D POTTER1, S BAWEJA2, W JAMES2,
1Liverpool Hospital, Liverpool, New South Wales; 2Lismore Hospital, Lismore, New South Wales
Background: Granulomatosis with Polyangitis (GPA) is a chronic multisystem condition primarily affecting the upper airway, lung and kidney. It is characterised by necrotising vasculitis of small to medium arteries with granulomatous inflammation. Gastrointestinal GPA has been noted in up to 24% of autopsy series but reported clinical manifestations are rare. Ileum and colon the primary affected areas often presenting as acute perforation and bleeding. In a case series of six patients none had jejunal involvement and in another of thirteen patients only two had jejunal pathology.
Case Report: A 40 year old male presented with two weeks of colicky epigastric pain without vomiting, altered bowel habit or malaena. Clinical examination revealed a tender abdomen without peritonism. He had renal failure with a creatinine of 255umol/L. Eight years prior he was treated for necrotising glomerulonephritis due to GPA with prednisolone and cyclophosphamide but subsequently was lost to follow up. Repeat renal biopsy demonstrated fibroepithelial crescents, an interstitial inflammatory cell infiltrate and widespread global and segmental glomerulosclerosis. Contrast tomography of the abdomen demonstrated thickening of proximal bowel loops and haziness to upper abdominal fat planes. Endoscopy revealed two jejunal ulcers without perforation. Biopsy demonstrated ill-defined granuloma formation with focal evidence of ischaemia. He received steroids for jejunitis secondary to GPA with an excellent response.
Conclusions: Gastrointestinal GPA is rare and limited primarily to descriptions of acute ileal and colonic abnormalities. Our case had subtle symptoms without major gastrointestinal signs at remote time from his diagnosis, suggesting gastrointestinal GPA may more common than in the literature and at risk for under evaluation. Gastrointestinal complications of GPA are important as delayed diagnosis may lead to fatal outcomes.