Aim: 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.
Methods: 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.
Results: 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.
Conclusion: 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.
Keywords: calciphylaxis; haemodialysis; rheopheresis; survival; wound.
© 2019 Asian Pacific Society of Nephrology.