Systemic Vasculitis

Systemic or necrotizing vasculitides are a group of rare diseases characterized by inflammation of diverse blood vessel walls. Diseases are categorized by blood vessel size, namely small, medium, or large vessel vasculitis. Some vasculitides are associated with the presence of antineutrophil cytoplasmic antibodies (ANCA), or so-called ANCA-associated vasculitides. Vasculitis can occur in association with other conditions, including infections, drug reactions, inflammatory bowel disease, and malignancy.

Tabs Content

Clinical Overview

Diagnosis

Indications for Testing

General presentation
- Fever
- Joint pain and inflammation
- Malaise, weight loss
Large vessel vasculitides
- Aortic dilation
- Asymmetric or absent pulses
- Bruits
- Pain due to claudication
- Stroke-like symptoms
Medium vessel vasculitides
- Mononeuritis multiplex
- Skin signs (eg, livedo reticularis, nodules)
- Digital necrosis
- Microaneurysms
- Abdominal pain
- Gastrointestinal ulcers
Small vessel vasculitides
- Alveolar hemorrhage
- Glomerulonephritis
- Skin signs (eg, purpura, splinter hemorrhages)
- Uveitis
- Episcleritis
- Purpura
- Urticaria

Laboratory Testing

Useful testing for the evaluation of vasculitis
- Antineutrophil cytoplasmic antibody (ANCA) – most useful for differentiating ANCA(+) vasculitis from other vasculitis
  - Indirect fluorescent antibody (IFA) test – preferred screening method for ANCA-associated vasculitis with 2 main patterns: C-ANCA and P-ANCA
  - Proteinase 3 (PR3) or myeloperoxidase (MPO) specific assays (by enzyme-linked immunosorbent assay [ELISA], Western blot, or multianalyte fluorescence detection [MAFD]) can confirm positive C-ANCA or P-ANCA result
    - C-ANCA is associated with PR3 antibodies
    - P-ANCA is associated with MPO antibodies
  - Absence of a positive test result does not rule out vasculitis
- C-reactive protein (CRP)
  - Often elevated in active disease; nonspecific marker suggestive of systemic inflammation
  - If CRP not available, erythrocyte sedimentation rate (ESR) may be used
- CBC – may demonstrate
  - Normochromic normocytic anemia – chronic inflammation
  - Eosinophilia – eosinophilic granulomatosis with polyangiitis
  - Leukocytosis – inflammation
  - Reactive thrombocytosis – inflammation
- Peripheral smear
- Renal function tests – evaluate type and extent of kidney damage
  - Urinalysis – evaluate for hematuria, proteinuria, red blood cell casts, nitrates, and leukocytes
  - Serum kidney function tests (blood urea nitrogen [BUN]/creatinine)
  - Spot urine albumin/creatinine
  - Estimated glomerular filtration rate
Evaluation for other vasculitis associations
- Liver function tests
  - Evaluate hepatic involvement – most common in polyarteritis nodosa
- Immunoglobulin levels/complement levels and functional assay/cryoglobulins
- Antiglomerular basement membrane (anti-GBM) testing – detect GBM antibodies in suspected or established anti-GBM disease (Goodpasture Syndrome)
  - By IFA and/or multiplex bead assay
- Coagulation and synthetic function testing
  - Prothrombin time
- Infectious vasculitis testing
  - Rickettsia rickettsii
  - Hepatitis C and B – cryoglobulinemic vasculitis
  - Treponema pallidum
  - HIV
  - Varicella zoster virus (VZV)
- Autoimmune rheumatic systemic disease testing – lupus, rheumatoid arthritis, antiphospholipid antibody syndrome (APS)
  - Antinuclear antibody (ANA)
  - Double-stranded DNA (dsDNA)
  - Extractable nuclear antigen (ENA) antibodies panel
  - Angiotensin converting enzyme (ACE)
  - Rheumatoid factor (RF)
  - Anticardiolipin antibodies, lupus anticoagulant

Histology

Biopsy required to confirm or rule out diagnosis
- ANCA has a sensitivity of ~85% in active Wegener granulomatosis (WG)
Assess damage and characterize disease
Size of blood vessels involved (small, medium, large)
IgA deposition for IgA vasculitis
Evaluation for eosinophils in eosinophilic granulomatosis with polyangiitis

