Systemic vasculitis

Last reviewed: 2 Sep 2023
Last updated: 26 Jul 2023



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

  • extremes of age
More risk factors

Diagnostic investigations

1st investigations to order

  • erythrocyte sedimentation rate (ESR)
  • C-reactive protein (CRP)
  • antineutrophil cytoplasmic autoantibodies (ANCA)
  • assessment for glomerulonephritis
  • 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


suspected giant cell arteritis


life-threatening or organ-threatening granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA)

non-life-threatening, non-organ-threatening granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA)


resistant to treatment at any stage of therapy



Brendan Antiochos, MD

Assistant Professor of Medicine

Director, Vasculitis Center

Division of Rheumatology

Johns Hopkins




BA has received compensation from Paradigm Medical Communications for CME activities regarding EGPA and Giant Cell Arteritis.


Dr Brendan Antiochos would like to gratefully acknowledge Dr Phillip Seo, the previous contributor for this topic.


PS is an author of some references cited in this topic.

Peer reviewers

Jason M. Springer, MD, MS

Associate Professor

Vanderbilt University Medical Center




JMS has served as a consultant and on an advisory board for ChemoCentryx, the manufacturer of avacopan.

  • Systemic vasculitis images
  • Differentials

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

    • American College of Rheumatology guideline for vaccinations in patients with rheumatic and musculoskeletal diseases
    • EULAR recommendations for the management of ANCA-associated vasculitis
    More Guidelines
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