Long-term outcomes in patients with inborn errors of immunity

Abstract

Inborn Errors of Immunity (IEI), formerly known as primary immunodeficiencies, represent a heterogeneous group of genetic disorders that impair the immune system's ability to combat infections and regulate immune responses. With advances in diagnosis and treatment, many patients with IEI now survive into adulthood. However, the long-term outcomes of these patients are influenced by various factors, including the type and severity of the immune defect, the effectiveness of treatment, and the management of complications. This review provides a comprehensive analysis of the long-term outcomes in patients with inborn errors of immunity, based on recent studies and clinical data. We discuss the challenges these patients face, including chronic infections, autoimmunity, malignancies, and quality of life issues, and explore strategies for improving long-term care and prognosis.