The systemic vasculitides are subacute 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 recognizable 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 characterized 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 characterized 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
- 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)
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.
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