Rheumatoid Vasculitis: What It Is and How It Is Treated

Written by Medicover Team and Medically Reviewed by Dr Bhavana Surapareddy , Rheumatologists

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1. Causes 2. Find a Doctor 3. Diagnosis 4. Treatment 5. FAQs

Rheumatoid vasculitis (RV) is a rare but serious complication of longstanding rheumatoid arthritis (RA). It occurs when inflammation damages small and medium-sized blood vessels, reducing blood flow to the skin, nerves and, in severe cases, internal organs.

Rheumatoid vasculitis is an autoimmune condition in which the immune system mistakenly attacks the blood vessel walls. The resulting inflammation can narrow or block blood vessels and reduce the supply of oxygen and nutrients to affected tissues. Early diagnosis and treatment are important because severe disease can cause permanent tissue or organ damage.

What Are the Types of Rheumatoid Vasculitis?

Rheumatoid vasculitis can affect different parts of the body. The type and severity of symptoms depend on the blood vessels and organs involved.

Cutaneous Rheumatoid Vasculitis: Affects blood vessels in the skin and may cause rashes, ulcers, purple spots or changes around the nails.
Peripheral Nerve Vasculitis: Damages blood vessels supplying the nerves and may cause numbness, tingling, weakness or sudden loss of nerve function.
Systemic Rheumatoid Vasculitis: A more severe form that can affect internal organs, including the heart, lungs, kidneys, eyes or gastrointestinal tract.
Digital Vasculitis: Affects blood vessels in the fingers or toes and may cause pain, skin discolouration, sores or tissue damage.

What Are the Symptoms of Rheumatoid Vasculitis?

Symptoms of rheumatoid vasculitis vary depending on the blood vessels and organs affected. Skin and nerve problems are among the most common manifestations.

Skin Symptoms

Rashes: Red or purple spots and patches may appear on the skin.
Skin Ulcers: Painful open sores, particularly on the legs or around the ankles, may develop due to reduced blood flow.
Nail Bed Changes: Small areas of bleeding, known as splinter haemorrhages, may appear beneath the nails.
Finger or Toe Changes: Reduced circulation may cause pain, discolouration or tissue damage.

Neurological Symptoms

Peripheral Neuropathy: Nerve damage may cause tingling, numbness, burning pain or weakness in the hands and feet.
Mononeuritis Multiplex: Damage to multiple individual nerves can cause sudden pain, weakness or loss of function in different areas of the body.

General Symptoms

Fatigue
Unexplained fever
Unintentional weight loss
A general feeling of malaise
Muscle pain
Joint pain and swelling associated with rheumatoid arthritis

What Causes Rheumatoid Vasculitis?

The exact cause of rheumatoid vasculitis is not fully understood. It usually develops in people with longstanding and severe rheumatoid arthritis when abnormal immune activity causes inflammation and damage to blood vessel walls.

Severe Rheumatoid Arthritis

Rheumatoid vasculitis is most strongly associated with longstanding, poorly controlled or severe rheumatoid arthritis. Persistent inflammation may increase the risk of blood vessel involvement.

Immune System Dysfunction

Abnormal immune activity can cause immune complexes and inflammatory cells to attack blood vessel walls. This can narrow the vessels and reduce blood flow to surrounding tissues.

High Rheumatoid Factor Levels

People with high levels of rheumatoid factor are more likely to develop severe extra-articular complications of rheumatoid arthritis, including vasculitis.

Genetic Predisposition

Certain genetic factors may increase susceptibility to severe rheumatoid arthritis and rheumatoid vasculitis, although genetics alone do not cause the condition.

Smoking

Smoking is strongly associated with severe rheumatoid arthritis and may increase the risk of rheumatoid vasculitis by contributing to blood vessel damage and inflammation.

When Should You See a Doctor for Rheumatoid Vasculitis?

Medical evaluation by a Rheumatologist is important if a person with rheumatoid arthritis develops unexplained skin sores, rashes, numbness, or weakness. Early diagnosis can identify blood vessel inflammation, prevent tissue damage, and reduce the risk of serious nerve or organ complications.

You should see a doctor if you have:

New skin rashes, purple spots or painful ulcers
Persistent numbness, tingling or weakness in the limbs
Unexplained fever, fatigue or weight loss

Get medical help immediately if:

Sudden severe weakness or loss of movement develops
Fingers or toes become severely painful, blue or black
Chest pain, breathing difficulty or severe abdominal pain occurs

These could be signs of a serious complication like organ damage, which needs urgent care.

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How Is Rheumatoid Vasculitis Diagnosed?

Diagnosing rheumatoid vasculitis can be challenging because its symptoms may resemble other conditions. Doctors use medical history, physical examination, blood tests, imaging studies and sometimes a tissue biopsy.

