RT Book, Section
A1 Duvuru, Geetha
A1 Stone, John H.
A2 Stone, John H.
SR Print(0)
ID 1180197690
T1 IgA Vasculitis (Henoch-Schönlein Purpura)
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=1180197690
RD 2022/08/12
AB The  sine  qua  non  of  IgA  vasculitis  (formerly  Henoch-Schönlein  purpura)  is  nonthrombocytopenic  purpura,  caused  by  inflammation  in  blood  vessels  of  the  superficial  dermis.The  pathologic  hallmarks  of  IgA  vasculitis  are  a  leukocytoclastic  vasculitis  and  deposition  of  immunoglobulin  (Ig)  A  in  the  walls  of  involved  blood  vessels.The  tetrad  of  purpura,  arthritis,  glomerulonephritis,  and  abdominal  pain  is  often  observed.  However,  all  four  elements  are  not  required  for  the  diagnosis.More  than  90%  of  cases  occur  in  children.  The  disease  is  self-limited  most  of  the  time,  resolving  within  a  few  weeks.  Adult  cases  are  sometimes  more  recalcitrant.Renal  insufficiency  develops  in  less  than  5%  of  patients  with  IgA  vasculitis.  The  long-term  renal  prognosis  depends  mainly  on  the  degree  of  initial  damage  to  the  kidney.IgA  vasculitis  can  be  mimicked  by  other  forms  of  systemic  vasculitis  that  are  more  often  life-threatening.  For  example,  antineutrophil  cytoplasmic  antibody  (ANCA)–associated  vasculitides  such  as  granulomatosis  with  polyangiitis  and  microscopic  polyangiitis  may  also  present  with  purpura,  arthritis,  and  renal  inflammation.  Both  of  these  disorders  have  the  potential  for  serious  involvement  of  other  organs  (eg,  the  lungs  and  peripheral  nerves)  and  carry  more  dire  renal  prognoses.