Systemic necrotizing vasculitis

Systemic Necrotizing Vasculitis (SNV) is a group of rare inflammatory diseases characterized by inflammation and necrosis of the blood vessel walls. The exact cause of SNV is unknown, but it is thought to involve the immune system attacking the blood vessels by mistake. This condition can affect vessels of any size, including arteries, arterioles, veins, and capillaries, leading to a wide range of symptoms depending on the organs affected.

Classification

Systemic Necrotizing Vasculitis is classified based on the size of the affected vessels and the specific organs involved. Major categories include:

• Giant Cell Arteritis and Takayasu's Arteritis, affecting large vessels.
• Polyarteritis Nodosa (PAN) and Kawasaki Disease, affecting medium-sized vessels.
• Granulomatosis with Polyangiitis (formerly known as Wegener's Granulomatosis), Microscopic Polyangiitis, and Churg-Strauss Syndrome (also known as Eosinophilic Granulomatosis with Polyangiitis), affecting small vessels.

Symptoms

Symptoms of SNV vary widely depending on the specific type of vasculitis and the organs involved but may include fever, fatigue, weight loss, muscle and joint pain, and symptoms related to specific organ involvement such as skin rashes, kidney dysfunction, and nerve problems.

Diagnosis

Diagnosis of SNV involves a combination of clinical evaluation, laboratory tests (including blood tests for inflammation markers and specific antibodies), imaging studies, and often a biopsy of affected tissue to confirm the presence of vasculitis.

Treatment

Treatment for SNV typically involves immunosuppressive medications to reduce inflammation and prevent further damage to the blood vessels and affected organs. Corticosteroids are commonly used, often in combination with other immunosuppressive agents such as cyclophosphamide, methotrexate, or azathioprine. The choice of treatment depends on the specific type of vasculitis, the severity of the disease, and the organs involved.

Prognosis

The prognosis for individuals with SNV varies depending on the type of vasculitis, the organs involved, and the response to treatment. Early diagnosis and appropriate treatment can significantly improve outcomes, but some forms of vasculitis can be chronic and relapsing, requiring long-term management.