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.
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Acknowledgements
We are very grateful to Dr. Serena Bettoni and Dr. Elisabetta Valoti (Istituto di Ricerche Farmacologiche Mario Negri IRCCS, Clinical Research Center for Rare Diseases Aldo e Cele Daccò) for serum complement factor assessment and genetic analysis, respectively.
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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.
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Manenti, L., Urban, M.L., Maritati, F. et al. Complement blockade in ANCA-associated vasculitis: an index case, current concepts and future perspectives. Intern Emerg Med 12, 727–731 (2017). https://doi.org/10.1007/s11739-017-1636-6
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DOI: https://doi.org/10.1007/s11739-017-1636-6