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Vasculitides and fibromuscular dysplasia 

Vasculitides and fibromuscular dysplasia
Chapter:
Vasculitides and fibromuscular dysplasia
Author(s):

Catherine Hill

, Scott Graf

, and Robert Fitridge

DOI:
10.1093/med/9780199658220.003.0004
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date: 20 September 2019

Vasculitides are a group of related diseases that are characterized by histological evidence of inflammatory leucocytes in vessel wall with reactive damage to mural structures. Vasculitides are often serious and may be life threatening. Therefore, early diagnosis and treatment is imperative to ensure the best patient outcome. Although the distribution of affected organs may suggest a certain type of vasculitis, there is significant overlap between them. Early treatment, in most cases glucocorticoids and immunosuppressants, has improved outcomes. However, vasculitides can relapse, requiring further or maintenance therapy. Long-term complications may result from adverse effects of treatment or vascular injury due to scarring and stenosis of affected vessels. The latter may lead to ischaemia or vessel rupture that may not necessarily reflect active vasculitis. Early deaths are generally due to active vasculitis with late deaths, predominantly due to complications of therapy. This chapter will outline the pathogenesis, clinical features, and principles of management of vasculitides and fibromuscular dysplasia.

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