Systemic vasculitis

Systemic vasculitis refers to a group of disorders characterized by inflammation of blood vessels, which can affect various organs and tissues throughout the body. This condition can lead to vessel wall damage, resulting in tissue ischemia and organ dysfunction.

Classification

Systemic vasculitis is classified based on the size of the affected blood vessels:

Large vessel vasculitis

• Giant cell arteritis
• Takayasu's arteritis

Medium vessel vasculitis

• Polyarteritis nodosa
• Kawasaki disease

Small vessel vasculitis

• Granulomatosis with polyangiitis
• Microscopic polyangiitis
• Eosinophilic granulomatosis with polyangiitis

Pathophysiology

The pathophysiology of systemic vasculitis involves immune-mediated inflammation of the blood vessel walls. This can be triggered by infections, drugs, or autoimmune processes. The inflammation leads to vessel wall damage, which can cause stenosis, aneurysm formation, or rupture.

Clinical manifestations

The clinical manifestations of systemic vasculitis vary depending on the size and location of the affected vessels. Common symptoms include:

• Fever
• Fatigue
• Weight loss
• Muscle and joint pain

Organ-specific symptoms

• Renal: Hematuria, proteinuria, renal failure
• Pulmonary: Cough, hemoptysis, dyspnea
• Dermatological: Purpura, ulcers, nodules

Diagnosis

Diagnosis of systemic vasculitis involves a combination of clinical evaluation, laboratory tests, and imaging studies. Common diagnostic tools include:

• Blood tests: Elevated inflammatory markers, ANCA
• Biopsy: Histological examination of affected tissue
• Imaging: Angiography, MRI, CT scan

Treatment

Treatment of systemic vasculitis aims to reduce inflammation and prevent organ damage. Common treatment options include:

• Corticosteroids: Prednisone
• Immunosuppressive drugs: Cyclophosphamide, methotrexate
• Biologic agents: Rituximab

Prognosis

The prognosis of systemic vasculitis depends on the type and severity of the disease, as well as the response to treatment. Early diagnosis and appropriate management are crucial for improving outcomes.

Related pages

• Autoimmune disease
• Inflammation
• Rheumatology

Micrograph showing vasculitis.