Medical History and Physical Examination

The doctor reviews the duration and severity of rheumatoid arthritis and checks for skin ulcers, rashes, nerve problems and signs of reduced blood flow.

Blood Tests

Inflammatory Markers: ESR and C-reactive protein (CRP) tests may show increased inflammation.
Rheumatoid Factor: High rheumatoid factor levels are commonly associated with rheumatoid vasculitis.
Anti-CCP Antibodies: Elevated levels may support the diagnosis of severe rheumatoid arthritis.
Complete Blood Count: This test can help identify anaemia or other abnormalities associated with systemic inflammation.

Imaging Studies

Angiography: This test can show narrowing, blockage or other abnormalities in affected blood vessels.
MRI or CT Scan: These imaging tests may help assess internal organ involvement and identify complications.

Nerve Tests

Nerve conduction studies and electromyography may be performed when symptoms suggest peripheral nerve damage or mononeuritis multiplex.

Biopsy

A biopsy of affected skin, nerve, muscle or another tissue may be needed to confirm the diagnosis. The tissue sample is examined for inflammation and damage in the blood vessel walls.

How Is Rheumatoid Vasculitis Treated?

Treatment for rheumatoid vasculitis aims to control blood vessel inflammation, protect affected tissues and organs and manage the underlying rheumatoid arthritis. Treatment depends on the severity and extent of the disease.

Corticosteroids

Corticosteroids may be prescribed to rapidly reduce inflammation. Severe cases may require high-dose treatment, while milder disease may be managed with lower doses.

Immunosuppressive Medicines

Cyclophosphamide: May be used for severe or life-threatening vasculitis involving major organs.
Methotrexate: May help control rheumatoid arthritis and some less severe forms of vasculitis.
Other Immunosuppressants: Additional medicines may be considered depending on disease severity and treatment response.

Biologic Medicines

Targeted biologic medicines, such as rituximab, may be considered for severe rheumatoid vasculitis or disease that does not respond adequately to conventional treatments.

Wound Care

Skin ulcers require proper cleaning, protection and monitoring. Infected ulcers may need antibiotics and specialised wound care to prevent further tissue damage.

Physical Therapy

Physical therapy may help maintain muscle strength, joint movement and mobility, particularly in people affected by nerve damage or longstanding rheumatoid arthritis.

Smoking Cessation

Stopping smoking is an important part of treatment because tobacco use can worsen blood vessel damage and is associated with more severe rheumatoid arthritis.

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What Are the Risk Factors for Rheumatoid Vasculitis?

Rheumatoid vasculitis is uncommon, but certain factors can increase the likelihood of developing this complication.

Longstanding Rheumatoid Arthritis: The condition is more common after many years of rheumatoid arthritis.
Severe Rheumatoid Arthritis: Persistent and poorly controlled disease increases the risk.
High Rheumatoid Factor Levels: High levels are strongly associated with rheumatoid vasculitis.
Rheumatoid Nodules: People with subcutaneous nodules may have a higher risk of vasculitis.
Smoking: Tobacco use is an important risk factor for severe rheumatoid arthritis and vascular complications.
Male Sex: Although rheumatoid arthritis is more common in women, rheumatoid vasculitis has historically been more frequent in men with severe disease.

What Are the Complications of Rheumatoid Vasculitis?

Untreated or severe rheumatoid vasculitis can restrict blood flow and cause permanent damage to tissues, nerves and internal organs.

Chronic Skin Ulcers: Poor circulation can cause persistent wounds that are difficult to heal.
Infection: Open skin ulcers may become infected.
Permanent Nerve Damage: Reduced blood flow to nerves can cause lasting pain, numbness or weakness.
Tissue Death: Severe loss of blood supply may cause gangrene in the fingers or toes.
Organ Damage: In rare cases, the heart, lungs, kidneys, eyes or gastrointestinal tract may be affected.
Reduced Mobility: Nerve damage and severe joint disease may interfere with movement and daily activities.

What Is the Prognosis for Rheumatoid Vasculitis?

The outlook for rheumatoid vasculitis depends on the severity of blood vessel inflammation, the organs involved and how quickly treatment begins. Mild disease limited to the skin may respond well to treatment, while systemic disease involving major organs requires more aggressive care.

Advances in rheumatoid arthritis treatment have made rheumatoid vasculitis less common. Early diagnosis, appropriate immunosuppressive treatment, smoking cessation and regular follow-up can help control the condition and reduce the risk of permanent tissue or organ damage.

Frequently Asked Questions

1. What are the symptoms of rheumatoid vasculitis?

Symptoms may include joint pain, skin rashes, and systemic symptoms like fever, indicating an autoimmune condition that requires evaluation and treatment.