Antibodies, antineutrophilic cytoplasmic

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Definition
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  • 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.

General information
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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.

Diagnosis
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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.

Literature
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  1. Day CJ et al (2003) New developments in the pathogenesis of ANCA-associated vasculitis. Clin Exp Rheumatol 21(6 Suppl 32): S35-48
  2. Gross WL et al (1995) ANCA-associated vasculitis. Dermatologist 46: 511-524
  3. Kallenberg CG et al (2002) New insights into the pathogenesis of antineutrophil cytoplasmic autoantibody-associated vasculitis. Autoimmune Rev 1: 61-66
  4. Keogh KA et al (2003) Churg-Strauss syndrome: clinical presentation, antineutrophil cytoplasmic antibodies, and leukotriene receptor antagonists. On J Med 115: 284-290

Tables
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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

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Last updated on: 29.10.2020