Vasculitis

Vasculitis is a broad term that refers to a group of rare diseases characterized by inflammation of the blood vessels. This inflammation can cause the walls of the blood vessels to thicken, leading to a reduction in blood flow and, in severe cases, tissue and organ damage. Vasculitis can affect arteries, veins, and capillaries of different sizes, impacting various parts of the body.

This blog explores the causes, symptoms, types, diagnosis, treatment, and long-term outlook of the disease.

What is Vasculitis?

Vasculitis, also known as angiitis or arteritis, occurs when the immune system mistakenly attacks blood vessels, leading to inflammation. This can cause blood vessel narrowing, blockage, weakening, or rupture, potentially leading to serious health complications.

The severity of the disease varies depending on which blood vessels and organs are affected. It may be mild and self-limiting or severe and life-threatening.

Causes of Vasculitis

The exact cause is often unknown, but it is believed to result from an abnormal immune response. Some factors that may contribute to vasculitis include:

1. Autoimmune Disorders – Conditions such as rheumatoid arthritis, lupus, and scleroderma can trigger vasculitis.
2. Infections – Viral infections like hepatitis B and hepatitis C can lead to vasculitis.
3. Certain Medications – Some drugs, including antibiotics and nonsteroidal anti-inflammatory drugs (NSAIDs), have been linked to drug-induced vasculitis.
4. Genetic Predisposition – Some types of vasculitis may run in families.
5. Environmental Triggers – Exposure to toxins, allergens, or extreme stress may contribute to vasculitis in susceptible individuals.

Types

Vasculitis is classified based on the size of the affected blood vessels:

1. Large Vessel Vasculitis

• Giant Cell Arteritis (GCA) – Mostly affects older adults, leading to headaches, vision problems, and jaw pain.
• Takayasu Arteritis – Primarily affects young women, leading to weakened pulses, fatigue, and high blood pressure.

2. Medium Vessel Vasculitis

• Polyarteritis Nodosa (PAN) – Affects the skin, kidneys, nerves, and digestive system, often linked to hepatitis B infection.
• Kawasaki Disease – Mainly affects children, leading to fever, rash, swollen lymph nodes, and heart complications.

3. Small Vessel Vasculitis

• Granulomatosis with Polyangiitis (GPA, formerly Wegener’s Granulomatosis) – Affects the sinuses, lungs, and kidneys, causing inflammation and granuloma formation.
• Microscopic Polyangiitis (MPA) – Involves kidney and lung inflammation, often leading to kidney failure.
• Eosinophilic Granulomatosis with Polyangiitis (EGPA, formerly Churg-Strauss Syndrome) – Associated with asthma and high eosinophil counts, affecting the lungs, skin, and nerves.
• Henoch-Schönlein Purpura (HSP) – Affects children, leading to purpura (skin rash), abdominal pain, and kidney inflammation.

Vasculitis symptoms

Symptoms depend on the affected organs but commonly include:

• General Symptoms:
  • Fever
  • Fatigue
  • Weight loss
  • Muscle and joint pain
• Organ-Specific Symptoms:
  • Skin – Rash, purpura (red or purple spots), ulcers
  • Nervous System – Stroke, numbness, tingling, weakness
  • Lungs – Shortness of breath, coughing up blood
  • Heart – Chest pain, myocardial infarction
  • Digestive System – Abdominal pain, bloody stool
  • Kidneys – Blood in urine, glomerulonephritis
  • Eyes – Vision problems, redness

How is Vasculitis Diagnosed?

Diagnosis involves a combination of clinical evaluation, laboratory tests, imaging studies, and sometimes a biopsy.

1. Blood Tests

• Complete Blood Count (CBC) – Checks for signs of infection, inflammation, or anemia.
• C-Reactive Protein (CRP) and Erythrocyte Sedimentation Rate (ESR) – Detects inflammation levels.
• Antineutrophil Cytoplasmic Antibody (ANCA) Test – Helps diagnose certain types of vasculitis like GPA and MPA.

2. Urine Tests

• Checks for blood or protein in the urine, indicating kidney involvement.

3. Imaging Tests

• X-rays, CT Scans, or MRI – Identify blood vessel inflammation or organ damage.
• Angiography – Uses contrast dye to visualize blood vessels.

4. Biopsy

• A small tissue sample from the affected organ (e.g., skin, kidney, or lung) is examined under a microscope for signs of vasculitis.

Vasculitis treatment

The treatment depends on its severity and type. The goal is to control inflammation, prevent complications, and minimize long-term damage.

1. Medications

• Corticosteroids (Prednisone, Methylprednisolone) – Reduce inflammation and suppress the immune system.
• Immunosuppressants (Cyclophosphamide, Methotrexate, Azathioprine) – Used for severe cases to control the immune response.
• Biologic Therapies (Rituximab, Tocilizumab) – Target specific immune pathways and are used for refractory cases.
• NSAIDs (Ibuprofen, Naproxen) – Help relieve joint and muscle pain.
• Plasma Exchange (Plasmapheresis) – Used in severe cases to remove harmful antibodies from the blood.

2. Lifestyle Modifications

• Healthy Diet – A balanced diet rich in fruits, vegetables, and lean proteins helps reduce inflammation.
• Regular Exercise – Improves cardiovascular health and reduces fatigue.
• Smoking Cessation – Smoking worsens blood vessel damage.
• Stress Management – Meditation, yoga, and counseling can help manage stress and improve overall well-being.

3. Surgery

In rare cases, surgery may be required to repair damaged blood vessels or remove aneurysms.

Complications

If left untreated, vasculitis can lead to severe complications, including:

• Organ failure (kidney, lung, or heart)
• Stroke or neurological damage
• Vision loss
• Tissue death (gangrene)
• Aneurysms or ruptured blood vessels

Prognosis and Long-Term Management

The prognosis for vasculitis depends on its type, severity, and response to treatment. Some forms of vasculitis, like Kawasaki disease, may resolve completely, while others, like GPA or MPA, may require lifelong management.

Tips for Managing Vasculitis Long-Term

• Regular follow-ups with a rheumatologist or specialist
• Adherence to prescribed medications
• Routine blood and urine tests to monitor organ function
• Promptly addressing infections or new symptoms

Vasculitis is a complex disease that can affect multiple organs and systems in the body. Early diagnosis and proper treatment are crucial to prevent severe complications. If you experience unexplained symptoms such as persistent fever, fatigue, joint pain, skin rashes, or organ-related issues, consult a doctor for further evaluation.

Managing vasculitis requires a combination of medical treatment, lifestyle modifications, and ongoing monitoring. With the right care, many individuals with vasculitis can lead fulfilling lives.

Would you like to raise awareness about vasculitis? Share this blog with your friends and family to help spread knowledge about this rare but serious condition!

To consult a Rheumatologist at Sparsh Diagnostic Centre, call our helpline number 9830117733.

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No content on this site, regardless of date, should ever be used as a substitute for direct medical advice from your doctor or other qualified clinician.