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Everything You Need to Know About Rheumatoid Vasculitis

Medically reviewed by Stella Bard, MD — By Corey Whelan on October 24, 2022Rheumatoid vasculitis (RV) is a rare and potentially severe complication of long-term rheumatoid arthritis (RA). RV causes small or medium-sized blood vessels in various parts of your body to become inflamed. The walls of the affected blood vessels thicken and narrow, inhibiting blood flow. Read on to learn about the causes, treatments, and risk factors for RV.

What causes rheumatoid vasculitis

It’s not exactly clear what causes RV. Since rheumatoid arthritis is an autoimmune disease, some research suggests that an immune system overreaction may cause RV. Unlike RA, which primarily impacts your joints, RV may cause your body to attack its own blood vessels, causing inflammation and other symptoms. Genetics, infections, and drug reactions may also play a role, either on their own or in some combination. More research is needed into what causes or triggers RV.

Who gets rheumatoid vasculitis

Less than 1% of people with RA develop RV. This figure continues to decline as treatments for RA improve. However, you are most at risk for this condition if you’ve had severe RA for 10 years or longer. In addition to long-term RA, risk factors for RV include:smoking cigaretteshaving high blood levels of rheumatoid factor antibodies (proteins your immune system produces that attack healthy tissue)having rheumatoid nodules (firm lumps under your skin near joints)Felty’s syndrome (RA, low white blood cell count, and an enlarged spleen) Though RA and many of its complications are much more common in females, RV is more than twice as common in males.

Can you get RV if you have not had RA for very long

Though it’s rare, you could develop RV without longstanding RA. Reports from 2017 and 2019 describe such cases. A case study from 2017 even reports a diagnosis of RV prior to an RA diagnosis. In all cases, there were other risk factors at play.

What are the symptoms of rheumatoid vasculitis

Some general symptoms of RV are also due to RA. These include:fatigueloss of appetiteweight lossfevermuscles weakness With RV, these symptoms may be more severe. Localized symptoms of RV can occur anywhere in your body, including your internal organs. Examples include:chest pain or shortness of breath if RV affects your heart or lungsstomach pain if it affects your gastrointestinal systemeye redness or impaired vision if it affects your eyes RV most typically affects these areas:

Skin

RV can affect the blood vessels under your skin, causing visual changes. You may notice:red, painful rashes, especially on your legspurple-looking bruisesulcers (open sores)

Peripheral nerves

Peripheral nerves are those nerves outside of your brain and spinal cord. RV can cause inflammation that affects these nerves anywhere in your body. You may experience:numbnesstinglingweakness You’re most likely to feel this in your hands and feet.

Fingers and toes

The tips of your fingers and toes have many very small blood vessels. When RV affects these vessels, it can affect blood flow to these parts. You may experience:small, pitted areas in your fingertips.ulcers (open sores) around one or more fingernailsnecrosis (tissue death) of the fingers and toes You may also experience numbness and tingling from peripheral nerve damage in your fingers and toes.When to contact a doctor If you have RA and experience RV symptoms, let your doctor know immediately. While uncommon, serious and life threatening complications of RV can happen if the internal organs are affected by vasculitis or inflammation.

How do doctors diagnose rheumatoid vasculitis

If you have RA, a doctor may diagnose you with RV based on your physical symptoms. They’ll check your body for signs of rash, pitting, ulcers, and tissue necrosis. There’s no specific diagnostic test to confirm RV. Still, a doctor may run tests that may help confirm the diagnosis. Testing can rule out other conditions with similar symptoms, such as diabetes. Tests may include:blood tests to check for antibodies and proteins that might signal high levels of inflammationurine test to check for proteins, sugar, and other substancesimaging tests, such as a chest X-ray, CT scan, MRI, and angiogram (a technique that uses a dye that allows you to see the inside of blood vessels)electrocardiogram (ECG)tissue biopsy to check for signs of tissue damage and inflammationendoscopy (for gastrointestinal symptoms)nerve conduction test to check for nerve damage and test nerve function

What is the treatment for rheumatoid vasculitis

Your treatments for RV may depend on the extent of damage to your blood vessels. A doctor will also consider which parts of your body are affected. Some RA drugs may help relieve RV symptoms. These include certain biologic treatments and disease-modifying anti-rheumatic drugs (DMARDs). If RV symptoms are limited to your fingertips, a doctor may prescribe corticosteroids, such as prednisone. These reduce inflammation in the area and the potential for infection. More widespread RV symptoms may require the use of corticosteroids and immunosuppressant drugs. Immunosuppressants stop your immune system’s overreaction to healthy tissues, joints, and veins. If RV has progressed to affect internal organs, treatment may include steroids, biologics, and chemotherapy-type drugs, such as cyclophosphamide. No matter how advanced your symptoms are, a clinician will work with you on a treatment plan to provide relief. But even if treatment is effective, relapses may still occur. It will be important for you to continue to monitor your symptoms and overall health, even when not experiencing symptoms. Keep in touch with your doctor and let them know if new symptoms emerge.

