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Systemic Vasculitis

Rheumatology

Systemic vasculitis is an autoimmune disease that causes inflammation of the blood vessels, affecting blood flow and vessel structure. Inflamed vessels can narrow through wall thickening or develop cracks due to the inflammatory response, leading to serious health issues. Systemic vasculitis can be classified into large, medium, and small vessel vasculitis based on the size of the affected vessels.

Symptoms

Symptoms vary depending on the location and severity of the affected vessels and include:

Large Vessel Vasculitis (e.g., Takayasu Arteritis)	Headache, dizziness, low-grade fever, fatigue, hypertension, absence of pulse, and possibly heart failure.
Medium Vessel Vasculitis (e.g., Kawasaki Disease)	High fever, skin rash, enlarged lymph nodes, red eyes, and joint pain.
Small Vessel Vasculitis (e.g., Henoch-Schönlein Purpura)	Purpura and bruising, primarily on the lower limbs, along with abdominal pain and joint pain.

Causes

The exact cause of systemic vasculitis is not completely understood but is typically associated with an autoimmune response, potentially triggered by genetic, infectious, or environmental factors.

Risk Factors

Genetic Factors: Family history of autoimmune diseases.
Environmental Factors: Infections and certain medications.
Other Autoimmune Diseases: Conditions like rheumatoid arthritis or lupus.

Diagnosis

Clinical Examination: Checking for symptoms like fever, fatigue, and weight loss.
Blood Tests: Measuring inflammatory markers such as ESR (erythrocyte sedimentation rate) and CRP (C-reactive protein).
Imaging Tests: Including ultrasound, MRI, and CT scans to check for vessel narrowing or blockage.
Tissue Biopsy: Taking skin, kidney, or other tissue samples for pathological examination.

Treatments

The goal of treatment is to control inflammation and prevent organ damage, primarily through medication and, if necessary, surgery.

Medication:
- Corticosteroids: Such as prednisone, to reduce inflammation.
- Immunosuppressants: Such as azathioprine and cyclophosphamide, to suppress the overactive immune system.
- Intravenous Immunoglobulin (IVIG): Especially used for treating Kawasaki disease.
- Antiplatelet Drugs: Such as aspirin, to reduce blood clot formation.
Surgery: If vessels are severely narrowed or blocked, procedures like stenting, angioplasty, or bypass surgery may be needed.

FAQ

Systemic Vasculitis cannot be cured entirely at present, but the condition can be effectively controlled through medication and lifestyle management. Systemic vasculitis requires long-term management. Patients should closely collaborate with a rheumatologist, regularly monitor their condition, and adjust treatment plans to reduce the chances of disease progression and the occurrence of complications.

Improper management of systemic vasculitis can lead to organ damage, such as kidney failure, heart disease, intestinal gangrene, and other serious complications.