- Group of predominantly antilysosomal autoantibodies (mostly IgG), which are primarily detected using indirect immunofluorescence techniques. Three different fluorescence patterns are observed, which leads to the classification into three subtypes: cANCA (classical ANCA), pANCA (perinuclear ANCA), atypical ANCA (aANCA or xANCA) (see Table 1).
- Occurrence in vasculitis, chronic inflammatory rheumatic syndromes, chronic liver and intestinal diseases and infections. ANCA-associated vasculitides include Wegener's granulomatosis, Churg-Strauss syndrome and microscopic polyangiitis.
Antibodies, antineutrophilic cytoplasmic
DefinitionThis section has been translated automatically.
General informationThis section has been translated automatically.
ANCA cytokine sequence theory: Under proinflammatory cytokine influence, translocation of intracellular ANCA target antigens onto the cytoplasmic membrane of neutrophils and monocytes and secretion of adhesion molecules (e.g. ICAM) of neutrophils and endothelial cells. Degranulation of granulocytes with endothelial cell damage and consecutive necrotizing vasculitis. Blocking of the natural deactivation of the receptor molecule protease 3 by ANCA. For vasculitis with additional symptoms in the upper respiratory tract, lung and/or kidney, ANCA-associated vasculitis must be considered, see Table 2.
DiagnosisThis section has been translated automatically.
The standard screening method is ANCA detection with indirect immunofluorescence technique (fluorescent antibodies against granulocyte-bound ANCA). ELISA, RIA, immunoblotting and immunoprecipitation are also possible.
LiteratureThis section has been translated automatically.
- Day CJ et al (2003) New developments in the pathogenesis of ANCA-associated vasculitis. Clin Exp Rheumatol 21(6 Suppl 32): S35-48
- Gross WL et al (1995) ANCA-associated vasculitis. Dermatologist 46: 511-524
- Kallenberg CG et al (2002) New insights into the pathogenesis of antineutrophil cytoplasmic autoantibody-associated vasculitis. Autoimmune Rev 1: 61-66
- Keogh KA et al (2003) Churg-Strauss syndrome: clinical presentation, antineutrophil cytoplasmic antibodies, and leukotriene receptor antagonists. On J Med 115: 284-290
TablesThis section has been translated automatically.
ANCA subtypes, target antigens and associated diseases (according to Gross WL and Schmitt WH)
Acronym |
Target antigen(s) |
Associated diseases |
cANCA |
Proteinase 3 = PR3 |
Wegener's granulomatosis; more rarely in microscopic polyangiitis, Churg-Strauss syndrome and amoeba-induced liver abscess |
pANCA |
myeloperoxidase (MPO) |
Microscopic polyangiitis, renal vasculitis (idiopathic rapid-progressive GN) |
lactoferrin, cathepsin G, elastase, lysozyme |
Inflammatory rheumatic diseases like rheumatoid arthritis and vasculitis, SLE, Sjögren's syndrome |
|
a/pANCA |
BPIa (= CAP57)? |
Ulcerative colitis, primary sclerosing cholangitis, 10-20% Crohn's disease |
ANCA in vasculitis, collagenosis and other rheumatic diseases (according to Gross WL and Schmitt WH)
Entity |
CANCA [%] |
pANCA [%] |
Target antigen(s) |
|
Vasculitides |
Wegener's granulomatosis (initial phase) |
50 |
< 5 |
PR3a |
Wegener's granulomatosis (generalisation) |
90 |
< 5 |
PR3a |
|
Churg-Strauss Syndrome |
20 |
20 |
PR3, MPOa |
|
Microscopic polyangiitis |
10 |
60 |
||
Purpura Beauty Enoch |
- |
< 5 |
||
cryoglobulinemic vasculitis |
- |
- |
||
Leucocytoclastic vasculitis |
- |
- |
||
polyarteritis nodosa |
< 5 |
< 5 |
||
Kawasaki's Arteritis |
- |
- |
||
Giant Cell Arteritis |
- |
5 |
||
Takayasu arteritis |
- |
- |
||
| ||||
Collagenoses and related inflammatory rheumatic diseases |
SLE |
- |
25 |
LF, HLE, LZb |
Sjögren's Syndrome |
- |
25 |
LF, HLE, CG, LZb |
|
Polymyositis |
- |
< 10 |
||
Rheumatoid Arthritis |
- |
20 |
LF, HLE, CG, LZb |
|
rheumatoid vasculitis |
- |
50 |
LF, HLE, CG, LZb |
|
Felty Syndrome |
- |
50 |
LF, HLE, CG, LZb |
|
Spondylarthritides |
- |
< 10 |
||