Granulomatosis with polyangiitis (GPA) is a small-vessel necrotizing granulomatous vasculitis typically involving upper airways, lungs, and kidneys, which may lead to end-organ damage and life-threatening complications. Major infections during GPA course represent a considerable concern in the management of the disease. Cytomegalovirus (CMV) infection and disease are rare but significant complications in the course of GPA being associated with high morbidity and mortality rates. Colonic perforation due to CMV colitis is exceedingly rare and has so far almost exclusively been documented in HIV, renal transplant, and systemic lupus erythematosus patients. We reported the case of a patient affected with upper airways-limited GPA who developed acute renal failure from rapidly progressive glomerulonephritis and then experienced colonic perforation due to CMV colitis a few weeks after immunosuppressive treatment with high-dose steroids and cyclophosphamide (CYC) for remission induction of the disease. We also reviewed the literature on CMV-related gastro-intestinal complications in the course of GPA and discussed contributing factors to severe manifestations of CMV infection and its reactivation.
Keywords: ANCA-associated vasculitis (AAV); Colonic perforation; Cytomegalovirus (CMV); Granulomatosis with polyangiitis (GPA); Immunosuppression.