Imaging Studies

Chest X-ray – nonspecific pulmonary nodules, cavitation, consolidation, or pleural effusion suggest pulmonary involvement
Angiogram of affected area – cardiac, central nervous system (CNS)
- May demonstrate aneurysms and vascular occlusion
- Magnetic resonance angiography or computed tomography angiography may be preferred
Echocardiography
- Use to evaluate extent of disease in large and medium vessel vasculitis
- 40% detection rate for Kawasaki
- Also used in Takayasu arteritis
Ultrasound – for giant cell arteritis diagnosis and monitoring
Computed tomography (CT) scan
- Sinus – useful in granulomatosis with polyangiitis (GPA)
- Chest – useful to detect pulmonary involvement

Other Testing

Nerve conduction testing if neurologic manifestations present
Combination of patient’s presentation, history, laboratory, imaging, and clinical findings are critical to optimal classification of systemic vasculitis
- See Vasculitis in Adults Testing Algorithm and Vasculitis in Children Testing Algorithm

Differential Diagnosis

Large vessel
- Mycotic aneurysms
- Atherosclerosis
- Congenital – aortic coarctation
- Hereditary disorders
  - Marfan syndrome
  - Ehlers-Danlos syndrome (type IV)
  - Loeys-Dietz syndrome
- Fibromuscular dysplasia
- Postradiation syndrome
- Chronic aortic aneurysm
- Chronic infection
  - Mycobacterium tuberculosis
  - Treponema pallidum
Medium vessel/small vessel
- Vasculitis
  - Cogan syndrome
  - Behçet syndrome
- Autoimmune disease
- Neoplasm
  - Lymphoma
  - Leukemia (acute lymphocytic leukemia [ALL], acute myeloid leukemia [AML])
  - Plasma cell dyscrasias
  - Paraneoplastic syndrome
- Infection
  - HIV
  - Hepatitis C virus
  - Hepatitis B virus
  - Herpes simplex virus
  - Endocarditis
- Glomerulonephritis – acute, infectious, other etiologies
- Sickle cell disease
- Hypercoagulable states
  - Antiphospholipid syndrome (APS)
  - Thrombotic thrombocytopenic purpura (TTP)
- Mycotic aneurysms
- Hereditary disorders
  - Ehlers-Danlos syndrome
  - Neurofibromatosis
  - Grange syndrome
- Fibromuscular dysplasia
- Drugs
  - Cocaine
  - Amphetamines
  - Hypersensitivity reactions
Central nervous system vasculitis
- Sarcoidosis
- Infection
  - Bacterial
  - Fungal – yeasts and molds
  - Viral – progressive multifocal leukoencephalopathy
  - Parasitic
- Malignancy
  - Glioma
  - Angiocentric lymphoma
- Hypercoagulable states
  - TTP
  - APS
- Stroke-like syndromes
  - Sickle cell disease
  - Migraine headaches
  - Fabry disease
- Cerebral hemorrhage
- Embolic disease
  - Myxoma
  - Endocarditis

Monitoring

Antineutrophil cytoplasmic antibody (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 – perform every visit to monitor for infection or renal involvement (European League Against Rheumatism [EULAR]/European Renal Association-European Dialysis and Transplant Association [ERA-EDTA], 2016)
Inflammatory markers and renal function testing – perform every 1-3 months (EULAR/ERA-EDTA, 2016)
Malignancy evaluation – higher risk for cancer in Wegener’s granulomatosis and microscopic polyangiitis, specifically for bladder, skin, and hematologic malignancies (Kermani, 2011)
- Consider careful monitoring

Background

Epidemiology

Incidence – 100/million
Age
- Peak onset is 65-74 years
- Unusual in children
Sex – M>F; minimal

Nomenclature

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

Chapel Hill Consensus Conference Nomenclature of Systemic Vasculitis (Revised 2012)

