PMID- 12195410
OWN - NLM
STAT- MEDLINE
DCOM- 20031126
LR  - 20131121
IS  - 0393-5590 (Print)
IS  - 0393-5590 (Linking)
VI  - 19
IP  - 2
DP  - 2002 Mar-Apr
TI  - [ANCA-associated vasculitis].
PG  - 125-36
AB  - ANCA-associated vasculitis. The term "antineutrophil cytoplasm antibody (ANCA)- 
      associated vasculitis" (AASV) ihighers generally used to include primary 
      vasculitis syndromes in which circulating ANCA against proteinase 3 (PR3) and 
      myeloperoxidase (MPO) are commonly found. AASV syndromes include Wegener's 
      granulomatosis, microscopic polyangiitis, idiopathic pauci- immune necrotizing 
      crescentic glomerulonephritis and Churg-Strauss syndrome (CSS). AASV syndromes 
      share some general clinical-histological manifestations, such as rapidly 
      progressive renal failure and focal necrotizing glomerulonephritis with 
      extracapillary proliferation in the absence (or in the presence of modest) 
      immunoglobulins deposits (pauci- immune). Untreated AASV follow a progressive 
      course with a fatal outcome due to vital organ failure. The combination of 
      cyclophosphamide and prednisone is now established as the treatment of choice for 
      patients with AASV, but there is considerable debate over the duration of therapy 
      and the best way to administer cyclophosphamide. Treatment of AASV can be divided 
      into two phases: an induction of remission and a maintenance of remission phase. 
      Patients with AASV and renal involvement (serum creatinine less than 500 ml/L or 
      5.6 mg/dl) should be treated with a combination of oral prednisone with gradual 
      tapering and cyclophosphamide. Once remission is achieved, usually after 3-6 
      months, azathioprine should replace cyclophosphamide. It is not known for how 
      long treatment should be continued but at least one year of treatment after 
      remission is warranted. When serum creatinine is than 500 ml/L (5.6 mg/dl) and/or 
      oliguria is present, the addition of methylprednisolone pulses and/or plasma 
      exchange should be considered.
FAU - Sinico, R A
AU  - Sinico RA
AD  - Unita' Operativa di Nefrologia e Centro di Immunologia Clinica e Reumatologia, 
      Milano, Italy. renato.sinico@oscb.sined.net
FAU - Sabadini, E
AU  - Sabadini E
FAU - Boeri, R
AU  - Boeri R
FAU - Radice, A
AU  - Radice A
LA  - ita
PT  - English Abstract
PT  - Journal Article
PT  - Review
TT  - Vasculiti anca-associate.
PL  - Italy
TA  - G Ital Nefrol
JT  - Giornale italiano di nefrologia : organo ufficiale della Societa italiana di 
      nefrologia
JID - 9426434
RN  - 0 (Antibodies, Antineutrophil Cytoplasmic)
RN  - 0 (Autoantigens)
RN  - 0 (Immunosuppressive Agents)
RN  - 8N3DW7272P (Cyclophosphamide)
RN  - EC 1.11.1.7 (Peroxidase)
RN  - EC 3.4.21.- (Serine Endopeptidases)
RN  - EC 3.4.21.76 (Myeloblastin)
RN  - MRK240IY2L (Azathioprine)
RN  - VB0R961HZT (Prednisone)
SB  - IM
MH  - Antibodies, Antineutrophil Cytoplasmic/*immunology
MH  - Autoantigens/immunology
MH  - Autoimmune Diseases/classification/*immunology/pathology/therapy
MH  - Azathioprine/administration & dosage/therapeutic use
MH  - Cyclophosphamide/administration & dosage/therapeutic use
MH  - Humans
MH  - Immunosuppressive Agents/administration & dosage/therapeutic use
MH  - Kidney Failure, Chronic/immunology
MH  - Myeloblastin
MH  - Neutrophils/enzymology/immunology
MH  - Peroxidase/immunology
MH  - Plasma Exchange
MH  - Prednisone/administration & dosage/therapeutic use
MH  - Remission Induction
MH  - Serine Endopeptidases/immunology
MH  - Vasculitis/classification/*immunology/pathology/therapy
RF  - 57
EDAT- 2002/08/27 10:00
MHDA- 2003/12/03 05:00
CRDT- 2002/08/27 10:00
PHST- 2002/08/27 10:00 [pubmed]
PHST- 2003/12/03 05:00 [medline]
PHST- 2002/08/27 10:00 [entrez]
PST - ppublish
SO  - G Ital Nefrol. 2002 Mar-Apr;19(2):125-36.