The systemic vasculitides are a group of uncommon conditions characterized by inflammation and necrosis of blood vessel walls. Some of these syndromes are also categorized by the presence of antineutrophil cytoplasmic antibodies (ANCA) – so called ANCA-associated vasculitides (Chapel Hill 2012).

  • Diagnosis
  • Algorithms
  • Monitoring
  • Background
  • Lab Tests
  • References
  • Related Topics

Indications for Testing

  • Multisystem disease presentation – including upper airway disease, renal disease, pulmonary disease, palpable purpura, urticaria, or mononeuritis multiplex

Laboratory Testing

  • Initial testing – helpful in excluding other diagnoses or identifying organ dysfunction
    • CBC –  evaluate for anemia, leukocytosis, thrombocytopenia
    • C-reactive protein (CRP)
    • Urinalysis – evaluate for presence of hematuria, proteinuria, or and/or red blood cell casts
    • Renal function tests (BUN/creatinine) – assess for renal involvement
    • Liver function tests – provide clues for hepatic involvement (most common in polyarteritis nodosa)
    • ANCA – most useful for differentiating ANCA(+) vasculitis from other vasculitis
      • Indirect immunofluorescence assay (IFA) – sensitive marker for ANCA-associated vasculitis
      • To confirm positive results, PR3 or MPO specific assays (ELISA, Western blot or multianalyte fluorescence detection [MAFD]) required (European League Against Rheumatism [EULAR], 2010 grade A recommendation)
      • Pattern of ANCA frequently helpful – pANCA vs. cANCA
      • Absence of a positive test result does not rule out vasculitis
      • ANCA(+) vasculitides – granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis
  • Other possible secondary testing – antinuclear antibody, rheumatoid arthritis, hepatitis B and C viruses


  • Determines size of artery involvement (EULAR, 2009 grade C recommendation; Chapel Hill, 2012)
  • In conjunction with clinical presentation, combination of ANCA, urinalysis
  • Usually identifies specific vasculitis (see the Vasculitis in Adults Testing Algorithm and Vasculitis in Children Testing Algorithm)

Imaging Studies

  • Chest X-ray – nonspecific pulmonary nodules, cavitation, consolidation or pleural effusion suggest pulmonary involvement
  • Angiogram of affected area – demonstrates aneurysms and vascular occlusion
    • Magnetic resonance angiography or computed tomography angiography may be preferred
  • Echocardiography
    • 40% detection rate for Kawasaki
    • Also used in Takayasu arteritis
  • Ultrasound – for giant cell arteritis diagnosis and monitoring
  • CT sinus – useful in granulomatosis with polyangiitis (GPA)

Other Testing

  • Nerve conduction testing if neurologic manifestations present

Differential Diagnosis

  • ANCA – if positive in initial evaluation
    • Titers may decrease after induction of remission and elevation may herald relapse
      • Rising titers do not reliably predict relapse
      • Titers cannot be used to guide treatment
    • Urinalysis should be performed every visit to monitor for renal involvement (EULAR, 2010)
    • CBC, inflammatory markers, renal and liver function testing should be performed every 1-3 months (EULAR, 2010 grade C recommendation)


  • Incidence – 100/1,000,000
  • Age – peak onset is 65-74 years; unusual in children
  • Sex – M>F (minimal)


  • Based on affected blood vessel size – small, medium, or large

Clinical Presentation

  • Nonspecific signs/symptoms early in disease – fever, arthralgias, fatigue, weight loss, myalgias
  • Multisystem involvement later in disease – dermatologic, ophthalmologic, renal, pulmonary, hepatic, gastrointestinal tract, vascular, central nervous system
  • Patients present with diverse organ involvement in most cases
Tests generally appear in the order most useful for common clinical situations. Click on number for test-specific information in the ARUP Laboratory Test Directory.

ANCA-Associated Vasculitis Profile (ANCA/MPO/PR-3) with Reflex to ANCA Titer 2006480
Method: Semi-Quantitative Indirect Fluorescent Antibody/Semi-Quantitative Multiplex Bead Assay


All interpretation of antibody patterns must be done in conjunction with clinical presentation

There may be overlap between diseases and antibodies detected

Cross-reaction may occur with cationic protein 57 (CAP 57), cathepsin G, elastase, lactoferrin, and other lysosomal proteins


Biopsy for histology

Urinalysis, Complete 0020350
Method: Reflectance Spectrophotometry/Microscopy

C-Reactive Protein 0050180
Method: Quantitative Immunoturbidimetry

CBC with Platelet Count and Automated Differential 0040003
Method: Automated Cell Count/Differential

Hepatic Function Panel 0020416
Method: Quantitative Enzymatic/Quantitative Spectrophotometry

Renal Function Panel 0020144
Method: Quantitative Chemiluminescent Immunoassay/Quantitative Enzyme-Linked Immunosorbent Assay


Choosing Wisely. An initiative of the ABIM Foundation. [Accessed: Nov 2017]

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Last Update: November 2017