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The ANCA-associated vasculitides (AAV) are comprised of Granulomatosis with Polyangiitis (GPA, formerly Wegener's Granulomatosis), Microscopic Polyangiitis (MPA) and Eosinophilic Granulomatosis with Polyangiitis (EGPA, formerly Churg-Strauss Syndrome). They share the features of small vessel vasculitis but are otherwise a heterogeneous group with different preferences of organ involvement and frequency of ANCA positivity. In general, GPA and EGPA are characterized by granulomatous lesions (especially of the respiratory tract) and small to medium size vessel vasculitis in biopsy specimens, whereas MPA is a small to medium size vessel vasculitis without granuloma. PR3 and MPO ANCA are present in almost all patients with MPA and GPA in the active generalized stage of the disease; in EGPA, ANCA are present in less than 40% and their presence is associated with typical vasculitis manifestations such as glomerulonephritis.1
In GPA, ANCA are mainly directed against the neutrophil serine protease proteinase 3 (PR3), whereas, in MPA and EGPA, ANCA mainly target the neutrophil enzyme myeloperoxidase (MPO).2
A deeper clinical evaluation can reveal more about which ANCA-associated vasculitis could be at the root of your patient’s symptoms.3
Depending on the particular blood vessels involved, patients with vasculitis can present with a wide spectrum of nondescript symptoms. More specific signs, however, that may steer you to consider vasculitis are:4-6
Vasculitis is a condition that causes inflammation of vessel walls.4
Vasculitides are grouped based on the types of blood vessels or organs they affect, which can vary widely.
Anti-Neutrophil Cytoplasmic Antibodies (ANCAs) associated vasculitides are characterized by inflammation and lesions in the small blood vessels.7 ANCA-associated vasculitides include three conditions:5
GPA which affects the nose, lungs and kidneys
MPA which primarily affects the kidneys
EGPA which affects the lungs, kidneys, heart, and skin
Laboratory diagnostics, when combined with clinical evaluation can help you discover the ANCA-associated condition that is the cause of the symptoms.
Test for PR3 and MPO ANCA in patients with:8
If your patients are exhibiting one or more common ANCA-associated vasculitis symptoms, testing can help provide a quicker diagnosis, while ruling out other possible diseases. Generally, a biopsy confirms a vasculitis diagnosis.7
Discover how antibody testing can aid in the diagnosis of ANCA-associated vasculitis and why they are such a valuable tool for your practice.