Complement alternative pathway (cAP) hyperactivation seems to be involved in ANCA-associated vasculitis (AAV). We here describe a case of AAV with severe activation of cAP that developed acute renal failure. No mutation predisposing to cAP dysregulation was identified. We treated our patient with the standard immunosuppressive therapy, but disease progression was only reversed after the addition of eculizumab, a monoclonal antibody against C5; the patient eventually achieved an almost complete renal function recovery. A review of the available literature about the role of complement targeted therapies in the treatment of AAV is discussed.
Complement blockade in ANCA-associated vasculitis: an index case, current concepts and future perspectives / Manenti, Lucio; Urban, Maria Letizia; Maritati, Federica; Galetti, Maricla; Vaglio, Augusto. - In: INTERNAL AND EMERGENCY MEDICINE. - ISSN 1970-9366. - ELETTRONICO. - 12:(2017), pp. 727-731. [10.1007/s11739-017-1636-6]
Complement blockade in ANCA-associated vasculitis: an index case, current concepts and future perspectives
Urban, Maria Letizia;Vaglio, Augusto
2017
Abstract
Complement alternative pathway (cAP) hyperactivation seems to be involved in ANCA-associated vasculitis (AAV). We here describe a case of AAV with severe activation of cAP that developed acute renal failure. No mutation predisposing to cAP dysregulation was identified. We treated our patient with the standard immunosuppressive therapy, but disease progression was only reversed after the addition of eculizumab, a monoclonal antibody against C5; the patient eventually achieved an almost complete renal function recovery. A review of the available literature about the role of complement targeted therapies in the treatment of AAV is discussed.I documenti in FLORE sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.