From the Guidelines
Vasculitis is a group of disorders characterized by inflammation of blood vessels, which can lead to damage and narrowing of the vessels, potentially restricting blood flow to organs and tissues. This condition occurs when the immune system mistakenly attacks blood vessel walls, causing them to become inflamed, weakened, and thickened. Vasculitis can affect vessels of any size—from large arteries to small capillaries—and can target specific organs or multiple body systems simultaneously. Symptoms vary widely depending on which blood vessels and organs are affected but commonly include fever, fatigue, weight loss, muscle and joint pain, and organ-specific problems.
Key Characteristics of Vasculitis
- Inflammation of blood vessels
- Damage and narrowing of vessels
- Restriction of blood flow to organs and tissues
- Immune system mistakenly attacks blood vessel walls
- Can affect vessels of any size
- Can target specific organs or multiple body systems simultaneously Treatment typically involves corticosteroids like prednisone to reduce inflammation, often combined with immunosuppressants such as cyclophosphamide, methotrexate, or rituximab for more severe cases, as recommended by the EULAR guidelines 1. The specific treatment regimen depends on the type of vasculitis, its severity, and which organs are involved.
Diagnosis and Treatment
- ANCA testing, including indirect immunofluorescence and ELISA, should be performed in the appropriate clinical context 1
- A positive biopsy is strongly supportive of vasculitis and can assist diagnosis and further evaluation for patients suspected of having vasculitis 1
- A structured clinical assessment, urine analysis, and other basic laboratory tests should be conducted at each clinical visit for patients with vasculitis 1 Early diagnosis and treatment are crucial to prevent permanent organ damage, as vasculitis can be life-threatening if major blood vessels or vital organs are affected. The underlying cause of most forms of vasculitis remains unknown, though some cases are linked to immune system disorders, infections, or reactions to certain medications.
Importance of Early Diagnosis and Treatment
- Prevent permanent organ damage
- Reduce risk of life-threatening complications
- Improve quality of life for patients with vasculitis It is essential to manage patients with primary small and medium vessel vasculitis in collaboration with, or at centers of expertise, as recommended by the EULAR guidelines 1.
From the Research
Definition of Vasculitis
- Vasculitis is a pathologic condition defined by inflammatory cell infiltration and destruction of blood vessels 2
- It is characterized by the inflammation of blood vessels, and can occur as a primary process or secondary to many systemic diseases 3
- Vasculitis is an autoimmune connective tissue disease that targets blood vessels in the body, leading to inflammation and potential damage to vital organs 4
Classification of Vasculitis
- Vasculitis can be classified as primary (eg, polyarteritis nodosa, Churg-Strauss syndrome) or secondary, the latter associated with connective tissue disorders, infections, medications, and rarely, as a paraneoplastic phenomenon 2
- Vasculitides refers to the different forms of vasculitis, often classified according to the size of the blood vessel that is involved 3
Forms of Vasculitis
- Systemic vasculitis is a condition where the inflammation affects multiple blood vessels throughout the body 2
- Nonsystemic vasculitic neuropathy (NSVN) is a condition where the inflammation is limited to the blood vessels supplying the nerves 2
- Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a type of vasculitis that can present with life-threatening lung-kidney syndromes 5
Diagnosis and Treatment
- The diagnosis of vasculitis should be established by tissue (preferably nerve) biopsy 2
- Treatment of vasculitic neuropathy requires long-term immunosuppressive therapies with potential side effects 2
- High-dose prednisone is the standard platform therapy for patients with systemic and NSVN 2
- Combination therapy with rituximab, low-dose cyclophosphamide, and plasma exchange may be effective for severe ANCA-associated vasculitis 5, 6