Anti-neutrophil cytoplasmic antibody

ANCAanti-neutrophil cytoplasmic antibodiesantineutrophil cytoplasmic antibodiesANCA-associated vasculitidesantineutrophil cytoplasmic antibodyantibodies, antineutrophil cytoplasmicANCA Associated VasculitisANCA vasculitisANCA-associated vasculitisANCAs
Anti-neutrophil cytoplasmic antibodies (ANCAs) are a group of autoantibodies, mainly of the IgG type, against antigens in the cytoplasm of neutrophil granulocytes (the most common type of white blood cell) and monocytes.wikipedia
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Autoantibody

autoantibodiesauto-antibodiesanti-drug antibodies
Anti-neutrophil cytoplasmic antibodies (ANCAs) are a group of autoantibodies, mainly of the IgG type, against antigens in the cytoplasm of neutrophil granulocytes (the most common type of white blood cell) and monocytes.

Vasculitis

vasculitidesvasculiticvasculitis, central nervous system
They are detected as a blood test in a number of autoimmune disorders, but are particularly associated with systemic vasculitis, so called ANCA-associated vasculitides (AAV). ANCAs are associated with small vessel vasculitides including granulomatosis with polyangiitis, microscopic polyangiitis, primary pauci-immune necrotizing crescentic glomerulonephritis (a type of renal-limited microscopic polyangiitis), eosinophilic granulomatosis with polyangiitis and drug induced vasculitides.

Proteinase 3

proteinase-3PRTN3
The c-ANCA antigen is specifically proteinase 3 (PR3).
It is also the target of anti-neutrophil cytoplasmic antibodies (ANCAs) of the c-ANCA (cytoplasmic subtype) class, a type of antibody frequently found in the disease granulomatosis with polyangiitis.

Myeloperoxidase

MPOverdoperoxidase.
p-ANCA antigens include myeloperoxidase (MPO) and bacterial permeability increasing factor (BPI).
Antibodies are also known as anti-neutrophil cytoplasmic antibodies (ANCAs), though ANCAs have also been detected in staining of the perinuclear region.

Granulomatosis with polyangiitis

Wegener's granulomatosiscoin lesion, pulmonary
It has also been shown that up to 70% of patients with granulomatosis with polyangiitis (previously known as Wegener's granulomatosis) are chronic nasal carriers of Staphylococcus aureus, with carriers having an eight times increased risk of relapse. ANCAs are associated with small vessel vasculitides including granulomatosis with polyangiitis, microscopic polyangiitis, primary pauci-immune necrotizing crescentic glomerulonephritis (a type of renal-limited microscopic polyangiitis), eosinophilic granulomatosis with polyangiitis and drug induced vasculitides.
It is now widely presumed that the anti-neutrophil cytoplasmic antibodies (ANCAs) are responsible for the inflammation in GPA.

Rapidly progressive glomerulonephritis

Rapidly progressing glomerulonephritiscrescentsglomerular crescent
ANCAs are associated with small vessel vasculitides including granulomatosis with polyangiitis, microscopic polyangiitis, primary pauci-immune necrotizing crescentic glomerulonephritis (a type of renal-limited microscopic polyangiitis), eosinophilic granulomatosis with polyangiitis and drug induced vasculitides.
In the pathophysiology of rapidly progressive glomerulonephritis the antineutrophil cytoplasmic antibodies (ANCA) interact with antigens in cytoplasm of neutrophils.

Eosinophilic granulomatosis with polyangiitis

Churg-Strauss syndromeChurg–Strauss syndromeAllergic angiitis
ANCAs are associated with small vessel vasculitides including granulomatosis with polyangiitis, microscopic polyangiitis, primary pauci-immune necrotizing crescentic glomerulonephritis (a type of renal-limited microscopic polyangiitis), eosinophilic granulomatosis with polyangiitis and drug induced vasculitides.

Primary sclerosing cholangitis

sclerosing cholangitisCholangitis, primary sclerosingcholangitis, sclerosing
c-ANCA (atypical) is present in 80% of cystic fibrosis (with BPI as the target antigen) and also in inflammatory bowel disease, primary sclerosing cholangitis and rheumatoid arthritis (with antibodies to multiple antigenic targets).
For example, approximately 80% of people with PSC have perinuclear anti-neutrophil cytoplasmic antibodies (P-ANCA); however, this and other immunoglobulin findings are not specific to those with PSC and are of unclear clinical significance/consequence.

Microscopic polyangiitis

ANCAs are associated with small vessel vasculitides including granulomatosis with polyangiitis, microscopic polyangiitis, primary pauci-immune necrotizing crescentic glomerulonephritis (a type of renal-limited microscopic polyangiitis), eosinophilic granulomatosis with polyangiitis and drug induced vasculitides.

