A Girl with End-Stage Kidney Disease Due to Granulomatosis with Polyangiitis Presenting with Gastrointestinal Bleeding “a rare presentation”

Abstract

Granulomatosis with polyangiitis (GPA) is an uncommon vasculitis affecting small- and medium-sized vessels, predominantly in adults (average age approximately 45 years) and infrequently in children. It can involve multiple organ systems. 
In patients with end-stage kidney disease (ESKD) due to GPA, disease activity is often tends to wane after the commencement of dialysis.
We report a 12-year-old girl with ESKD due to GPA who presented with severe gastrointestinal (GI) bleeding and profound anemia, owing to diffuse microaneurysms in the small bowel (particularly the ileum) and colon—an exceedingly rare manifestation. She responded dramatically to induction therapy comprising methylprednisolone pulse, plasmapheresis, rituximab followed by maintenance therapy with prednisolone and mycophenolate mofetil. 
This case highlights that while the recurrence risk of ANCA-associated vasculitis in dialysis patients is low, the possibility remains and may present with atypical extrarenal involvement. Given the elevated risk of infection with immunosuppression in ESKD patients, maintenance immunosuppressive therapy may be discontinued in the absence of extrarenal disease—but this decision necessitates careful consideration.