An unusual case of pediatric granulomatosis with polyangiitis complicated by splenic infarction presenting as inflammatory bowel disease.

ABSTRACT

We describe a case of granulomatosis with polyangiitis (GPA) in a 7-year-old-male who initially presented with symptoms concerning for Inflammatory bowel disease. GPA is a rare, multisystemic necrotizing vasculitis involving small arteries and veins. The clinical presentation can be variable given its multisystemic involvement but more commonly involves the upper and lower airways and kidneys. This case highlights rare gastrointestinal symptoms of GPA, further complicated by an additional unique finding of splenic infarction. We hope to raise awareness for this rare illness to assist in diagnosis and treatment, as timely induction of remission can reduce significant morbidity and mortality in the pediatric population.