Abstract
BackgroundChildhood Polyarteritis nodosa (PAN) is a systemic vasculitis with necrotizing inflammation of medium- and small-sized arteries. Disease evolution may be severe and refractory to standard treatment including prednisone, azathioprine and cyclophosphamide.Case presentationWe present the case of a young girl with severe PAN resulting in progressive ischemia and necrosis of fingers and toes. Biological work-up revealed increased acute phase reactants and interleukin-6 levels. She was only partially controlled despite high-dose corticosteroids and cyclophosphamide infusions, and eventually achieved rapid improvement and sustained remission on tocilizumab.Further, we review the current evidence of the interleukin-6-inhibitor tocilizumab for the treatment of PAN.ConclusionTocilizumab may be an efficient therapeutic option in a subset of treatment-refractory children with PAN.
Highlights
Childhood Polyarteritis nodosa (PAN) is a systemic vasculitis with necrotizing inflammation of medium- and small-sized arteries
Polyarteritis nodosa (PAN) is a rare systemic vasculitis with necrotizing inflammation of medium or small sized arteries that may start in childhood [1]
We describe the use of tocilizumab (TCZ), an interleukin-6 (IL-6) receptor inhibitor, in a little girl with severe, refractory, necrotizing PAN
Summary
Childhood Polyarteritis nodosa (PAN) is a systemic vasculitis with necrotizing inflammation of medium- and small-sized arteries. Treatment recommendations include high-dose corticosteroids and cyclophosphamide for severe disease; their long-term use may be associated with unfavourable adverse events, especially in children. We describe the use of tocilizumab (TCZ), an interleukin-6 (IL-6) receptor inhibitor, in a little girl with severe, refractory, necrotizing PAN. Therapy with high dose prednisone at 4 mg/kg/day, three immunoglobulin infusions at 5 g/kg and methotrexate at 0.2 mg/kg/week was initiated without improvement.
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