Severe intestinal involvement as initial manifestation of systemic childhood polyarteritis nodosa: report of two...

Area of Science:

• Pediatric Rheumatology
• Vascular Biology
• Gastroenterology

Background:

• Systemic childhood polyarteritis nodosa (C-PAN) is a rare vasculitis affecting medium and small arteries.
• Abdominal angina, post-prandial pain due to mesenteric ischemia, is a known complication of polyarteritis nodosa (PAN), typically in adults.
• The presentation of abdominal angina as the initial manifestation of C-PAN has not been previously described.

Observation:

• Two young boys presented with malignant arterial hypertension and recurrent, severe post-prandial cramping mimicking an acute abdomen.
• Laparotomy revealed extensive intestinal necrosis in both patients; one also had multiple intestinal perforations.
• Despite aggressive treatment including antihypertensives, immunosuppressants, and IV immunoglobulin, both patients died due to disseminated disease activity.

Findings:

• This report details the first two fatal cases of C-PAN where severe abdominal pain was the primary presenting symptom.
• The findings underscore that abdominal angina can be the initial, critical manifestation of C-PAN in pediatric patients.
• The cases highlight the aggressive nature and potential lethality of C-PAN when presenting with gastrointestinal complications.

Implications:

• The diagnosis of PAN should be considered in pediatric patients presenting with acute abdominal angina of unknown etiology.
• Early recognition and diagnosis of C-PAN presenting with abdominal symptoms are crucial for timely intervention.
• This case series expands the understanding of C-PAN's clinical spectrum and initial presentations in children.