What are the causes of distal renal tubular acidosis (type I)?

Updated: Sep 03, 2020
  • Author: Sai-Ching Jim Yeung, MD, PhD, FACP; Chief Editor: Romesh Khardori, MD, PhD, FACP  more...
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A summary of the causes of dRTA (type I) is as follows:

  • Primary - Idiopathic, isolated, and sporadic

  • Tubulointerstitial conditions - Renal transplantation, chronic pyelonephritis, obstructive uropathy, and leprosy

  • Genetic - Familial, Marfan syndrome, Wilson disease, Ehlers-Danlos syndrome, medullary cystic disease (dRTA and pRTA), and osteopetrosis

  • Conditions associated with nephrocalcinosis - Hyperoxaluria, primary hypercalciuria, hyperthyroidism, primary hyperparathyroidism, vitamin D intoxication, milk-alkali syndrome, and medullary sponge kidney

  • Autoimmune disorders - Chronic active hepatitis, primary biliary cirrhosis, Sjögren syndrome (dRTA and pRTA), systemic lupus erythematosus, autoimmune thyroiditis, pulmonary fibrosis, and vasculitis

  • Drugs and toxicity - Amphotericin B, analgesics, lithium, toluene, ifosfamide (dRTA and pRTA)

  • Hypergammaglobulinemic states - Myeloma (both dRTA and pRTA), amyloidosis (dRTA and pRTA), and cryoglobulinemia

  • Miscellaneous - Hepatic cirrhosis and acquired immunodeficiency syndrome (AIDS) (possibly)

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