Raynaud's phenomenon triggered by the vasopressin V2 receptor antagonist tolvaptan in a patient with autosomal dominant polycystic kidney disease and Sjögren's syndrome

Abstract

Autosomal dominant polycystic kidney disease (ADPKD) is the most widespread monogenic kidney disease, accounting for 5–10% of all end-stage kidney disease cases among adults. Tolvaptan, a highly selective non-peptide arginine vasopressin V2 receptor antagonist, down-regulates the total kidney volume overload and delays kidney function decline in patients with ADPKD. The European Medicines Agency has approved the use of tolvaptan to delay the progression of cyst development and renal insufficiency in adult patients with ADPKD associated with chronic kidney disease (CKD) stages 1–3 while initiating treatment for cases with evidently rapid disease progression. The ERA-EDTA Working Groups on Inherited Kidney Disorders and European Renal Best Practice have proposed a hierarchical decision algorithm to accurately identify rapid disease progression.

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