非典型HUSにおける遺伝子変異と腎移植時の抗補体治療

抄録

Atypical hemolytic uremic syndrome (aHUS) is a type of complement-mediated thrombotic microangiopathy; to date, causative genes including complement genes (CFH, CFI, CD46 (MCP), C3, CFB, THBD. DGKE, PLG, INF2, VTN) have been identified, and it has been reported that life outcomes, risk of disease recurrence, and prognosis of kidney transplantation differ depending on the presence and type of causative gene mutations. Recurrence of aHUS after kidney transplantation is a major risk for abolition of transplant kidney function The availability of anti-C5 monoclonal antibodies and prophylactic complement therapy with these drugs at the time of kidney transplantation has made it possible to perform kidney transplantation in combination with prophylactic complement therapy in patients classified as high risk for recurrence who could not undergo kidney transplantation due to high recurrence rate. In this article, we review the disease entity, epidemiology, pathophysiology, diagnosis and treatment of aHUS, as well as prophylactic treatment for kidney transplantation in patients with recurrence risk. We hope that future research will lead to more detailed risk assessments and decision-making on indications for prophylactic complement therapy.