What are the types of vasculitis?

From the Guidelines

Vasculitis encompasses several types of inflammatory blood vessel disorders, including Giant Cell Arteritis, Takayasu's Arteritis, Polyarteritis Nodosa, Kawasaki Disease, ANCA-associated vasculitides, and others, which require prompt diagnosis and treatment to prevent significant organ damage 1. The main types of vasculitis include:

• Giant Cell Arteritis (affecting large arteries, especially temporal arteries)
• Takayasu's Arteritis (affecting the aorta and its branches)
• Polyarteritis Nodosa (medium-sized arteries)
• Kawasaki Disease (medium-sized vessels, primarily in children)
• ANCA-associated vasculitides, including Granulomatosis with Polyangiitis, Microscopic Polyangiitis, and Eosinophilic Granulomatosis with Polyangiitis
• Other types, such as Behçet's Disease, Cryoglobulinemic Vasculitis, IgA Vasculitis, and Central Nervous System Vasculitis Treatment typically involves corticosteroids, like prednisone, and immunosuppressants, such as cyclophosphamide, rituximab, methotrexate, or azathioprine, with the specific regimen depending on the vasculitis type, severity, and organ involvement 1. For example, severe ANCA-associated vasculitis often requires cyclophosphamide or rituximab plus corticosteroids, while milder cases might be managed with methotrexate and lower-dose steroids 1. The most recent and highest quality study, published in 2024, recommends a combination of cyclophosphamide and glucocorticoids for remission induction of generalized primary small and medium vessel vasculitis 1. Key points to consider in the management of vasculitis include:
• Prompt diagnosis and treatment to prevent significant organ damage
• Use of corticosteroids and immunosuppressants, with the specific regimen depending on the vasculitis type, severity, and organ involvement
• Monitoring for disease activity and treatment side effects
• Consideration of alternative immunomodulatory therapy choices for patients who do not achieve remission or relapse on maximal doses of standard therapy 1.

From the Research

Types of Vasculitis

• Vasculitis comprises a broad group of syndromes characterized by inflammation and necrosis on the walls of blood vessels, resulting in narrowing or occlusion of the lumen 2
• The distribution of involved blood vessel varies considerably and serves as the basis for one classification of vasculitic syndromes:
  • Large vessels (Takayasu arteritis, giant cell arteritis)
  • Medium and small muscular arteries (polyarteritis nodosa, Churg-Strauss syndrome, Wegener's granulomatosis, vasculitis in rheumatic diseases)
  • Small vessels ("hypersensitivity" vasculitis, Henoch-Schonlein purpura, microscopic polyangiitis, cryoglobulinemia) 2

Large Vessel Vasculitis

• Giant cell arteritis and Takayasu arteritis are the two major forms of idiopathic large vessel vasculitis 3
• High doses of glucocorticoids are effective in inducing remission in both conditions, but relapses and recurrences are common, requiring prolonged glucocorticoid treatment with the risk of the related adverse events 3
• Large-vessel vasculitis includes giant cell arteritis (GCA) and Takayasu arteritis (TA), GCA can affect persons from the age of 50 years and is more frequent among women 4

Medium Vessel Vasculitis

• The most common causes of medium-vessel arteritis are polyarteritis nodosa and Kawasaki disease 5
• Medium vessel vasculitis refers to the main visceral arteries and veins and their initial branches 5

Classification and Diagnosis

• Systemic vasculitides are multisystem blood vessel disorders, which are defined by the size of the vessel predominantly affected, namely small, medium, or large vessels 5
• Prompt identification of vasculitides is important because they are associated with an increased risk of mortality 5
• The clinical presentation of vasculitis is highly variable, the cardiovascular clinician must have a high index of suspicion to establish a reliable and prompt diagnosis 5