What is Vasculitis?

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Vasculitis is a group of disorders characterised by inflammation of different types (arteries, veins or capillaries) and sizes of blood vessels. It can affect any organ or system in the body, which is why some types of vasculitis are known as systemic disorders. The inflammation can damage the wall of the blood vessel and obstruct (causing ischaemia or a lack of blood supply to the affected organ) or rupture the vessel (haemorrhage).

Classification of the types of Vasculitis

The different types of systemic vasculitis are categorised according to the size of the affected blood vessel:

Brain with secondary haemorrhages due to cerebral vascular malformations

Large-vessel vasculitis. Giant cell arteritis (GCA) ,Takayasu’s arteritis.

Kidneys and urinary system

Medium-vessel vasculitis. Polyarteritis nodosa, Kawasaki’s disease.

Man with skin blemishes

Small-vessel vasculitis. Granulomatosis with polyangiitis (formerly, Wegener’s granulomatosis); microscopic polyangiitis; eosinophilic granulomatosis with polyangiitis (formerly, Churg–Strauss syndrome). They are also known as part of a group called ANCA-associated vasculitis (antineutrophil cytoplasmic antibodies), immunoglobulin A-associated vasculitis, (formerly, Henoch–Schönlein purpura), cryoglobulinaemic vasculitis and hypocomplementaemic vasculitis.

New categories of vasculitis have recently been added:

Irritated red eye

Variable-vessel vasculitis. Behçet’s disease, Cogan’s syndrome.

Virus de la hepatitis C

Vasculitis secondary to a causative agent. Vasculitis secondary to hepatitis B virus (HBV) or other viruses and cryoglobulinaemic vasculitis secondary to hepatitis C virus (HCV).

Woman with spots on her face typical of Lupus or cutaneous erythematosus.

Vasculitis associated with a systemic disease. Such as systemic lupus erythematosus, rheumatoid arthritis or sarcoidosis.

Brain with secondary haemorrhages due to cerebral vascular malformations

Single-organ vasculitis. Isolated central nervous system vasculitis, cutaneous arteritis, isolated cutaneous leucocytoclastic vasculitis, isolated aortitis, isolated focal vasculitis.

How many people are affected by Vasculitis?

All types of vasculitis are classified as rare diseases, given they affect less than 5 in every 10,000 people. In Spain and the Western society, the most common form of vasculitis in adults is giant cell arteritis, with an annual incidence of 2–11 patients per 100,000 inhabitants aged over 50. The most frequent type affecting the paediatric population is IgA-associated vasculitis, with around 10 new cases every year per 100,000 children aged under 15.

Substantiated information by:

Georgina Espigol Frigolé
José Hernández Rodríguez
Maria Cinta Cid Xutgla
Sergio Prieto González

Published: 30 November 2018
Updated: 30 November 2018

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