RT Book, Section
A1 Stone, John H.
A2 Stone, John H.
SR Print(0)
ID 1180197326
T1 Granulomatosis with Polyangiitis
T2 Current Diagnosis & Treatment: Rheumatology, 4e
YR 2021
FD 2021
PB McGraw Hill
PP New York, NY
SN 9781259644641
LK accessmedicine.mhmedical.com/content.aspx?aid=1180197326
RD 2024/04/23
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.Migratory  pauciarticular  or  polyarticular  arthritis.Persistent  upper  respiratory  tract  and  ear  “infections”  that  do  not  respond  to  antibiotic  therapy.Orbital  pseudotumor,  nearly  always  associated  with  chronic  nasosinus  conditions.Nodular  or  cavitary  lung  lesions  that  are  misdiagnosed  initially  as  malignancies  or  infections.Rapidly  progressive  glomerulonephritis.Antineutrophil  cytoplasmic  antibody  (ANCA)  assays  are  extremely  helpful  in  diagnosis  but  have  significant  shortcomings  as  indicators  of  disease  activity  or  guides  to  when  to  treat.  A  significant  minority  of  patients  with  granulomatosis  with  polyangiitis  are  ANCA  negative,  particularly  those  with  “limited”  disease.