Types of Vasculitis

Vasculitis is classified by the size of blood vessels affected — large, medium, and small vessel disease.

Types of Vasculitis by size of vasculature

The Chapel Hill Consensus Conference classification system categorises vasculitis by the size of vessels primarily affected. Understanding the type helps guide treatment and prognosis.

Large Vessel Medium Vessel Small Vessel Variable

Large Vessel Vasculitis

Giant Cell Arteritis (GCA)

Large

Inflammation of the lining of arteries, most often those in the head, especially the temporal. Usually affects people over 50. Can cause severe headaches, jaw pain, and vision loss if untreated. Treated with high-dose corticosteroids.

Takayasu's Arteritis

Large

A rare form affecting the aorta and its major branches. Most common in young women, particularly in Asia. Can cause reduced blood flow to arms and legs. Close monitoring is critical during pregnancy for affected women.

Medium Vessel Vasculitis

Polyarteritis Nodosa (PAN)

Medium

Affects medium-sized arteries and can damage the skin, kidneys, nerves, and intestines. Can be associated with hepatitis B. Symptoms include skin nodules, joint pain, and abdominal pain.

Kawasaki Disease

Medium

Primarily affects children under 5. Causes inflammation in the walls of medium-sized arteries, including coronary arteries, and can lead to heart complications if not treated promptly with IVIG and aspirin.

Small Vessel Vasculitis

Granulomatosis with Polyangiitis (GPA)

Small

Previously known as Wegener's granulomatosis. Affects small and medium vessels, commonly in the nose, sinuses, lungs, and kidneys. An ANCA-associated vasculitis treated with immunosuppression.

Microscopic Polyangiitis (MPA)

Small

Affects small blood vessels, commonly in the kidneys and lungs. An ANCA-associated vasculitis that can cause rapidly progressive kidney disease and pulmonary haemorrhage requiring urgent treatment.

EGPA (Eosinophilic Granulomatosis)

Small

Previously known as Churg-Strauss syndrome. Associated with asthma and high eosinophil count, affecting small to medium vessels. Managed with corticosteroids and biologics like mepolizumab.

IgA Vasculitis (Henoch-Schönlein)

Small

The most common vasculitis in children. Causes palpable purpura (raised red-purple spots), joint pain, and can affect kidneys and intestines. Often self-limiting in children but may persist in adults.

Cryoglobulinemic Vasculitis

Small

Associated with abnormal proteins (cryoglobulins) that clump in cold temperatures and damage small blood vessels. Often linked to hepatitis C infection. Presents with purpura, joint pain, and kidney disease.

Cutaneous Small Vessel Vasculitis

Small

Primarily limited to the skin, causing palpable purpura, skin ulcers, and nodules. Often triggered by infections, medications, or systemic diseases. Usually self-limiting and managed conservatively.

Variable & Other Forms

Behçet's Disease

Variable

Causes inflammation of blood vessels throughout the body. Characterised by recurrent oral and genital ulcers, eye inflammation (uveitis), and skin lesions. More common in Middle Eastern and Asian populations.

DADA2 Syndrome

Variable

A rare genetic disorder involving mutations in the ADA2 gene causing enzyme deficiency, resulting in systemic inflammation and immune dysregulation. Can mimic PAN and cause strokes in young patients.

Cogan Syndrome

Variable

A rare autoimmune disease characterised by eye inflammation (interstitial keratitis) and inner ear disease, potentially causing deafness. Can affect large vessels similar to Takayasu's arteritis.

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