Dados do Trabalho

Title

REFRACTORY POLYARTERITIS NODOSA SUCCESSFULLY TREATED WITH TOCILIZUMAB: A CASE REPORT

Background

Polyarteritis nodosa is a systemic vasculitis that affects medium-sized vessels, which can be lethal. It can affect almost any system in the human body, although skin, muscles, joints, kidneys, nerves, gastrointestinal tract, and heart are the most common sites of involvement. Its treatment should be tailored according to the clinical manifestations, corticosteroids, methotrexate, azathioprine, mofetil mycophenolate, and cyclophosphamide are among the options. There are sparse treatment options for refractory cases, with only a few reported cases of successful use of rituximab, tocilizumab and anti-TNF drugs.
Herein we report the successful use of tocilizumab in the treatment of a refractory PAN.

Case report

A 22-year-old Caucasian man was admitted because of an acute episode of fever, arthralgia, vasculitic lesions (figure 1 and 2), skin ulcers, and paresthesia. He has received a diagnosis of PAN at the age of six. At that time, he presented migratory arthralgia, myalgia, erythema nodosum, hematuria, altered renal function, and necrosis of hand digits. He was treated with methylprednisolone, cyclophosphamide, IGIV, and hyperbaric oxygen; and remained asymptomatic since then.
The new vasculitis flare was treated with pulses of methylprednisolone, followed by prednisone 2 mg/Kd/day, IgIV and cyclophosphamide. This treatment resulted in amelioration of the manifestations but attempting to reduce the corticosteroids dose resulted in relapses. Tocilizumab 8 mg/Kg/dose was initiated, resulting in complete clinical and laboratory remission and allowing corticosteroids withdrawal. Interestingly, there was relapsing of the vasculitis manifestations when, due to lack of supply, treatment with tocilizumab was delayed.

Conclusion

Tocilizumab could be an option for the treatment of refractory PAN. Double-blinded clinical trials are warranted to evaluate its efficacy and safety in the treatment of this disease.

Arquivos

Área

Vasculitis