Fatal polyarteritis nodosa with gastrointestinal involvement in a child

Abstract

Polyarteritis nodosa (PAN) is a necrotizing angiitis that predominantly affects small- and medium-sized arteries. Polyarteritis nodosa occurs rarely during childhood. Boys and girls seem to be equally affected, with a peak at the age of 10 years. Rarely, severe and fatal gastrointestinal involvement is seen in PAN. Here, we report a 15-year-old boy with PAN, who had gastrointestinal involvement with multiple aneurysms of the hepatic and superior mesenteric arteries. This involvement could be demonstrated with conventional angiography and gastrointestinal bleeding scintigraphy. The progression of the symptoms and the decrease in the size of the aneurysms were noted after combination treatment with cyclophosphamide and prednisolone, but there was severe bleeding from small bowel, and it was taken under control by resection of jejunum. However, the patient died because of sepsis. In conclusion, severe gastrointestinal involvement in PAN is usually fatal despite aggressive therapy, as is the case in our patient.