Revised Systemic Vasculitis Nomenclature
Large vessel vasculitis	Takayasu arteritis Giant cell arteritis
Medium vessel vasculitis	Polyarteritis nodosa Kawasaki disease
Small vessel vasculitis	ANCA-associated SVV Microscopic polyangiitis Granulomatosis with polyangiitis Eosinophilic granulomatosis with polyangiitis Immune complex SVV Antiglomerular membrane disease Cryoglobulinemia vasculitis IgA vasculitis (Henoch-Schönlein) Hypocomplementemic urticarial vasculitis (anti-C1q vasculitis)
Variable vessel vasculitis	Behçet’s syndrome vasculitis Cogan’s syndrome vasculitis
Single organ vasculitis	Cutaneous leukocytoclastic angiitis Cutaneous arteritis Primary CNS vasculitis Isolated aortitis
Vasculitis associated with systemic disease	Lupus vasculitis Rheumatoid vasculitis Sarcoid vasculitis
Vasculitis associated with probable etiology	Hepatitis C virus-associated cryoglobulinemic vasculitis Hepatitis B virus-associated vasculitis Syphilis-associated aortitis Drug-associated ANCA-associated vasculitis Cancer-associated vasculitis
ANCA, antineutrophil cytoplasmic antibodies; CNS, central nervous system; SVV, small vessel vasculitis Source: International Chapel Hill Consensus 2012

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

Pediatrics

Vasculitis in pediatrics is categorized by the predominant size of the blood vessels affected; most vasculitides can affect a range of sizes (overlap) (Foster, 2012). The most common pediatric vasculitides are immunoglobulin A (IgA) vasculitis and Kawasaki disease; eosinophilic granulomatosis with polyangiitis is extremely rare in children, and giant cell arteritis is not seen. Laboratory testing and other evaluation with confirmation by biopsy is similar to adult testing. See Diagnosis for adult testing recommendations.

Epidemiology

IgA vasculitis (formerly Henoch-Schönlein purpura) (Piram, 2013)
- Incidence – 3-27/100,000 children and infants ≤18 years
  - 2-33 times more common in children than adults
- Age – predilection for children 3-12 years
- M>F
- Autumn-winter predominance
Kawasaki disease (Holman, 2010)
- Incidence – 20/100,000 U.S. children <5 years
  - Highest incidence in Japan
- Age – peak incidence <1 year
- M>F
- Winter-spring predominance

ARUP Lab Tests

Preferred reflex panel for the workup of suspected vasculitis

For patients with a history of vasculitis, refer to the antineutrophil cytoplasmic antibodies (ANCA) reflex panel that includes a titer, and myeloperoxidase (MPO) and proteinase 3 (PR3) antibodies

Individual antibody tests are available; refer to PR3 antibody or MPO antibody tests

Initial test in conjunction with autoimmune liver disease with reflex to smooth muscle antibody for evaluation of autoimmune liver disease

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

There may be overlap between diseases and antibodies detected

No single test shows absolute specificity

ANCA-Associated Vasculitis Profile (ANCA/MPO/PR3) with Reflex to ANCA Titer 2006480

Method: Semi-Quantitative Indirect Fluorescent Antibody/Semi-Quantitative Multiplex Bead Assay

Panel includes ANCA, IgG; MPO antibodies, IgG; and serine PR3, IgG

Reflex pattern: if the ANCA screen detects antibodies at a 1:20 dilution or greater, then a titer to end point will be added

May help in ruling out infectious process

CBC with Platelet Count and Automated Differential 0040003

Method: Automated Cell Count/Differential

Evaluate for kidney dysfunction in patients with known risk factors (eg, hypertension, diabetes, obesity, family history of kidney disease)

Monitor treatment of vasculitis

Renal Function Panel 0020144

Method: Quantitative Chemiluminescent Immunoassay/Quantitative Enzyme-Linked Immunosorbent Assay

Panel includes albumin, calcium, carbon dioxide, creatinine, chloride, glucose, phosphorous, potassium, sodium, blood urea nitrogen (BUN), and a calculated anion gap value

Screen for various metabolic and kidney disorders

Urinalysis, Complete 0020350

Method: Reflectance Spectrophotometry/Microscopy

Initial screening for hepatobiliary inflammation

Monitor treatment of vasculitis

Hepatic Function Panel 0020416

Method: Quantitative Enzymatic/Quantitative Spectrophotometry

Panel includes bilirubin, direct; bilirubin, total (serum or plasma); alkaline phosphatase; aspartate aminotransferase; alanine aminotransferase; protein, total (serum or plasma); albumin (serum or plasma)

