Rheumatoid vasculitis is a rare but serious complication of rheumatoid arthritis which occurs in 1-5% of patients diagnosed with RA (Genta 2006). In rheumatoid vasculitis, inflammation of small and medium-sized blood vessels in the body restricts blood flow and causes tissue damage. Multiple organs may be affected including the skin, nerves, eyes, heart, lung, brain, gastrointestinal tract or kidneys. It most commonly affects the skin, arteries of the fingers and toes, peripheral nervous system, and eyes causing scleritis (an inflammation of the white part of the eye).
Who develops rheumatoid vasculitis?
Vasculitis in RA is associated with longstanding (more than 10 years), erosive, seropositive disease. It is more common in men than women with RA and in patients who smoke. People who develop rheumatoid vasculitis usually have elevated sedimentation rate, rheumatoid factor, C-reactive protein, and anti-tissue antibodies (eg. anti-cyclic citrullinated polypeptides (anti-CCPs), antinuclear antibodies). Patients with rheumatoid vasculitis will also often have rheumatoid nodules.
Clinical reports have estimated the prevalence of RA vasculitis at less than 1% to 5% (Bartels 2009), whereas autopsy studies have reported 15% to 31% (Genta 2006). However, it appears that rheumatoid vasculitis has been on the decline since the introduction of biologic therapies to treat RA (Bartels 2010).
How is rheumatoid vasculitis diagnosed?
Your rheumatologist may suspect rheumatoid vasculitis based on symptoms of rash, skin ulcers, numbness or tingling of the hands or feet, weakness in a hand or foot, cough and shortness of breath, chest pain, abdominal pain, or stroke symptoms.
Although patients diagnosed with rheumatoid vasculitis often have elevated lab results, there is no definitive test for the disease short of a tissue biopsy. The diagnosis is usually made with a combination of patient medical history and symptoms, physical examination, pertinent laboratory tests, specialized tests (such as nerve conduction studies), and tissue biopsy of involved skin, muscle, nerves, or other affected organ.
Because of the lack of specific signs and symptoms, the diagnosis relies on the exclusion of other causes of similar lesions (diabetes, atherosclerosis, drug reactions, infection, neoplasias) and, ideally, on the examination of tissue samples that reveal necrotizing vasculitis.
Persons with RA who develop new extra-articular symptoms such as skin ulcerations, decreased blood flow to the fingers or toes, sensory or motor nerve dysfunction (such as numbness, tingling, weakness), inflammation of the lining of the heart or lungs, or even weight loss or fever, should be evaluated for rheumatoid vasculitis. Early detection and treatment are necessary to prevent or limit permanent damage.
How is rheumatoid vasculitis treated?
Vasculitis is an inflammatory disease that often requires treatment with immunosuppressive drugs, such as glucocorticoids. Choice of treatment depends upon the severity and degree of organ system involvement.
Mild rheumatoid vasculitis involving the skin or peripheral nerves is typically treated with prednisone and methotrexate or azathioprine. More serious organ system involvement may require treatment with high-dose intravenous steroids and a cytotoxic or biologic agent (Bartels 2010). However, there have been no controlled studies which can be used to guide treatment decisions.
Patients with rheumatoid vasculitis have an increased risk of comorbidities, particularly cardiovascular disease. Thus patients should be monitored for traditional risk factors such as hypertension, smoking, diabetes, hyperlipidemia (high cholesterol), and obesity, and the threshold for treatment may need to be lower than for the general population (Turesson 2009).
What is the prognosis of RV?
The morbidity and mortality of rheumatoid vasculitis are substantial. Less common involvement of the heart, bowel, or kidney can lead to heart attack, bowel ischemia (restricted blood flow), and renal failure. Studies have shown that the 5-year mortality rate is 30% to 50%, with even higher rates of morbidity related to disease complications or vasculitis treatment-related toxicity.
Early and accurate diagnosis is imperative to choosing the most appropriate treatment to control symptoms and prevent damage.
Bartels CM, Bridges AJ. Rheumatoid vasculitis: vanishing menace or target for new treatments? Curr Rheumatol Rep. 2010 Dec;12(6):414-9. doi: 10.1007/s11926-010-0130-1.
Bartels C, Bell C, Rosenthal A, Shinki K, Bridges A. Decline in Rheumatoid Vasculitis Prevalence among US Veterans: A retrospective cross-sectional study. Arthritis Rheum. 2009 Sep;60(9): 2553-7. doi:10.1002/art.24775.
Genta MS, Genta RM, Gabay C. Systemic rheumatoid vasculitis: a review. Semin Arthritis Rheum. 2006 Oct;36(2):88-98.
Turesson C, Matteson EL. Vasculitis in Rheumatoid Arthritis. Curr Opin Rheumatol. 2009;21(1):35-40.
"Rheumatoid Vasculitis." The Johns Hopkins Vasculitis Center. Accessed at https://www.hopkinsvasculitis.org/types-vasculitis/rheumatoid-vasculitis/ (includes photos of lesions)
"Vasculitis." American College of Rheumatology. Accessed at https://www.rheumatology.org/Practice/Clinical/Patients/Diseases_And_Conditions/Vasculitis/
Lisa Emrich is a patient advocate, accomplished speaker, author of the award-winning blog Brass and Ivory: Life with MS and RA, and founder of the Carnival of MS Bloggers. Lisa uses her experience to educate patients, raise disease awareness, encourage self-advocacy, and support patient-centered research. Lisa frequently works with non-profit organizations and has brought the patient voice to health care conferences and meetings worldwide. Follow Lisa on Facebook, Twitter, and Pinterest.