The Vasculitis Foundation supports and empowers our community through education, awareness, and research. It is mainly characterized by inflammation of small and large joints resulting in significant pain, foundation arthritis rheumatoid, loss of function, deformities and structural joint damage. Rheumatoid vasculitis is a serious complication of long standing RA in which inflammation spreads to involve small to medium sized and rarely, large blood vessels in the body.
When inflamed, blood vessel walls become thickened and their lumen narrows down, often to the point of complete blockage. This compromises blood supply to the affected organ. Multiple organs may be affected including the skin, nerves, eye, heart, lung, brain, gastrointestinal tract or kidney. It can be life threatening in some circumstances where appropriate treatment is not initiated promptly. Research shows that since the advent of effective drug therapy for RA, fewer people are developing rheumatoid vasculitis in recent years.
The exact etiology of rheumatoid vasculitis is unknown. It is thought that an abnormally active immune system, starts acting against the patient’s own body, targeting blood vessels and causing vascular inflammation. Patients with rheumatoid vasculitis may have high levels of rheumatoid factor in their blood and abnormal proteins called immune complexes. Under the microscope, the inflamed blood vessels show a prominence of inflammatory cells. Rheumatoid vasculitis most often develops in patients with long-standing RA, generally more than 10-years duration.
It generally develops in patients with severe RA joint disease. For reasons that are unknown, rheumatoid vasculitis is more common in males. Up to 1 in 9 males with rheumatoid arthritis may develop vasculitis at some point in their lifetime. Research has shown that smokers are more likely to develop rheumatoid vasculitis. Some individuals with RA are genetically predisposed to developing vasculitis. Depending on the size of blood vessel and organ involved, rheumatoid vasculitis has many different clinical presentations. These are predisposed to developing infection, which can lead to gangrene or necessitate amputation.
Similar ulcers can develop when skin over the legs are affected. When severe, it can result in melting away of the sclera and permanent damage to the eye. Rarely, blood vessels in the brain, heart or abdomen can be involved to the point of narrowing or complete blockage leading to stroke, heart attack, severe abdominal pain or gastrointestinal bleeding. Rheumatoid vasculitis is often accompanied by constitutional symptoms like fevers, chills, night sweats, weight loss, fatigue and lack of energy with a general sense of feeling unwell.
There is no definitive laboratory test for rheumatoid vasculitis. Tissue biopsy is helpful is demonstrating inflammation in the blood vessels and confirms presence of vasculitis. Treatment of RV is based on the organ affected and severity of organ involvement. In mild cases like fingertip sores, pain control with local protection and prevention of infection in the goal. Optimizing RA treatment is definitely necessary.
This can be done by addition of immunosuppressant medications such as methotrexate, leflunomide or biologic response modifiers such as tumor necrosis factor inhibitors, rituximab, abatacept, and others that help control inflammation not only in the joints but the blood vessels as well. Dose and duration of corticosteroid treatment will vary based on your doctor’s judgment and expertise. When there are signs of impending organ damage to major organs and with large vessel vasculitis stronger immunosuppression is achieved by using cyclophosphamide in addition to steroids. Close monitoring of laboratory tests during treatment and regular follow up with your doctor is critical is achieving good outcomes.