Related Tests

Nonspecific test used to detect inflammation associated with infections, cancers, and autoimmune diseases

Sedimentation Rate, Westergren (ESR) 0040325

Method: Visual Identification

Monitor previously established myeloperoxidase (MPO)/proteinase 3 (PR3) antibodies or confirm an indirect fluorescent antibody (IFA) antineutrophil cytoplasmic antibodies (ANCA) positive test result

Test does not include ANCA testing by IFA

For ANCA testing, refer to the available panel tests

For the workup of suspected vasculitis, refer to ANCA-associated vasculitis profile (ANCA/MPO/PR3) with reflex to ANCA titer

For patients with history of vasculitis, refer to ANCA reflex to titer and MPO/PR3 antibodies

MPO/PR3 (ANCA) Antibodies 0050707

Method: Semi-Quantitative Multiplex Bead Assay

When used in conjunction with other autoantibody tests (ANCA, PR3), may assist in evaluating suspected immune-mediated vasculitis, especially microscopic polyangiitis (MPA)

May be useful when monitoring MPA disease and/or treatment response

Panel tests are available for the workup of suspected vasculitis; refer to ANCA-associated vasculitis profile (ANCA/MPO/PR3) with reflex to ANCA titer, and for patients with a history of vasculitis, refer to the ANCA reflex to titer and MPO/PR3 antibodies

Myeloperoxidase (MPO) Antibody 0050526

Method: Semi-Quantitative Multiplex Bead Assay

When used in conjunction with other autoantibody tests (ANCA, MPO), may assist in differentiating suspected Wegener granulomatosis (WG) from other vasculitides

May be useful when monitoring patients with PR3 antibodies

Serine Proteinase 3 (PR3) Antibody 0050527

Method: Semi-Quantitative Multiplex Bead Assay

First-line screening test for ANCA-associated vasculitis

For the workup of suspected vasculitis, refer to ANCA-associated vasculitis profile (ANCA/MPO/PR3) with reflex to ANCA titer

Recommended in the differential evaluation of autoimmune diseases with renal involvement (systemic lupus erythematosus [SLE] and antiglomerular basement membrane [anti-GBM] disease)

If ANCA screen detects antibodies ≥1:20 dilution, then titer to end point will be added

Anti-Neutrophil Cytoplasmic Antibody, IgG 0050811

Method: Semi-Quantitative Indirect Fluorescent Antibody

Preferred reflex panel for managing patients with a known diagnosis of vasculitis

May be assistive in evaluating suspected vasculitis

For the workup of suspected vasculitis, the preferred panel is ANCA-associated vasculitis profile (ANCA/MPO/PR3) with reflex to ANCA titer

Anti-Neutrophil Cytoplasmic Antibody with Reflex to Titer and MPO/PR3 Antibodies 2002068

Method: Semi-Quantitative Indirect Fluorescent Antibody/Semi-Quantitative Multiplex Bead Assay

Panel includes ANCA, IgG; MPO antibody; and PR3 antibody

Reflex pattern: if screen is positive, then titer and MPO/PR3 antibodies will be added to aid in antibody determination

Aid in evaluation of patients with vasculitis, macroglobulinemia, or multiple myeloma in whom symptoms occur with exposure to cold

Cryoglobulin, Qualitative with Reflex to IFE Typing and Quantitative IgA, IgG, and IgM 2002403

Method: Qualitative Cold Precipitation/Qualitative Immunofixation Electrophoresis/Quantitative Nephelometry

Reflex pattern: if qualitative is positive, immunofixation electrophoresis typing and quantitative IgA, IgG, and IgM will be added

Detect GBM antibodies in suspected or established anti-GBM disease

Glomerular Basement Membrane Antibody, IgG by Multiplex Bead Assay and IFA 2008403

Method: Semi-Quantitative Multiplex Bead Assay/Qualitative Indirect Fluorescent Antibody

