<p>Recurrent ruptured abdominal aneurysms in polyarteritis nodosa successfully treated with infliximab</p>

Abstract

Systemic polyarteritis nodosa (PAN) is a rare form of necrotizing vasculitis in children. Recurrent episodes of abdominal aneurysm ruptures are uncommon and life-threatening condition in children. Failures of response to immunosuppressive medications and radiological intervention also lead to high mortality. Some reports suggested that tumor necrosis factor (TNF) might have role in the inflammation of this disease. After an English-language literature review, this is the first case report in children of recurrent abdominal-ruptured aneurysms with a failure of conventional therapy but successfully treated with anti-TNF-α monoclonal antibody. We herein describe a 9-year-old girl who presented with chronic abdominal pain, hypertension, and massive lower gastrointestinal bleeding. The disease was refractory to conventional treatment, including administration of a corticosteroid, cyclophosphamide, and intravenous immunoglobulin, and recurrent-ruptured aneurysms developed in the gastrointestinal tract. Arterial embolization during angiography resulted in temporary improvement of the gastrointestinal bleeding. Infliximab, a chimeric anti-tumor necrosis factor-α monoclonal antibody, was initiated and resulted in disease remission with resolution of the gastrointestinal bleeding and abdominal pain. Anti-TNF therapy might be another treatment option for refractory disease to prevent ongoing inflammation that could lead to aneurysmal dilatation or even rupture. However, early recognition of refractory disease and aggressive treatment in the early course of the disease are crucial to reduce morbidity and mortality.

Gastrointestinal involvement in childhood vasculitides

“…Her disease resisted conventional treatment, including steroids, cyclophosphamide and intravenous immunoglobulins, and she developed recurrent‐ruptured aneurysms in the GI tract. After a temporary improvement, resulting from arterial embolisation during angiography, infliximab was initiated and the disease remitted 36 …”

Polyarteritis Nodosa with Cytomegalovirus Enteritis and Jejunoileal Perforation: Report of a Case with a Literature Review

“…A review of 11 reports showed that severe, life-threatening GI involvement was found as small intestinal stricture, enteritis, colitis, ischemic necrosis, and its complications, such as obstruction, perforation, peritonitis, and mesenteric infarction. 21 , [23][24][25][26][27][28][29][30][31][32] These pediatric patients with PAN who presented with aneurysms with or without rupture are shown in Table 1.…”

Endovascular Biopsy of Vertebrobasilar Aneurysm in Patient With Polyarteritis Nodosa

“…These results, specific to this patient, differ from other studies of bulk RNA sequencing on intracranial aneurysm tissue that implicates B-lymphocytes in aneurysm rupture ( 35 ). Future studies will be needed to demonstrate the causation and disease modification with the immunomodulating therapies, such as with the anti-TNFα monoclonal antibody infliximab, that has already shown promise in the treatment of extracranial aneurysms for the patients with polyarteritis nodosa ( 36 – 38 ).…”