When viewing this topic in a different language, you may notice some differences in the way the content is structured, but it still reflects the latest evidence-based guidance.

Systemic vasculitis

Last reviewed: 30 Sep 2024
Last updated: 29 Nov 2023

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)
Full details

Risk factors

  • extremes of age
Full details

Diagnostic tests

1st tests to order

  • erythrocyte sedimentation rate (ESR)
  • C-reactive protein (CRP)
  • antineutrophil cytoplasmic autoantibodies (ANCA)
  • assessment for glomerulonephritis
  • biopsy of affected tissue
Full details

Tests to consider

  • conventional angiography or magnetic resonance angiography (MRA)
  • positron emission tomography (PET) scan
Full details

Treatment algorithm

INITIAL

suspected giant cell arteritis

ACUTE

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)

ONGOING

resistant to treatment at any stage of therapy

Contributors

Authors

Brendan Antiochos, MD

Assistant Professor of Medicine

Director, Vasculitis Center

Division of Rheumatology

Johns Hopkins

Baltimore

MD

Disclosures

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

Acknowledgements

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

Disclosures

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

Peer reviewers

Jason M. Springer, MD, MS

Associate Professor

Vanderbilt University Medical Center

Nashville

TN

Disclosures

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
  • padlock-lockedLog in or subscribe to access all of BMJ Best Practice

Use of this content is subject to our disclaimer