What Is Rheumatoid Vasculitis?

Rheumatoid vasculitis is a rare, serious complication of rheumatoid arthritis in which inflammation spreads to small- or medium-size blood vessels in the body. This inflammation causes the walls of blood vessels to thicken and narrow, compromising blood flow. Rheumatoid vasculitis may involve multiple organs, including the skin, eyes, nerves, heart, lung, brain, kidney, or gastrointestinal tract.

Any organ of the body can be affected by rheumatoid vasculitis. Symptoms depend on which area of the body is affected. The skin and peripheral nerves (those that deliver information to and from the central nervous system, brain, and spinal cord) are most commonly involved.

Area-specific symptoms include:

• Eyes: Scleritis (inflammation of the white part of the eye) causing light sensitivity and pain
• Skin: Redness (purpura) and ulcers; ankles are particularly vulnerable to skin ulcers
• Fingers: Sores and redness around the nails, small pits in the fingertips, and in severe cases, tissue death (necrosis) that can cause gangrene
• Legs: Painful red rash or purple bruising (levido reticularis)
• Nervous system: Weakness, numbness and tingling, especially in the hands and feet. With the peripheral nervous system, breakdowns in nerve communications from nerve damage (peripheral neuropathies) may occur. Hand or foot drop may also occur.
• Lungs: Inflammation of the membrane covering the lungs and chest cavity (pleuritis).
• Heart: Inflammation of the sac surrounding the heart (pericarditis)
• Large arteries: Stomach pain, chest pain, and in worst cases, heart attack or stroke. Involvement of the larger arteries and more systemic vasculitis may bring more generalized symptoms such as fever, weight loss, loss of appetite, and loss of energy

While major organ involvement is considered less common, it is associated with significant morbidity and mortality.

There is no known cause of rheumatoid vasculitis. Rheumatoid arthritis, however, is an autoimmune disease that causes systemic inflammation, so it's possible that it could attack smaller blood vessels.

The following factors increase the risk of developing rheumatoid vasculitis:

• Gender: Men are more likely to develop RV (1 in 9 males with rheumatoid arthritis)
• Smoking
• Presence of rheumatoid nodules, hardened lumps that form under the skin, most often around the elbows, heels, or knuckles
• Age: Older at disease onset or long duration of rheumatoid arthritis (more than 10 years)
• Enlarged spleen
• Low white blood cell count (Felty's syndrome)

The presence of rheumatoid arthritis coupled with RV symptoms may be enough for a physician to suspect and even diagnose rheumatoid vasculitis, but a biopsy is required for a definitive diagnosis. Involved skin may be sampled, as well as part of a muscle or nerve within an affected region, or an affected organ. 

Certain blood tests may also be involved looking for the following RA-related serum markers:

• Positive for rheumatoid factor
• Positive for anti-cyclic citrullinated peptide (anti-CCP)
• Lower levels of plasma proteins in the blood (called complement), which are used up when inflammation occurs

Anti-neutrophil cytoplasmic antibodies (ANCA) and associated anti-myeloperoxidase and anti-proteinase-3 antibodies are typically negative in rheumatoid vasculitis.

First, if present, rheumatoid arthritis must be effectively treated using DMARDs or biologic drugs, such as TNF blockers. Controlling inflammation in both the joints and blood vessels is essential. Direct treatment of rheumatoid vasculitis itself is largely determined by which organs are involved.

The first line of treatment for rheumatoid vasculitis involves the use of corticosteroids (usually prednisone). Prednisone can be paired with methotrexate or azathioprine.

With advanced symptoms and severe organ involvement, a more aggressive effort for immunosuppression may involve cyclophosphamide along with higher doses of prednisone. 

While the prevalence of rheumatoid vasculitis appears to be decreasing, it is estimated that fewer than 5% of the rheumatoid arthritis patient population develops rheumatoid vasculitis.

Early detection and treatment are critical to prevent damage to blood vessels. If the skin is involved without other systemic involvement, RV prognosis is generally good.

However, severe cases may involve ongoing immunosuppressive therapy. Older studies have shown five-year RV mortality rates between 30% and 50% due to complications and treatment toxicity. Those rates may have improved with newer therapies, but more research is needed.

While rheumatoid vasculitis is a serious and concerning complication of rheumatoid arthritis, effective treatment of RA and vigilance for RV symptoms may allow patients to identify the condition early and begin treatment. Your rheumatologist will guide your treatment and advise you of potential risk factors.