The systemic vasculitides are sub-acute illnesses associated with signs and symptoms of chronic inflammation.
Vasculitis can affect virtually any organ system; many of these diseases have typical patterns of involvement that are recognisable by experienced clinicians.
The diagnostic value of a biopsy, performed early in the course of illness, cannot be overstated.
Drugs used to treat vasculitis depend on the severity of the clinical manifestations.
In its strictest sense, the term vasculitis denotes inflammation of a blood vessel, which is characterised by the presence of an inflammatory infiltrate and destruction of the vessel wall. Commonly, however, vasculitis refers to the systemic vasculitides, which are autoimmune disorders characterised by inflammation of blood vessels. The systemic vasculitides are a diverse group of disorders that demonstrate a wide range of organ involvement and clinical severity.
History and exam
Key diagnostic factors
- presence of risk 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)
- haematuria (small-vessel)
- palpable purpura (small-vessel)
- otorrhoea, ear pain, or muffled sensation in the ears (small-vessel)
- nasal symptoms (small-vessel)
- sinus pain (small-vessel)
- wheeze (small-vessel)
- haemoptysis (small-vessel)
- age >50 years
- white ancestry
1st investigations to order
- erythrocyte sedimentation rate (ESR)
- C-reactive protein (CRP)
- anti-neutrophil cytoplasmic auto-antibodies (ANCA)
- serum urea and creatinine
- biopsy of affected tissue
Investigations to consider
- conventional angiography or magnetic resonance angiography (MRA)
- positron emission tomography (PET) scan
suspected or confirmed giant cell arteritis
other than giant cell arteritis
resistant to treatment at any stage of therapy
Philip Seo, MD, MHS
Associate Professor of Medicine
Director, Fellowship Program
Division of Rheumatology
Johns Hopkins University School of Medicine
The Johns Hopkins Vasculitis Center
PS declares that he has no competing interests.
Megan Clowse, MD, MPH
Assistant Professor of Medicine
Division of Rheumatology and Immunology
MC declares that she has no competing interests.
Bridget Griffiths, MB ChB, MD, FRCP(UK)
Department of Rheumatology
Newcastle Upon Tyne
BG declares that she has no competing interests.
- Infective endocarditis
- Hypercoagulability syndromes
- Systemic lupus erythematosus (SLE)
- EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis
- BSR and BHPR guidelines for the management of adults with ANCA-associated vasculitis
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