Polyarteritis Nodosa Resistant to Conventional Treatment in a Pediatric Patient

Objective: To report the case of a child diagnosed with polyarteritis nodosa (PAN) that was unresponsive to conventional treatment alone but improved with the addition of iloprost and bosentan to her drug regimen. Case Summary: A 3-year-old girl who had been diagnosed with PAN was referred to our hospital from another region. With conventional treatment of high doses of a corticosteroid and cyclophosphamide, her condition resolved. Six months later, our patient had a relapse that required hospital admission. In this second hospital stay, some cutaneous lesions evolved into digital necrosis. Off-label therapeutic alternatives, including a single dose (2 g/kg) of intravenous immunoglobulin (IVIG), intravenous iloprost 2 ng/kg/min over 6 h for 5 days and, approximately 4 wk later, oral bosentan 37.25 mg twice daily for 4 wk followed by 62.5 mg twice daily for 8 wk, were added to the conventional regimen to treat the serious cutaneous manifestations. Her fingers improved very slowly, and she was discharged on gradually tapered doses of oral corticosteroids, bosentan, and monthly pulsed injections of cyclophosphamide. The digital necrosis and other cutaneous lesions had resolved completely 6 months after the second discharge. Discussion: The dosages of IVIG and iloprost were based on those used for PAN, Raynaud's phenomenon, and digital necrosis in children. The use of bosentan for vasculitis had not been reported in children before the treatment of our patient, so its dosage was based on that used to produce vasodilation in children with pulmonary hypertension. Conclusions: Digital necrosis and cutaneous manifestations not resolved with conventional PAN treatment improved within 5 days with iloprost and 12 weeks with bosentan.