Summary about Disease
Rheumatoid vasculitis (RV) is a rare but serious complication of long-standing, severe rheumatoid arthritis (RA). It involves inflammation of the blood vessels, leading to damage of organs and tissues supplied by those vessels. It's a systemic condition, meaning it can affect various parts of the body. The severity can range from mild skin rashes to life-threatening organ damage.
Symptoms
Symptoms vary based on which blood vessels are affected, but common ones include:
Skin: Ulcers (especially on legs), purpura (small, reddish-purple spots), livedo reticularis (a net-like pattern on the skin)
Nerves: Mononeuritis multiplex (nerve damage affecting individual nerves, leading to weakness, pain, or numbness in specific areas), peripheral neuropathy (generalized nerve damage in the extremities)
Eyes: Scleritis (inflammation of the white part of the eye), episcleritis (inflammation of the outer layer of the sclera)
Heart: Pericarditis (inflammation of the sac surrounding the heart), myocarditis (inflammation of the heart muscle), coronary arteritis (inflammation of the arteries supplying the heart)
Kidneys: Glomerulonephritis (inflammation of the kidney's filtering units)
Gastrointestinal Tract: Bowel ischemia (reduced blood flow to the intestines)
Other: Fever, weight loss, fatigue
Causes
RV is an autoimmune condition, meaning the body's immune system mistakenly attacks its own blood vessels. The exact cause isn't fully understood, but it's associated with:
Long-standing, severe rheumatoid arthritis (RA)
High levels of rheumatoid factor and anti-CCP antibodies (markers of RA)
Inflammation
Medicine Used
Treatment aims to suppress the immune system and reduce inflammation. Common medications include:
High-dose corticosteroids (e.g., prednisone): To quickly reduce inflammation.
Immunosuppressants:
Cyclophosphamide: A potent immunosuppressant, often used in severe cases.
Methotrexate: Commonly used for RA, may help control vasculitis.
Azathioprine: Another immunosuppressant.
Mycophenolate mofetil: An immunosuppressant
Biologic Agents:
Rituximab: Targets B cells (immune cells).
TNF inhibitors: Used in RA, but may not be effective for RV in some cases.
Other:
Intravenous immunoglobulin (IVIG): Can help modulate the immune system.
Antiplatelet agents (e.g., aspirin): To prevent blood clots.
Is Communicable
No. Rheumatoid vasculitis is not contagious or communicable. It is an autoimmune disease.
Precautions
Precautions focus on managing RA and minimizing risk factors:
Strict adherence to RA treatment plan: Taking prescribed medications as directed to control RA.
Regular monitoring for RV symptoms: Being vigilant for any new or worsening symptoms.
Healthy lifestyle: Balanced diet, regular exercise (as tolerated), and avoiding smoking.
Infection prevention: Washing hands frequently and avoiding close contact with sick individuals, as immunosuppressants increase the risk of infection.
Vaccinations: Staying up-to-date on vaccinations, but consulting with a doctor about which vaccines are safe when taking immunosuppressants.
How long does an outbreak last?
The duration of an RV "outbreak" or flare can vary significantly depending on the severity of the vasculitis, the organs affected, and the effectiveness of treatment. It can range from weeks to months. Without effective treatment, the condition can become chronic and cause ongoing damage.
How is it diagnosed?
Diagnosis involves a combination of:
Clinical evaluation: Review of symptoms and medical history, particularly related to RA.
Blood tests:
Inflammatory markers: ESR (erythrocyte sedimentation rate) and CRP (C-reactive protein) are often elevated.
Rheumatoid factor and anti-CCP antibodies: Typically positive and often high.
Complete blood count: To assess for anemia or other abnormalities.
Kidney and liver function tests: To assess organ involvement.
ANCA (antineutrophil cytoplasmic antibodies): May be positive in some cases.
Nerve conduction studies/EMG: To evaluate for nerve damage (mononeuritis multiplex).
Skin biopsy: To examine blood vessels in the skin for signs of inflammation.
Angiography: X-ray of blood vessels to look for abnormalities, narrowing, or blockages.
Organ-specific tests: Depending on symptoms (e.g., kidney biopsy if kidney involvement is suspected).
Timeline of Symptoms
RV typically develops in people who have had RA for many years (often 10 years or more). The timeline can vary:
Early stages: May start with mild skin manifestations or vague symptoms like fatigue or weight loss.
Progression: Over days, weeks, or months, symptoms can become more pronounced and involve multiple organs. Nerve symptoms (mononeuritis multiplex) or skin ulcers may develop.
Severe cases: Rapidly progressive organ damage can occur if the vasculitis is not promptly treated.
Important Considerations
RV is a serious complication of RA that requires prompt diagnosis and treatment.
Early diagnosis and aggressive treatment are crucial to prevent permanent organ damage.
Patients with RA should be educated about the signs and symptoms of RV and seek medical attention promptly if they develop any concerning symptoms.
Treatment often requires a combination of medications and close monitoring by a rheumatologist and other specialists as needed (e.g., nephrologist, neurologist).
Because of the immunosuppressive nature of treatments, close monitoring for infections and side effects is essential.