Systemic vasculitis

Last reviewed: 21 Apr 2022
Last updated: 04 Jun 2019

Summary

Definition

History and exam

Key diagnostic factors

  • constitutional symptoms (e.g., malaise, fever, arthralgia, myalgia)
  • headache and scalp tenderness (giant cell arteritis)
  • visual changes (large-vessel)
  • upper extremity or jaw claudication (large-vessel)
  • asymmetric brachial pulses (large-vessel)
  • bruits (large-vessel)
  • abdominal pain (medium-vessel)
  • foot drop, wrist drop (medium-vessel)
  • cutaneous ulcers (medium-vessel)
  • hematuria (small-vessel)
  • palpable purpura (small-vessel)
  • otorrhea, ear pain, or muffled sensation in the ears (small-vessel)
  • nasal symptoms (small-vessel)
  • sinus pain (small-vessel)
  • wheeze (small-vessel)
  • hemoptysis (small-vessel)
More key diagnostic factors

Risk factors

  • age >50 years
  • white ancestry
More risk factors

Diagnostic investigations

1st investigations to order

  • erythrocyte sedimentation rate (ESR)
  • C-reactive protein (CRP)
  • antineutrophil cytoplasmic auto-antibodies (ANCA)
  • blood urea nitrogen and serum creatinine
  • urinalysis
  • biopsy of affected tissue
More 1st investigations to order

Investigations to consider

  • conventional angiography or magnetic resonance angiography (MRA)
  • positron emission tomography (PET) scan
More investigations to consider

Treatment algorithm

INITIAL

suspected or confirmed giant cell arteritis

ACUTE

other than giant cell arteritis

ONGOING

resistant to treatment at any stage of therapy

Contributors

Authors

Philip Seo, MD, MHS
Philip Seo

Associate Professor of Medicine

Director, Fellowship Program

Division of Rheumatology

Johns Hopkins University School of Medicine

Director

The Johns Hopkins Vasculitis Center

Baltimore

MD

Disclosures

PS declares that he has no competing interests.

Peer reviewers

Megan Clowse, MD, MPH

Assistant Professor of Medicine

Division of Rheumatology and Immunology

Duke University

Durham

NC

Disclosures

MC declares that she has no competing interests.

Bridget Griffiths, MB ChB, MD, FRCP(UK)

Consultant Rheumatologist

Department of Rheumatology

Freeman Hospital

Newcastle Upon Tyne

UK

Disclosures

BG declares that she has no competing interests.

  • Systemic vasculitis images
  • Differentials

    • Infective endocarditis
    • Hypercoagulability syndromes
    • Systemic lupus erythematosus (SLE)
    More Differentials
  • Guidelines

    • EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis
    • BSR and BHPR guidelines for the management of adults with ANCA-associated vasculitis
    More Guidelines
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