Panel includes GBM antibody, IgG by multiplex bead assay and IFA

Aid in evaluation of patients with vasculitis, macroglobulinemia, or multiple myeloma in whom symptoms occur with exposure to cold

Cryoglobulin, Qualitative 0050185

Method: Qualitative Cold Precipitation

Initial test for suspected bleeding disorder

Prothrombin Time 0030215

Method: Electromagnetic Mechanical Clot Detection

Preferred test for acute or convalescent phase of disease

Acute and convalescent titers often necessary

Rickettsia rickettsii (Rocky Mountain Spotted Fever) Antibodies, IgG & IgM by IFA 0050371

Method: Semi-Quantitative Indirect Fluorescent Antibody

Order to evaluate viral etiology in patients with acute hepatitis

Not recommended for screening asymptomatic patients

Hepatitis Panel, Acute with Reflex to HBsAg Confirmation 0020457

Method: Qualitative Chemiluminescent Immunoassay

Panel includes hepatitis A virus (HAV) IgM, hepatitis B virus (HBV) core antibody IgM, HBV surface antigen, hepatitis C virus (HCV) antibody

2014 CDC Recommended Algorithm for Laboratory Diagnosis of HIV infection

This 4th generation test screens for HIV-1 p24 antigen and antibodies to HIV-1 (groups M and O) and HIV-2

Repeatedly reactive HIV-1/2 antigen/antibody screening results are confirmed with an HIV-1/HIV-2 antibody differentiation test

Negative or indeterminate results for HIV-1/2 antibody differentiation are confirmed with a quantitative NAAT test

Human Immunodeficiency Virus (HIV) Combo Antigen/Antibody (HIV-1/O/2) by CIA, Reflexive Panel 2012674

Method: Qualitative Chemiluminescent Immunoassay/Qualitative Immunoassay/Quantitative Transcription-Mediated Amplification

CDC recommended test for the screening and diagnosis of syphilis

For rapid plasma reagin (RPR) test that follows reverse algorithm, refer to RPR test with reflex to RPR titer or Treponema pallidum antibody

Rapid Plasma Reagin (RPR) with Reflex to Titer and TP-PA Confirmation 0050478

Method: Semi-Quantitative Charcoal Agglutination/Semi-Quantitative Particle Agglutination

May aid in diagnosing acute infections and detecting past exposure and/or vaccination

Varicella-Zoster Virus Antibodies, IgG and IgM 0050162

Method: Semi-Quantitative Enzyme-Linked Immunosorbent Assay /Semi-Quantitative Chemiluminescent Immunoassay

Aid in evaluation of patients with vasculitis, macroglobulinemia, or multiple myeloma in whom symptoms occur with exposure to cold

Antinuclear Antibody (ANA) with HEp-2 Substrate, IgG by IFA 3000082

Method: Semi-Quantitative Indirect Fluorescent Antibody

Preferred antinuclear antibodies (ANA) screening test for connective tissue disease

Cryoglobulin, Qualitative 0050185

Method: Qualitative Cold Precipitation

Secondary screening for systemic lupus erythematosus (SLE) based on ANA results

Double-Stranded DNA (dsDNA) Antibody, IgG by ELISA with Reflex to dsDNA Antibody, IgG by IFA 0050215

Method: Qualitative Enzyme-Linked Immunosorbent Assay/Semi-Quantitative Indirect Fluorescent Antibody

Recommended for the differential diagnosis of SLE and Sjögren syndrome

Extractable Nuclear Antigen Antibodies (Smith/RNP, Smith, SSA 52, SSA 60, and SSB) 0050652

Method: Semi-Quantitative Multiplex Bead Assay

Preferred panel for the workup of suspected rheumatoid arthritis or undifferentiated inflammatory arthritides

Rheumatoid Arthritis Panel 2003277

Method: Semi-Quantitative Enzyme-Linked Immunosorbent Assay/Immunoturbidimetry

Acceptable initial test when antiphospholipid syndrome (APS) is strongly suspected

