Vedolizumab use for severe chronic enterocolitis in a child with Hoyeraal–Hreidarsson syndrome: A case report

We report a patient with Hoyeraal-Hreidarsson syndrome (HHS), a telomere biology disorder, who developed severe chronic enterocolitis with inflammatory bowel disease-like features. Vedolizumab led to improvement in stool frequency, reduced fecal calprotectin, and clinical remission without infectious complications. Remission was maintained with vedolizumab monotherapy for 2 years after discontinuation of budesonide and parenteral nutrition. This report highlights vedolizumab as a potentially safe therapeutic option for enterocolitis in HHS, emphasizing the value of gut-selective therapy when systemic immunosuppression is risky. Further studies are needed to establish the long-term efficacy and safety of vedolizumab in patients with telomere biology disorders.