Autoimmune Vasculitis

VasculitisA comprehensive range of enzyme immunoassays and immunofluorescence assays is available from Binding Site for the investigation of vasculitis.

Vasculitis

Vasculitis encompasses a large and heterogeneous group of disorders that vary considerably in their clinical presentations. As a consequence these diseases can be difficult to diagnose. An attempt to classify the diverse forms of vasculitis resulted in the Chapel Hill International Consensus definitions, which used vessel size as the determinant of classification.

Anti-Neutrophil Cytoplasmic Antibodies (ANCA) - associated systemic vasculitides including Wegener's granulomatosis (WG), Microscopic polyangiitis (MPA) and Churg-Strauss syndrome (CSS) are commonly described as small vessel vasculitides. These diseases are characterised by inflammation and necrosis of blood vessels, and typically have focal necrotising lesions that affect various vessels and organs.

Prior to the use of immunosuppressive drugs and ANCA testing, these diseases were often fatal, with biopsy of a relevant organ (typically kidney, nasal mucosa or occasionally lung) being required in order to establish diagnosis. For succesful treatment, vasculitis must be recognised and treated early to prevent permanent scarring. ANCA are now widely used as diagnostic markers for vasculitis and have had a dramatic impact on clinical outcomes.

ANCA

ANCA are autoantibodies that react with neutrophil specific antigens. The major ANCA antigens in patients with primary systemic small vessel vasculitis are the enzymes myeloperoxidase (MPO) and proteinase-3 (PR3) which are present within neutrophil granules.

Clinical Significance

Anti-PR3 antibodies are the most frequent specificity in WG patients, with levels being raised in active disease. Consequently anti-PR3 detection is a useful monitor of disease activity in treated patients. Anti-MPO antibodies are more common in MPA and CSS patients and occur in 77-100% of ANCA-associated glomerulonephritis cases.

Proposed Testing Protocol

Description Wells Code
ANCA COMBI kit 10 x 5 FK018
ANCA ethanol kit

10 x 5

25 x 10

FK016

FK016.1

ANCA formalin kit 10 x 5 FK017
Ethanol-fixed neutrophil slides

10 x 5

25 x 10

FS016.1

FS016.2

Formalin-fixed neutrophil slides

10 x 5

25 x 10

FS017.1

FS017.2

Bindazyme

Description Pack Code
Anti MPO IgG EIA

96 test

10 x 96 test

MK031

MK031.10

Anti PR3 IgG EIA

96 test

10 x 96 test

MK032

MK032.10

ASCA IgG EIA 96 test MK053
ASCA IgA EIA 96 test MK054

 

 

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Kidney glomeruli image courtesy of Susumu Nishinaga/Science Photo Library