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SP0012 Complement in the Rheumatic Disease: Complementopathies
  1. M. C. Pickering1
  1. 1Centre for Complement and Inflammation Research, Imperial College, London, United Kingdom

Abstract

Complement is an integral component of immunity. Inherited deficiencies in the ability to activate complement increases susceptibility to bacterial infection and autoimmunity. Examples include: meningitis and terminal component deficiency; systemic lupus erythematosus-like illness and classical pathway deficiency. Inherited deficiencies in the ability to control complement activation result in damage to host tissue. Examples include: red cell lysis in paroxysmal nocturnal haemoglobinuria (PNH); glomerular inflammation in C3 glomerulopathy; glomerular thrombosis in atypical haemolytic uraemic syndrome (aHUS). Therapeutic inhibition of complement activation is achieved through antibody-mediated inhibition of C5 activation (eculizumab). Eculizumab is currently licenced for both aHUS and transfusion-dependent PNH. I will discuss how complement deficiency results in disease, our current treatment approaches and how these may apply to rheumatic disease.

Disclosure of Interest None Declared

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