Management of autosomal dominant tubulointerstitial kidney disease (ADTKD) is broadly aligned with established chronic kidney disease guidelines, with specific considerations arising from the condition's effects on urinary concentration and its particular sensitivity to certain commonly used medications.
The protocol follows established CKD management frameworks and addresses fluid intake requirements stemming from impaired urinary concentration. Specific guidance covers which classes of agents may have a role in management and — critically — which medications carry particular risks and must be approached with caution or avoided entirely. The full structured regimen is available in the protocol.
DOI: 10.1038/ki.2015.28
Affected individuals should in general be treated according to established CKD guidelines.
Liberal water intake is recommended to compensate for possible urinary concentration defects.
There are no data in ADTKD patients concerning possible benefits of treatment with angiotensin-converting enzyme inhibitors or angiotensin receptor blockers on CKD progression.
Nonsteroidal anti-inflammatory drugs should be avoided in all patients with ADTKD; in particular patients with REN mutations are highly susceptible to acute worsening of kidney function in response to these agents.
View source ↗