Beta-2 Glycoprotein 1 Antibodies, IgG and IgM 0050321

Method: Semi-Quantitative Enzyme-Linked Immunosorbent Assay

Acceptable initial test when APS is strongly suspected

Cardiolipin Antibodies, IgG and IgM 0099344

Method: Semi-Quantitative Enzyme-Linked Immunosorbent Assay

Blood Smear with Interpretation 3001947

Method: Cytochemical Stain

Angiotensin Converting Enzyme, Serum 0080001

Method: Quantitative Enzymatic

Medical Experts

Tebo, Anne E., PhD, D(ABMLI), Medical Director, Immunology at ARUP Laboratories; Professor of Clinical Pathology, University of Utah

References

Guidelines

Ozen S, Pistorio A, Iusan SM, Bakkaloglu A, Herlin T, Brik R, Buoncompagni A, Lazar C, Bilge I, Uziel Y, Rigante D, Cantarini L, Hilario MO, Silva CA, Alegria M, Norambuena X, Belot A, Berkun Y, Estrella AI, Olivieri AN, Alpigiani MG, Rumba I, Sztajnbok F, Tambic-Bukovac L, Breda L, Al-Mayouf S, Mihaylova D, Chasnyk V, Sengler C, Klein-Gitelman M, Djeddi D, Nuno L, Pruunsild C, Brunner J, Kondi A, Pagava K, Pederzoli S, Martini A, Ruperto N, Paediatric Rheumatology International Trials Organisation (PRINTO). EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria. Ann Rheum Dis. 2010; 69(5): 798-806. PubMed
Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, Flores-Suarez LF, Gross WL, Guillevin L, Hagen EC, Hoffman GS, Jayne DR, Kallenberg CG, Lamprecht P, Langford CA, Luqmani RA, Mahr AD, Matteson EL, Merkel PA, Ozen S, Pusey CD, Rasmussen N, Rees AJ, Scott DG, Specks U, Stone JH, Takahashi K, Watts RA. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013; 65(1): 1-11. PubMed
Yates M, Watts RA, Bajema IM, Cid MC, Crestani B, Hauser T, Hellmich B, Holle JU, Laudien M, Little MA, Luqmani RA, Mahr A, Merkel PA, Mills J, Mooney J, Segelmark M, Tesar V, Westman K, Vaglio A, Yalçındağ N, Jayne DR, Mukhtyar C. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis. 2016; 75(9): 1583-94. PubMed

General References

Berden A, Göçeroglu A, Jayne D, Luqmani R, Rasmussen N, Bruijn JA, Bajema I. Diagnosis and management of ANCA associated vasculitis. BMJ. 2012; 344: e26. PubMed

Bossuyt X, Rasmussen N, van Paassen P, Hellmich B, Baslund B, Vermeersch P, Blockmans D, Tervaert JC, Csernok E, Damoiseaux J. A multicentre study to improve clinical interpretation of proteinase-3 and myeloperoxidase anti-neutrophil cytoplasmic antibodies. Rheumatology (Oxford). 2017; 56(9): 1533-1541. PubMed

Buggiani G, Krysenka A, Grazzini M, Vašků V, Hercogová J, Lotti T. Paraneoplastic vasculitis and paraneoplastic vascular syndromes. Dermatol Ther. 2010; 23(6): 597-605. PubMed

Caspary L. Vasculitides of large vessels. Vasa. 2011; 40(2): 89-98. PubMed

Chung SA, Seo P. Microscopic polyangiitis. Rheum Dis Clin North Am. 2010; 36(3): 545-58. PubMed

Damoiseaux J, Csernok E, Rasmussen N, Moosig F, van Paassen P, Baslund B, Vermeersch P, Blockmans D, Tervaert JC, Bossuyt X. 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. 2017; 76(4): 647-653. PubMed

de Souza AW, de Carvalho JF. Diagnostic and classification criteria of Takayasu arteritis. J Autoimmun. 2014; 48-49: 79-83. PubMed

Foster H, Brogan P. Paediatric Rheumatology. Foster H, Brogan PA, eds. Oxford Handbook of Paediatric Rheumatology. Oxford Specialist Handbooks in Paediatrics, PP. 168-222. Oxford, UK: Oxford University Press, 2012.

