Case Presentation: We present a 79-year-old woman with a longstanding history of seropositive, erosive, and nodular rheumatoid arthritis. Her rheumatoid arthritis was diagnosed over 40 years prior and had been complicated by Large Granular Lymphocytic (LGL) Leukemia and Felty syndrome with confirmed splenomegaly and neutropenia. She was originally treated with filgrastim for severe neutropenia. About nine months before hospitalization, the patient began to develop a small lower extremity ulcer that grew rapidly in size. She underwent a skin biopsy which was consistent with leukocytoclastic vasculitis and was subsequently treated with wound cares. She presented to the hospital due to concerns of ulcer enlargement and cellulitis. On exam, she had classic advanced sequelae of rheumatoid arthritis including ulnar deviation, metacarpophalangeal joint subluxations, and reduced range of motion. However, she had no active synovitis. Laboratory workup was significant for a WBC of 1.3 and absolute neutrophil count (ANC) of 0.7. There was no evidence of osteomyelitis on imaging. Filgrastim was discontinued because it could potentially worsen her underlying vasculitis. Due to the patient’s LGL leukemia and Felty syndrome with resulting leukopenia and neutropenia, there were concerns of initiating immunosuppressive agents given elevated risk of devastating infection. After multiple discussions with infectious disease, hematology, and the patient, rituximab was initiated prior to discharge as it was felt that the risk of worsening ulceration leading to potential amputation outweighed the risk of infection.
Discussion: Rheumatoid arthritis is a systemic disease with extra-articular manifestations that contribute to significant morbidity and mortality. Rheumatoid vasculitis is an increasingly rare form of extra-articular disease with a significant decline in hospitalizations in the past few decades theorized to be due to advances in rheumatoid arthritis treatments. The vasculitis is typically cutaneous, as in this case, but it can involve any organ and is one of the most serious rheumatoid arthritis complications with an estimated 30-50% five-year mortality. Treatment with biologic agents has demonstrated a 70% complete remission rate, however, there is a 34% adverse side effect rate mainly due to infection. This case was an interesting dilemma. On the one hand, she had existing LGL leukemia resulting in an immunocompromised status. On the other hand, she had the urgent need for immunosuppressant agents for treatment of her underlying vasculitis given the rapidly enlarging ulcer. She was additionally receiving G-CSF injections for years prior to presentation. Interestingly, vasculitis has previously been associated with the initiation of G-CSF injections, however, most cases have been described within the first 1-15 days after initiating G-CSF. Due to an abundance of caution, we discontinued G-CSF during hospitalization. The patient was initiated on rituximab prior to discharge with prior studies supporting its effectiveness in rheumatoid vasculitis, however, infection remains an active concern.
Conclusions: There has been a sharp decrease in the incidence of rheumatoid vasculitis, a rare and serious complication of rheumatoid vasculitis, in recent years due to the advent of effective therapy. Thorough discussion of risks should be discussed with the patient before standard immunosuppressive treatment is initiated.