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Vasculitis: Overview, Diagnosis, and Treatment

About Vasculitis

The antineutrophilic cytoplasmic autoantibody (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. Protease proteinase 3 (PR3) and myeloperoxidase (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 percent and their presence is associated with typical vasculitis manifestations such as glomerulonephritis.1

In GPA, ANCA are mainly directed against the neutrophil serine PR3, whereas in MPA and EGPA, ANCA mainly target the neutrophil enzyme 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

Less than 40%

In eosinophilic granulomatosis with polyangiitis (EGPA), antineutrophilic cytoplasmic autoantibody (ANCA) are present in less than 40 percent of patients.1

Depending on the particular blood vessels involved, patients with vasculitis can present with a wide spectrum of nondescript symptoms.

More specific signs that may steer you to consider vasculitis include:3-5

  • 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.
  • Late-onset asthma.
  • Peripheral blood and tissue eosinophilia.
  • Cardiac disease.
  • 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.
  • The most frequent radiological features are pulmonary infiltrates, hemorrhage, and hilar lymphadenopathy.
  • Neurological involvement with peripheral neuropathy or mononeuritis multiplex is less common at presentation but can be detected (often subclinically) with time.
  • Musculoskeletal symptoms are common, with most patients experiencing arthralgias and/or myalgias. A true synovitis can be seen in 25 percent of patients.
  • Mononeuritis multiplex.

Prompt recognition of vasculitis either as a new presentation or a clinical relapse is key to optimizing management and preventing organ damage.6

ANCA-associated vasculitides

Vasculitis is a condition that causes inflammation of vessel walls.3 ANCA-associated vasculitides are characterized by inflammation and lesions in the small blood vessels.7 ANCA-associated vasculitides include three conditions:4

  • GPA: Affects the nose, lungs, and kidneys
  • MPA: Primarily affects the kidneys
  • EGPA: Affects the lungs, kidneys, heart, and skin

Vasculitis: Refining differential diagnosis through testing

Prompt recognition of vasculitis either as a new presentation or a clinical relapse is key to optimizing management and preventing organ damage.6

The good news is that prompt diagnosis and treatment with immunosuppressive therapy, such as cyclophosphamide, corticosteroids, and methotrexate, can help patients mitigate this organ damage.5

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 vasculitis with systemic illness
  • Mononeuritis multiplex
  • Subglottic stenosis of the trachea
  • Retro orbital mass

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

Not sure which allergy or autoimmune disease could be behind your patient’s symptoms? Use this interactive tool to take the next step in making your differential diagnosis. 

Testing Confidence

Testing Increases Diagnostic Confidence

Adding diagnostic testing to aid in a differential diagnosis has been shown to increase confidence in diagnosis to 90 percent.i,ii Conventionally, a diagnosis of allergic or autoimmune disease relies on the case history and a physical examination. However, adding diagnostic testing to aid in a differential diagnosis has been shown to increase confidence in diagnosis.i,ii Diagnostic testing can also help to improve the patient’s quality of life and productivity, reduce costs associated with absenteeism, and optimize use of medication, in addition to decreasing unscheduled healthcare visits.iii,iv 

i. Duran-Tauleria E, Vignati G, Guedan MJ, et al. The utility of specific immunoglobulin E measurements in primary care. Allergy. 2004;59 (Suppl78):35-41.
ii. NiggemannB, Nilsson M, Friedrichs F. Paediatric allergy diagnosis in primary care is improved by in vitro allergen specific IgE testing. Pediatr Allergy Immunol. 2008;19:325-331
iii. Welsh N, et al. The Benefits of Specific Immunoglobulin E Testing in the Primary Care Setting. J Am Pharm Assoc. 2006;46:627.
iv. Szeinbach SL, Williams B, Muntendam P, et al. Identification of allergic disease among users of antihistamines. J Manag Care Pharm. 2004; 10 (3): 234-238

Learn about autoimmune disease.

Learn more about testing.

Management and care of patients with vasculitis

Advances in the medical treatment of vasculitis have resulted in patients living longer, but these patients frequently suffer substantial limitations in work and productivity—and face loss of personal income—due to their vasculitis.9

Minimizing the damage associated with vasculitis, particularly chronic pain and cognitive and functional impairment, may help mitigate future work limitations.9

Repeated assessment of vasculitis is necessary for planning therapy and modifying treatment. Therapy is based on the pattern of vasculitis and on careful evaluation of the disease extent and activity. Therapies typically include glucocorticoids and immunosuppressive agents, and there is increasing experience in the use of more specific biological therapies.10

As there are currently no clinical practice guidelines covering all aspects of the diagnosis and treatment of vasculitis in the United States—most physicians use the Chapel Hill nomenclature, which is based on clinical and histopathological features:11

The American College of Rheumatology Guideline for the Treatment and Management of Vasculitis has an anticipated final publication in late 2019/early 2020.

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.
  9. Barra L et al. Impact of vasculitis on employment and income. Clin Exp Rheumatol. 2018;36(Suppl 111): 58–64.
  10. Miller A et al. An approach to the diagnosis and management of systemic vasculitis. Clin Exp Immunol. 2010 May; 160(2): 143–160.
  11. Guillevin L, Dörner T. Vasculitis: mechanisms involved and clinical manifestations. Arthritis Res Ther. 2007;9 Suppl 2:S9.