Severe intestinal involvement as initial manifestation of systemic childhood polyarteritis nodosa: Report of two cases

Abstract

Systemic childhood polyarteritis nodosa (C-PAN) is a rare primary vasculitis involving medium or small sized arteries. Abdominal angina is an important and serious complication of PAN, occurring usually 15 to 30 min after food intake, and particularly in adult patients. However, to our knowledge, this involvement as the first manifestation of C-PAN was not described. Therefore, we reported herein two C-PAN cases that fulfilled the new criteria for this vasculitis. These patients were young boys that had malignant arterial hypertension and recurrent post-prandial cramping with acute abdomen. Both of them were submitted to laparotomy that revealed multiple and diffuse intestinal necrosis. One of our cases had a severe post-prandial cramping, even after drinking water, and the laparotomy evidenced multiple intestinal perforations. In spite of use of antihypertensive therapies, immunosuppressive agents (corticosteroids, cyclophosphamide and/or methotrexate) and intravenous immunoglobulin, they died possibly due to severe and disseminated activity disease. In conclusion, we described herein the first two fatal cases of C-PAN that presented severe abdominal pain as initial manifestation. We suggest that the diagnosis of PAN should be considered in patients under acute abdominal angina with no apparent etiology.