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Vasculitis Disease

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

Recognizing Vasculitis

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

  • Upper-airways disease is the most common presenting feature. Nasal disease presents with obstruction, nasal ulcers and septal perforation, serosanguinous discharge or epistaxis. Destruction of the nasal septum results in the typical saddle-nose deformity.
  • Renal disease from severe renal failure to proteinuria or haematuria with no impairment of renal function.
  • Pulmonary involvement including cough, haemoptysis and pleuritis are most common.
  • Cutaneous manifestations including ulcers, palpable purpura, papules and nodules.
  • Neurological involvement with peripheral neuropathy or mononeuritis multiplex is less common at presentation but can be detected (often subclinically) with time.
  • Ocular disease, with any compartment of the eye potentially being affected—keratitis, conjunctivitis, scleritis, episcleritis, uveitis, retro-orbital pseudotumour, retinal vessel occlusion and optic neuritis
  • Musculoskeletal symptoms are common, with most patients experiencing arthralgias and/or myalgias. A true synovitis can be seen in 25% of patients
  • The most frequent radiological features are pulmonary infiltrates, haemorrhage, and hilar lymphadenopathy.
  • Late-onset asthma
  • Peripheral blood and tissue eosinophilia
  • Mononeuritis multiplex
  • Cardiac disease

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

GPA which affects the nose, lungs and kidneys

MPA

MPA which primarily affects the kidneys

EGPA

EGPA which affects the lungs, kidneys, heart, and skin

Valuable Tools to Aid in Diagnosis

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

  • Chronic destructive upper airway disease
  • Pulmonary nodules
  • Renal and pulmonary inflammatory disease
  • Rapidly progressive glomerulonephritis
  • Skin veasculitis with systemic illness
  • Mononeuritis multiplex
  • Subglottic stenosis of the trachea
  • Retro orbital mass
testing can be quicker in diagnosis

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.
 

Take me to testing

References
  1. Bossuyt X, Cohen Tervaert J-W, Arimura Y, et al. Revised 2017 international consensus on testing of ANCAs in granulomatosis with polyangiitis and microscopic polyangiitis. Nature Rev Rheumatol. 2017;doi:10.1038/nrrheum.2017.140
  2. Csernok, E. & Moosig, F. Current and emerging techniques for ANCA detection in vasculitis. Nat. Rev. Rheumatol. 10, 494-501 (2014).
  3. Damoiseaux J, Csernock E, Rasmussen N. Detection of antineutrophil cytoplasmic antibodies (ANCAs): a multicentre European Vasculitis Study Group (EUVAS) evaluation of the value of indirect immunofluorescence (IIF) versus antigen-specific immunoassays. Ann Rheum Dis. 2016;76(4):647-653.
  4. Suresh E. Diagnostic approach to patients with suspected vasculitis. Postgrad Med J. 2006. 82(970):483-488.  
  5. Watts RA, Dharmapalaiah C. ANCA-associated vasculitis. Arthritis Research UK. Reports on Rheumatic Diseases: Topical reviews. 2012. http://www.arthritisresearchuk.org/health-professionals-and-students/reports/topical-reviews/topical-reviews-autumn-2012.aspx. Accessed November 2017.  
  6. Ntatsaki E, Carruthers D, Chakravarty K, et al. BSR and BHPR guideline for the management of adults with ANCA-associated vasculitis. Rheumatology (Oxford). 2014;53(12):2306-9.
  7. Roth AJ, Ooi JD, Hess JJ, et al. Epitope specificity determines pathogenicity and detectability in ANCA-associated vasculitis. J Clin Invest. 2013;123:1773-1783.
  8. Savige J, Gillis D, Benson E, et al. International Consensus Statement on Testing and Reporting of Antineutrophil Cytoplasmic Antibodies (ANCA). Am J Clin Pathol. 1999 Apr;111(4):507-13.