Harder N. Temporal arteritis: an approach to suspected vasculitides. Prim Care. 2010; 37(4): 757-66, vi-ii. PubMed

Harnden A, Tulloh R, Burgner D. Kawasaki disease. BMJ. 2014; 349: g5336. PubMed

Hellmark T, Segelmark M. Diagnosis and classification of Goodpasture's disease (anti-GBM). J Autoimmun. 2014; 48-49: 108-12. PubMed

Hernández-Rodríguez J, Alba MA, Prieto-González S, Cid MC. Diagnosis and classification of polyarteritis nodosa. J Autoimmun. 2014; 48-49: 84-9. PubMed

Holman RC, Belay ED, Christensen KY, Folkema AM, Steiner CA, Schonberger LB. Hospitalizations for Kawasaki syndrome among children in the United States, 1997-2007. Pediatr Infect Dis J. 2010; 29(6): 483-8. PubMed

Kallenberg CG. The diagnosis and classification of microscopic polyangiitis. J Autoimmun. 2014; 48-49: 90-3. PubMed

Kermani TA, Warrington KJ, Amin S. Malignancy risk in vasculitis. Ther Adv Musculoskelet Dis. 2011; 3(1): 55-63. PubMed

Kessel A, Vadasz Z, Toubi E. Cogan syndrome--pathogenesis, clinical variants and treatment approaches. Autoimmun Rev. 2014; 13(4-5): 351-4. PubMed

Lutalo PM, D'Cruz DP. Diagnosis and classification of granulomatosis with polyangiitis (aka Wegener's granulomatosis). J Autoimmun. 2014; 48-49: 94-8. PubMed

Mouthon L, Dunogue B, Guillevin L. Diagnosis and classification of eosinophilic granulomatosis with polyangiitis (formerly named Churg-Strauss syndrome). J Autoimmun. 2014; 48-49: 99-103. PubMed

Piram M, Mahr A. Epidemiology of immunoglobulin A vasculitis (Henoch-Schönlein): current state of knowledge. Curr Opin Rheumatol. 2013; 25(2): 171-8. PubMed

Saguil A, Fargo M, Grogan S. Diagnosis and management of Kawasaki disease. Am Fam Physician. 2015; 91(6): 365-71. PubMed

Saulsbury FT. Henoch-Schönlein purpura. Curr Opin Rheumatol. 2010; 22(5): 598-602. PubMed

Sharma P, Sharma S, Baltaro R, Hurley J. Systemic vasculitis. Am Fam Physician. 2011; 83(5): 556-65. PubMed

Sinico RA, Radice A. Antineutrophil cytoplasmic antibodies (ANCA) testing: detection methods and clinical application. Clin Exp Rheumatol. 2014; 32(3 Suppl 82): S112-7. PubMed

Sánchez-Manubens J, Bou R, Anton J. Diagnosis and classification of Kawasaki disease. J Autoimmun. 2014; 48-49: 113-7. PubMed

Ting TV. Diagnosis and management of cutaneous vasculitis in children. Pediatr Clin North Am. 2014; 61(2): 321-46. PubMed

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Testing Algorithms

Vasculitis in Adults Testing Algorithm

Read more about Vasculitis in Adults Testing Algorithm

Vasculitis in Children Testing Algorithm

Read more about Vasculitis in Children Testing Algorithm

Content Review:

October 2017

Last Update: November 2019

Systemic Vasculitis

Diagnosis

Indications for Testing

Laboratory Testing

Histology

Imaging Studies

Other Testing

Differential Diagnosis

Monitoring

Background

Epidemiology

Nomenclature

Clinical Presentation

Pediatrics

Epidemiology

ARUP Lab Tests

Medical Experts

References

Guidelines

General References

Vasculitis in Adults Testing Algorithm

Vasculitis in Children Testing Algorithm

ARUP Laboratories

ARUP Websites

ARUP Consult


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Systemic Vasculitis

Diagnosis

Indications for Testing

Laboratory Testing

Histology

Imaging Studies

Other Testing

Differential Diagnosis

Monitoring

Background

Epidemiology

Nomenclature

Clinical Presentation

Pediatrics

Epidemiology

ARUP Lab Tests

Medical Experts

References

Guidelines

General References

Vasculitis in Adults Testing Algorithm

Vasculitis in Children Testing Algorithm

ARUP Laboratories

ARUP Websites

ARUP Consult