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.

Mycophenolate mofetil treatment in two children with severe polyarteritis nodosa refractory to immunosuppressant drugs

“…The proper diagnosis was delayed as the initial neurological disease was not regarded as brain ischemia and cutaneous features in the boy were misdiagnosed as HSP. In several cases, the disease may be fatal as visceral involvement is aggressive [10][11][12][13][14]. Recently, tumor necrosis factor alpha blockade has been proposed in patients refractory to other therapies [15].…”

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“…Some has severe gastrointestinal hemorrhage and sepsis. End stage renal disease is also observed in patient with PAN [21].…”

Polyarteritis nodosa presenting as profuse gastrointestinal bleeding

“…The presence of profuse GI bleeding as the sole manifestation at presentation in PAN is under-reported and not widely known in clinical practice 5 6. Perez et al 7 reported the first case of GI bleeding at presentation in a PAN case in 2000.…”