In autosomal dominant tubulointerstitial kidney disease driven by a mutation in the renin (REN) gene, some patients develop symptomatic hypotension. When this occurs without declining kidney function, hypertension, hyperkalemia, or edema, a specific pharmacological approach is indicated to resolve hypotensive symptoms.
ADTKD caused by a REN gene mutation, presenting with symptomatic hypotension. Key clinical features that define eligibility for this protocol: no declining kidney function, no hypertension, no hyperkalemia, and no edema. The presence of any of these features changes the risk–benefit profile and alters the treatment choice.
A targeted pharmacological agent is available for symptomatic hypotension in this setting. Its use is conditional on the specific clinical exclusion criteria above — the full protocol defines when it applies, the conditions under which it must be avoided, and all further management details.
Treatment goal: Resolution of symptomatic hypotension.
DOI: 10.1038/ki.2015.28
In ADTKD-REN patients, erythropoiesis-stimulating agents and fludrocortisone can be used for the treatment of anemia and symptomatic hypotension, respectively.
Fludrocortisone should not be used in those with declining kidney function, hypertension, hyperkalemia, or edema.
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