A 59-Year-Old Woman With a Painful Rash and Fingertip Ulcerations

Victoria M.F. Mank, MD; Jefferson R. Roberts, MD

Disclosures

October 11, 2019

Rheumatoid vasculitis can involve the nervous system, arteries, and sclera among other systems. Digital ischemia and gangrene due to involvement of the arteries of the distal extremities was a manifestation of this patient's rheumatoid vasculitis. The patient's initial pruritic lesions, for which she presented to the clinic years prior to her hospitalization for osteomyelitis, were likely a cutaneous vasculitis due to obstruction of smaller-sized superficial vessels.

Rheumatoid vasculitis tends to occur in smokers who have erosive RA and subcutaneous nodules and who have had the disease longer than 10 years. It is more common in men and is associated with high concentrations of rheumatoid factor.[5] The patient was encouraged to stop smoking to prevent further lesions and vasculitis progression, but she declined assistance with quitting her nicotine dependence and continued to smoke cigarettes.

Rheumatoid vasculitis must be differentiated from drug-induced vasculitis, the most common form of vasculitis.[6] Typically, patients with this type of vasculitis recover well when the offending agent is removed. This was not the case with the patient presented here, as she was frequently not taking any of her medications. Treatment is focused on the underlying RA. Rheumatoid vasculitis can be treated similarly to other forms of primary systemic vasculitis with high-dose steroids and biologic agents or DMARDs.

The mainstay of treatment is controlling the disease process and attempting to reduce the autoantibody response. Prior to beginning therapy, assessing for viral and bacterial infections and other causes that may be mimicking RA is important. These include HIV, hepatitis, tuberculosis, endocarditis, and drugs.[7] A careful history and physical examination plus simple laboratory tests are generally sufficient to exclude these disorders. Corticosteroids help in reducing the inflammatory response associated with RA and can be used by themselves in patients with mild disease. Mild disease in relation to rheumatoid vasculitis usually involves small lesions to distal extremities (fingers and toes). For more severe and widespread vasculitis, as in this patient, immunosuppressants and corticosteroids are used in combination to control the disease process. Biologic agents that specifically target tumor necrosis factor can be used to decrease the inflammatory response that is associated with endothelial dysfunction leading to vasculitis and is a potential medication choice.[8] Cyclophosphamide, rituximab, leflunomide, methotrexate, and other biologic agents are other options to treat severe rheumatoid vasculitis.

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