 (distal) renal tubular acidosis (RTA)
Primary |
Idiopathic (sporadic) |
Familial |
Autosomal dominant (mainly due to mutations causing defects in the kidney anion exchanger [kAE1] in distal tubule/collecting duct intercalated cells) |
Autosomal recessive (due to a variety of mutations causing defects in one of the multiple subunits of V-ATPase in distal tubule/collecting duct intercalated cells) |
Secondary |
Autoimmune disorders |
Sjögren's syndrome |
Autoimmune hepatitis/primary biliary cirrhosis |
Systemic lupus erythematosus (also can generate hyperkalemic RTA) |
Rheumatoid arthritis |
Drugs |
Ifosfamide (also can cause proximal RTA with Fanconi syndrome) |
Amphotericin B |
Lithium carbonate |
Ibuprofen |
Hypercalciuric conditions |
Hyperparathyroidism |
Vitamin D intoxication |
Sarcoidosis |
Idiopathic hypercalciuria |
Other |
Medullary sponge kidney |
Obstructive uropathy |
Renal transplant rejection |
Wilson disease |
REF. UpToDate 2020.08.11