Rheopheresis: A new therapeutic approach in severe calciphylaxis.

Abstract

Uremic calciphylaxis, also called calcific uraemic arteriolopathy (CUA), is a rare disease with a poor prognosis (mortality between 45% and 80%). Treatment is currently not standardized, and is based mainly on risk factor control, often with administration of sodium thiosulfate. We report the use of rheopheresis, a double filtration apheresis technique, specifically designed to improve blood rheology and tissue perfusion, as adjunctive therapy in eight patients with severe CUA. We retrospectively analysed eight cases of severe CUA treated by rheopheresis after failure of conventional measures, including administration of sodium thiosulfate and discontinuation of vitamin K antagonists. Of the patients, there were 5 (63%) women, the median age was 69 (63.9-73) years. Four (50%) patients had biopsy-proven CUA. At diagnosis, the median dialysis vintage was 35 (3.9-42) months; five (63%) patients were anuric. Weekly median dialysis duration and dose were 12 (12-12.75) hours and 1.19 (1.13-1.48) Kt/V per dialysis session, respectively. Median time from CUA onset to first rheopheresis therapy was 26 (3.2-68) days. Patients started with 2-3 weekly sessions, coupled with haemodialysis. Complete remission was obtained in five patients (66%) after 25 (19-39) sessions over a duration of 119 (114-196) days. Three patients died, two of which resulted from an infectious complication related to CUA. Rheopheresis is a promising approach, with a good safety profile, for the treatment of CUA. A prospective study with a larger population, would clarify its place in the therapeutic armamentarium.