What Is Rheumatoid Vasculitis?

A Complication of Rheumatoid Arthritis

Patient with numbness in hand.
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Rheumatoid vasculitis is a rare, serious complication of rheumatoid arthritis which can potentially be life-threatening. In rheumatoid vasculitis, inflammation spreads to small or medium size blood vessels in the body. Usually arteries are involved, but veins can be as well. Large blood vessels are rarely involved.

The walls of inflamed blood vessels become thickened and as the lumen of the affected blood vessel narrows, it may become nearly blocked.

The blood supply to an organ may then be compromised, if not impeded. Rheumatoid vasculitis may involve multiple organs, including the skin, eyes, nerves, heart, lung, brain, kidney, or gastrointestinal tract. But, there is good news as well. Fewer rheumatoid arthritis patients are developing rheumatoid vasculitis, likely due to more effective drugs which were developed and marketed in the past decade or so (i.e., the biologic drugs).

Prevalence and Risk of Rheumatoid Vasculitis

While the prevalence of rheumatoid vasculitis appears to be decreasing, it is estimated that less than one to 5% of the rheumatoid arthritis patient population develops rheumatoid vasculitis. However, autopsy studies have reported 15-31%. Morbidity and mortality rates associated with rheumatoid vasculitis are significant with the 5-year mortality rate at 30-50% with even greater morbidity rates tied to disease complications or treatment toxicity.

The following factors increase the risk of developing rheumatoid vasculitis:

  • Positive for rheumatoid factor
  • Positive for anti-CCP
  • Male (1 in 9 males with rheumatoid arthritis develop rheumatoid vasculitis)
  • Smoking
  • Presence of rheumatoid nodules
  • Older at disease onset or long duration of rheumatoid arthritis (over 10 years)

    Symptoms and Manifestations of Rheumatoid Vasculitis

    Any organ of the body can be affected by rheumatoid vasculitis. The skin and peripheral nerves are most commonly involved. While major organ involvement is considered less common, it is associated with significant morbidity and mortality.

    Skin involvement may include purpura, nodules, ulcers, and necrosis of the digits, especially the fingertips. Livedo reticularis is a common finding. Skin involvement calls for further investigation of other organ involvement. Scleritis, pleuritis, or pericarditis are examples of what may develop. If skin is involved without other systemic involvement, the prognosis is better.

    With the peripheral nervous system, peripheral neuropathies may develop, such as distal symmetric sensory polyneuropathy, distal motor or combined neuropathy, or mononeuritis multiplex. When vasculitis damages the nerves. there can be numbness, tingling, loss of sensation, weakness, and loss of function in the hands or feet. More generalized symptoms can also occur, such as fever, weight loss, loss of appetite, and loss of energy.

     

    Diagnosis of Rheumatoid Vasculitis

    While a pattern of symptoms may cause a doctor to suspect rheumatoid vasculitis and laboratory tests may provide supporting evidence for a probable diagnosis, a biopsy is required for a definitive diagnosis. Involved skin may be biopsied, as well as a muscle or nerve within an affected region, or an affected organ. 

    While blood tests for rheumatoid factor and anti-CCP are typically elevated in rheumatoid vasculitis, results are not specific. The two antibodies are also prevalent in rheumatoid arthritis without vasculitis. Anti-neutrophil cytoplasmic antibodies (ANCA) and associated anti-myeloperoxidase and anti-proteinase-3 antibodies are typically negative in rheumatoid vasculitis.

    Treatment of Rheumatoid Vasculitis

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

    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. Rituxan (rituximab) is emerging as a first line therapy for rheumatoid vasculitis. According to a report in Arthritis Care and Research (2012), an analysis of data from the Autoimmunity and Rituximab registry revealed that remission was achieved in 12 of 17 rheumatoid vasculitis patients treated with rituximab. The patients were also able to decrease their dose of prednisone. 

    Sources:

    Rheumatoid Vasculitis. Johns Hopkins Vasculitis Center. Accessed 10/09/15.
    http://www.hopkinsvasculitis.org/types-vasculitis/rheumatoid-vasculitis/

    Rheumatoid Vasculitis. Cleveland Clinic. Accessed 10/09/15.
    http://my.clevelandclinic.org/services/orthopaedics-rheumatology/diseases-conditions/hic-rheumatoid-vasculitis

    Rheumatoid Vasculitis. Vasculitis Foundation. September 2012.
    http://www.vasculitisfoundation.org/education/forms/rheumatoid-vasculitis/

    Rheumatoid Vasculitis: Vanishing Menace or Target for New Treatments? Bartels and Bridges. Current Rheumatology Reports. December 2010.
    http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2950222/

    Rituximab therapy for systemic vasculitis associated with rheumatoid arthritis: Results from the Autoimmunity and Rituximab Registry. Puechal X et al. Arthritis Care and Research. March 2012.
    http://www.ncbi.nlm.nih.gov/pubmed/22076726

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