Successful management with Tocilizumab in refractory systemic polyarteritis nodosa: Pediatric case report

Abstract

Polyarteritis nodosa (PAN) is a necrotizing vasculitis of medium and small vessels. The incidence in adults is 2-9 per million population, in children data are scarce. Although rare in childhood it is the most common form of vasculitis after IgA vasculitis and Kawasaki disease.

PAN can affect almost any organ. Cutaneous, musculoskeletal and gastrointestinal involvement are more common in children and more rarely cardiac, neurologic and respiratory symptoms are seen. The disease course can be severe and refractory to standard treatment, which includes prednisone, azathioprine and cyclophosphamide.

The aim of this article is to present the case of a girl who debuted at age 7 years with systemic PAN, with intermittent fever and pain in the inguinal region and leg with a history of ankle synovitis, admitted to our hospital with VAS (visual analog scale) pain 10/10, elevated acute phase reactants, Computerized Tomography Angiography (CTA) reported multianeurysmal disease, and rupture of the left external iliac artery. She received monthly corticosteroids and cyclophosphamide, and 2 months after treatment she presented again with pain in the left iliac fossa, myalgia, synovitis and erythema nodosum. Laboratory tests showed an increase in C Reactive Protein (CRP), and CT scan showed an increase in the iliac aneurysm. Intravenous tocilizumab was started, and she has remained stable and in remission. Tocilizumab is an alternative in refractory treatment.