RT Book, Section
A1 Yazdany, Jinoos
A1 Manno, Rebecca L.
A2 Papadakis, Maxine A.
A2 McPhee, Stephen J.
A2 Rabow, Michael W.
A2 McQuaid, Kenneth R.
SR Print(0)
ID 1184193379
T1 Eosinophilic Granulomatosis with Polyangiitis
T2 Current Medical Diagnosis & Treatment 2022
YR 2022
FD 2022
PB McGraw-Hill Education
PP New York, NY
SN 9781264269389
LK accessmedicine.mhmedical.com/content.aspx?aid=1184193379
RD 2024/04/19
AB Eosinophilic  granulomatosis  with  polyangiitis  (previously  called  Churg-Strauss  syndrome)  is  an  ANCA-associated  vasculitis  (along  with  granulomatosis  with  polyangiitis  and  microscopic  polyangiitis),  although  the  presence  of  ANCA  occurs  in  less  than  50%  of  patients  (usually  anti-MPO).  It  is  characterized  by  peripheral  eosinophilia,  sinusitis  with  polyposis,  asthma,  lung  infiltrates,  vasculitic  skin  involvement,  glomerulonephritis,  and  vasculitic  neuropathy.  Myocarditis  can  lead  to  arrhythmias  and  heart  failure  if  untreated.  Eosinophilic  granulomatosis  with  polyangiitis  should  be  considered  in  patients  with  an  unexplained  peripheral  eosinophilia  and  vasculitic  features.  Laboratory  examination  of  unexplained  eosinophilia  should  include  obtaining  ANCA  and  serum  tryptase  peripheral  flow  cytometry  for  PDGF  receptor  abnormalities  and  testing  for  helminthic  infections.  Eosinophilic  infiltrates  on  tissue  samples  strongly  suggest  the  diagnosis  of  eosinophilic  granulomatosis,  especially  if  accompanied  by  vasculitis  (purpura,  glomerulonephritis,  vasculitic  ulcers,  mononeuritis  multiplex).  Corticosteroids  remain  first-line  treatment  with  azathioprine  and  methotrexate  demonstrating  efficacy  for  mild  to  moderate  disease.  Mepolizumab,  an  IL-5  inhibitor,  is  FDA  approved  for  the  treatment  of  eosinophilic  granulomatosis  with  polyangiitis,  although  it  has  not  been  studied  for  severe  life-  or  organ-threatening  vasculitic  disease  manifestations  (which  generally  require  cyclophosphamide).  Rituximab  may  be  considered  as  an  option  for  patients  with  refractory  disease.