Side effects of medications for RV

The drugs used to treat RV can be highly effective but may also cause serious side effects. You and your doctor can work together to determine the benefits of each medication versus its risks. Immunosuppressants can lower your body’s ability to fight off infections. They may also make vaccines, such as the COVID-19 vaccine, less effective. Immunosuppressants may also cause complications, such as bone loss (osteoporosis). Cyclophosphamide can cause nausea, vomiting, hair loss, and other chemotherapy-type side effects. In many instances, a doctor may prescribe additional medications to offset the side effects of RV medications.

What are the possible complications of rheumatoid vasculitis

RV can lead to serious complications. While uncommon, these complications may be the result of RV’s impact on internal organs, including the heart. They include:strokeheart attackkidney failureatherosclerosis (plaque buildup in your arteries)intestinal ischemia (restricted blood flow to your bowels)iritis (inflammation of your eyes)pericarditis (inflammation around your heart)pleurisy (inflammation around your lungs)fibrosing alveolitis (inflammation of the air sacs of your lungs)arrhythmia (irregular heartbeat)

Can I prevent or reduce my risk of rheumatoid vasculitis

Next to having long-term RA, smoking cigarettes is the most significant risk factor for RV. If you smoke, talk with a doctor about a smoking cessation program. Quitting smoking is your best way to prevent or reduce RV. It’s also very important to take your RA medications as prescribed. Talk with a doctor about changes to existing symptoms or any new symptoms. Early treatment can help reduce RV’s impact and spread.

What is the outlook for people with rheumatoid vasculitis

Thanks to improved RA treatments, RV is much less common than it was decades ago. Still, it continues to remain a potentially serious complication for RA. The most significant factor in determining your outlook is how much RV affects your internal organs. Experts associate systemic RV (meaning you have symptoms throughout your body) with a poorer outlook. Mild RV that primarily affects your fingers is easily treated and has an excellent outlook. More serious RV may be manageable with ongoing medication. But it can also cause damaging or life threatening complications. Early diagnosis and treatment, combined with not smoking, help ensure the most positive outlook for people with RV.

Takeaway

Rheumatoid vasculitis (RV) is a serious potential complication of long-term rheumatoid arthritis (RA). You’re at greatest risk if you’ve had RA for 10 years or more or if you smoke. Symptoms of this condition range from minor to life threatening. If you have RA and notice symptoms of RV, let your doctor know immediately. Early treatment can help ensure the best possible outcome. Last medically reviewed on October 24, 2022

How we vetted this article

SourcesHistoryHealthline has strict sourcing guidelines and relies on peer-reviewed studies, academic research institutions, and medical associations. We avoid using tertiary references. You can learn more about how we ensure our content is accurate and current by reading our editorial policy.Anwar MM, et al. (2019). Rheumatoid vasculitis: Is it always a late manifestation of rheumatoid arthritis?
ncbi.nlm.nih.gov/pmc/articles/PMC6827848Cojocaru M, et al. (2015). New insight into the rheumatoid vasculitis.
pubmed.ncbi.nlm.nih.gov/26402981/Khabbazi A, et al. (2019). Cigarette smoking and risk of primary systemic vasculitis: A propensity score matching analysis.
ncbi.nlm.nih.gov/pmc/articles/PMC7247086/Kishore S, et al. (2017). Rheumatoid vasculitis: A diminishing yet devastating menace.
pubmed.ncbi.nlm.nih.gov/28631066/Lee J-E, et al. (2017). A case of rheumatoid vasculitis involving hepatic artery in early rheumatoid arthritis.
ncbi.nlm.nih.gov/pmc/articles/PMC5461328/Rheumatoid arthritis. (2021).
rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Rheumatoid-ArthritisRheumatoid vasculitis. (n.d.).
vasculitisfoundation.org/education/forms/rheumatoid-vasculitis/Sacks S, et al. (2017). Can rheumatoid arthritis predate a diagnosis of rheumatoid arthritis?
ncbi.nlm.nih.gov/pmc/articles/PMC5335888/Taha ZI, et al. (2022). Rheumatoid vasculitis, an uncommon complication of non-deforming rheumatoid arthritis: A case report.
ncbi.nlm.nih.gov/pmc/articles/PMC9307864/Watts R. (2016). Rheumatoid vasculitis (RV).
vasculitis.org.uk/about-vasculitis/rheumatoid-vasculitisOur experts continually monitor the health and wellness space, and we update our articles when new information becomes available. Current Version Oct 24, 2022 By Corey Whelan Edited By A. L. Heywood Medically Reviewed By Stella Bard, MD Copy Edited By Suan Pineda Share this articleMedically reviewed by Stella Bard, MD — By Corey Whelan on October 24, 2022

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