Glomerulonephritis

C3 glomerulonephritisglomerular nephritisglomerulonephritis, membranoproliferative
ANCAs were originally described in Davies et al. in 1982 in segmental necrotising glomerulonephritis, and by van der Woude et al. in 1985 in granulomatosis with polyangiitis.

Immunoglobulin G

IgGIgG1IgG4
Anti-neutrophil cytoplasmic antibodies (ANCAs) are a group of autoantibodies, mainly of the IgG type, against antigens in the cytoplasm of neutrophil granulocytes (the most common type of white blood cell) and monocytes.

Antigen

antigensantigenicantigenic proteins
Anti-neutrophil cytoplasmic antibodies (ANCAs) are a group of autoantibodies, mainly of the IgG type, against antigens in the cytoplasm of neutrophil granulocytes (the most common type of white blood cell) and monocytes.

Cytoplasm

cytoplasmiccytosolicintracytoplasmic
Anti-neutrophil cytoplasmic antibodies (ANCAs) are a group of autoantibodies, mainly of the IgG type, against antigens in the cytoplasm of neutrophil granulocytes (the most common type of white blood cell) and monocytes.

White blood cell

leukocyteleukocyteswhite blood cells
Anti-neutrophil cytoplasmic antibodies (ANCAs) are a group of autoantibodies, mainly of the IgG type, against antigens in the cytoplasm of neutrophil granulocytes (the most common type of white blood cell) and monocytes.

Blood test

blood testsbloodblood testing
They are detected as a blood test in a number of autoimmune disorders, but are particularly associated with systemic vasculitis, so called ANCA-associated vasculitides (AAV).

Autoimmune disease

autoimmune diseasesautoimmune disorderautoimmune
They are detected as a blood test in a number of autoimmune disorders, but are particularly associated with systemic vasculitis, so called ANCA-associated vasculitides (AAV).

Immunofluorescence

immunofluorescentindirect immunofluorescenceimmunofluorescence microscopy
Immunofluorescence (IF) on ethanol-fixed neutrophils is used to detect ANCA, although formalin-fixed neutrophils may be used to help differentiate ANCA patterns.

Cathepsin G

chymotrypsin-likeCTSG
p-ANCA without nuclear extension occurs with antibodies to BPI, cathepsin G, elastase, lactoferrin and lysozyme.

Elastase

neutrophil elastase
p-ANCA without nuclear extension occurs with antibodies to BPI, cathepsin G, elastase, lactoferrin and lysozyme.

Lactoferrin

lactotransferrinLTFRecombinant human lactoferrin
p-ANCA without nuclear extension occurs with antibodies to BPI, cathepsin G, elastase, lactoferrin and lysozyme.

Lysozyme

LYZmuramidaselysozymes
p-ANCA without nuclear extension occurs with antibodies to BPI, cathepsin G, elastase, lactoferrin and lysozyme.

HMGB1

HMG1amphoterinhigh-mobility group box 1 protein
Other less common antigens include HMG1 (p-ANCA pattern), HMG2 (p-ANCA pattern), alpha enolase (p and c-ANCA pattern), catalase (p and c-ANCA pattern), beta glucuronidase (p-ANCA pattern), azurocidin (p and c-ANCA pattern), actin (p and a-ANCA) and h-lamp-2 (c-ANCA).

HMGB2

HMG-B2HMG2
Other less common antigens include HMG1 (p-ANCA pattern), HMG2 (p-ANCA pattern), alpha enolase (p and c-ANCA pattern), catalase (p and c-ANCA pattern), beta glucuronidase (p-ANCA pattern), azurocidin (p and c-ANCA pattern), actin (p and a-ANCA) and h-lamp-2 (c-ANCA).

Alpha-enolase

ENO1alpha enolaseEnolase 1
Other less common antigens include HMG1 (p-ANCA pattern), HMG2 (p-ANCA pattern), alpha enolase (p and c-ANCA pattern), catalase (p and c-ANCA pattern), beta glucuronidase (p-ANCA pattern), azurocidin (p and c-ANCA pattern), actin (p and a-ANCA) and h-lamp-2 (c-ANCA).

Catalase

catalase testcatalase-negativeCAT
Other less common antigens include HMG1 (p-ANCA pattern), HMG2 (p-ANCA pattern), alpha enolase (p and c-ANCA pattern), catalase (p and c-ANCA pattern), beta glucuronidase (p-ANCA pattern), azurocidin (p and c-ANCA pattern), actin (p and a-ANCA) and h-lamp-2 (c-ANCA).