Version May 2024
| 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 |
| Kidney transplant rejection |
| Wilson disease |
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