RT Book, Section
A1 Stone, John H.
A2 Imboden, John B.
A2 Hellmann, David B.
A2 Stone, John H.
SR Print(0)
ID 57273174
T1 Chapter 32. Granulomatosis with Polyangiitis (Wegener Granulomatosis)
T2 CURRENT Diagnosis & Treatment: Rheumatology, 3e
YR 2013
FD 2013
PB The McGraw-Hill Companies
PP New York, NY
SN 978-0-07-163805-0
LK accessmedicine.mhmedical.com/content.aspx?aid=57273174
RD 2023/02/07
AB Three  pathologic  hallmarks:  granulomatous  inflammation,  vasculitis,  and  necrosis.Classic  clinical  features  are  found  in  multiple  organ  systems:Nonspecific  constitutional  symptoms,  such  as  fatigue,  myalgias,  weight  loss,  and  fevers.Persistent  upper  respiratory  tract  and  ear  “infections”  that  do  not  respond  to  antibiotic  therapy.Orbital  pseudotumor,  nearly  always  associated  with  chronic  nasosinus  conditions.Migratory  pauciarticular  or  polyarticular  arthritis.Nodular  or  cavitary  lung  lesions  that  are  misdiagnosed  initially  as  malignancies  or  infections.Rapidly  progressive  glomerulonephritis.Antineutrophil  cytoplasmic  antibody  (ANCA)  assays  are  helpful  in  diagnosis  if  positive  by  both  immunofluorescence  and  enzyme  immunoassay.  A  significant  minority  of  patients  with  granulomatosis  with  polyangiitis  are  ANCA-negative,  particularly  those  with  “limited”  disease.