Abstract
BackgroundPolyarteritis nodosa is a disease that presents with necrotizing vasculitis in small and medium-sized arteries. It may occur in various organs, but approximately half of cases have gastrointestinal involvement. Prognosis is not favorable once organ dysfunction begins as evidenced by gastrointestinal symptoms; thus, treatment with steroids should be promptly initiated. We report the case of a patient who presented with necrosis of the small intestine, which was pathologically diagnosed as polyarteritis nodosa and treated successfully with steroids.Case presentationAn 18-year-old Japanese woman reported a sudden onset of abdominal pain and vomiting that led her to visit our emergency department, where she was evaluated by a physician. On physical examination, tenderness to palpation in the upper umbilical region was noted, and diagnostic imaging with computed tomography showed emphysema of the wall of her small intestine. She was diagnosed as having necrosis of the small intestine requiring urgent surgery. No strangulations were noted intraoperatively but approximately 20 cm of her small intestine was necrotized. The surrounding arteries were examined and no palpable pulse was observed; therefore, segmentectomy of the necrotized regions was performed. Pathological findings revealed active vasculitis with fibrinoid necrosis, as well as destruction, fibrogenesis, and luminal stenosis of the elastic lamina found in the muscular arteries. A diagnosis of polyarteritis nodosa was confirmed as the cause of the necrosis of her small intestine. No recurrence of polyarteritis nodosa symptoms was observed when she was administered 40 mg of prednisolone daily.ConclusionIn cases of idiopathic intestinal necrosis or perforation, systemic diseases such as polyarteritis nodosa should be considered in the differential diagnosis.
Highlights
Polyarteritis nodosa is a disease that presents with necrotizing vasculitis in small and medium-sized arteries
We report a case who presented with necrosis of the small intestine, which was pathologically diagnosed as Polyarteritis nodosa (PAN) and treated successfully with steroids
In this case, emergency surgery was performed based on a diagnosis of necrosis of the small intestine, caused by an internal hernia or strangulated intestinal obstruction, and PAN was diagnosed pathologically after the operation
Summary
Polyarteritis nodosa (PAN) is a condition involving necrotizing vasculitis in small and medium-sized arteries [1]. Case presentation An 18-year-old Japanese woman experienced a sudden onset of abdominal pain and vomiting, and was transported to the out-patient emergency ward at our institution She was a student with no employment history. Her consciousness was normal and there were no neurological abnormalities She had been receiving antihistamines for atopic dermatitis but had no other conditions, such as neuropathy or hematuria, which would lead us to suspect neuritis or vasculitis. Palpation revealed a flat abdomen with tenderness in the upper umbilical region, and no signs of peritoneal irritation Her temperature was 36.3 °C, blood pressure was 159/123 mmHg, pulse was 85 beats/minute, and oxygen saturation was 99% on room air.
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