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Exploring ANCA Pathogenicity in Ulcerative Colitis: A Case Report Highlighting the Risk of Progression to
Abir Hamid1, Frederic Vandergheynst1, Maxime Ilzkovitz1
1Internal Medicine Department, Hôpital Erasme, Hôpital Universitaire de Bruxelles (HUB), Université Libre de Bruxelles (ULB), Brussels, Belgium.
Background:
The relationship between anti-neutrophil cytoplasmic antibodies (ANCA) and inflammatory bowel disease has attracted significant attention due to shared immunopathological mechanisms and clinical associations.
Case Description:
This case report describes a 20-year-old African woman with a history of ulcerative colitis who developed diffuse alveolar haemorrhage in the setting of elevated proteinase 3 (PR3)-ANCA. She was treated for ANCA-associated vasculitis with corticosteroids, plasma exchange, and rituximab, leading to a complete resolution.
Conclusion:
The case highlights the association between ulcerative colitis and ANCA-associated vasculitis, particularly with PR3-ANCA, and the importance of recognizing this overlap. Although ANCA are frequently present in inflammatory bowel disease, especially ulcerative colitis, their pathogenic role remains unclear. The incidence of ANCA-associated vasculitis is higher in inflammatory bowel disease patients, with ulcerative colitis often preceding ANCA-associated vasculitis. Despite the common presence of ANCA in inflammatory bowel disease, not all patients progress to ANCA-associated vasculitis. This case underscores the need for careful monitoring in ulcerative colitis patients with elevated PR3-ANCA and the role of ANCA in guiding diagnosis and treatment.
Learning Points:
High proteinase 3 (PR3)-anti-neutrophil cytoplasmic antibodies (ANCA) titre impacts the severity and management of ulcerative colitis patients.ANCA-associated vasculitis is more frequent in ulcerative colitis patients and occurs several years after ANCA positivity.Close monitoring of ulcerative colitis patients with PR3-ANCA is essential for early detection of progression to ANCA-associated vasculitis.
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