Inflammation plays a critical role in 2,8-dihydroxyadenine nephropathy

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Bouderlique, Elise | Tang, Ellie | Perez, Joëlle | Ea, Hang-Korng | Renaudin, Felix | Coudert, Amélie | Vandermeersch, Sophie | Bazin, Dominique | Haymann, Jean-Philippe | Saint-Jacques, Camille | Frochot, Vincent | Daudon, Michel | Letavernier, Emmanuel

Edité par CCSD ; Académie des sciences (Paris) -

International audience. Adenine phosphoribosyltransferase (APRT) deficiency is a genetic disease characterized by an increased production of 2,8 dihydroxyadenine (2,8-DHA) precipitating in urine, leading to a crystalline nephropathy and end-stage renal disease. Here, we describe the high prevalence of granuloma (88%) in biopsies from patients with APRT deficiency. A murine model of 2,8-DHA nephropathy was generated, showing that anakinra or dexamethasone, combined with allopurinol, improved renal function to a larger extent than allopurinol alone, the standard therapy. Inflammation plays a critical role in the development of 2,8-DHA nephropathy, and therapy based upon drugs targeting innate immunity could improve renal function recovery.

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