Journal Mobile

DC Kluth, J Hughes
Journal Issue: 
Volume 37: Issue 2: 2007




Anti-neutrophil cytoplasmic antibody-associated systemic vasculitis is a multi-system  disease  that  can  present  to  a  number  of  medical  specialties, and where early identification and treatment improves outcome. The vasculitides are commonly divided into WG, MPA, and CSS.  This review will revise the common clinical  presentations  of  these  diseases  and  initial  investigations.  Anti-neutrophil cytoplasmic antibodies have provided a valuable test to aid in diagnosis and there is  increasing  clinical  and  experimental  evidence  that  suggests  they  have  an important  role  in  the  pathogenesis  of  disease.    Anti-neutrophil  cytoplasmic antibodies are directed at either MPO or PR3 which are present in the granules and  on  the  cell  surface  of  neutrophils.   They  can  be  detected  by  indirect immunofluoresence and ELISA-based assay, and both are essential when vasculitis is suspected. The antibodies are able to activate neutrophils and promote injury, and  recent  data  have  shown  they  can  directly  cause  glomerulonephritis  and systemic vasculitis in animal models.

Diagnosis  of  AASV  is  made  using  clinical  features, ANCA  test, and  relevant  tissue biopsy.  Treatment  with  cytotoxics  has  transformed  the  prognosis  but  until  recently there has been limited trial evidence to determine which regime is best. This article will  review  a  range  of  studies  published  over  the  last  five  years  and  preliminary evidence  from  ongoing  studies  to  give  an  outline  of  current  practice  for immunosuppression and monitoring of disease. There is also now a range of biological therapies  available  including  anti-TNF  and  anti-B-cell  antibodies, which  may  have  an increasing role as either adjunctive therapy or new regimes to induce remission.