Hepatic complications in common variable immunodeficiency (CVID)

Abstract

Primary immunodeficiencies (PIDs), also known as inborn errors of immunity (IEIs), comprise a heterogeneous group of disorders characterized by increased susceptibility to infections, autoimmunity, autoinflammatory disorders and malignancies. Common variable immunodeficiency (CVID) is the most prevalent symptomatic PID and is characterized by markedly reduced serum immunoglobulin levels and impaired vaccine-specific antibody responses. Hepatic involvement affects approximately 10% of patients and requires a high index of clinical suspicion. The spectrum of liver disease in CVID is broad, encompassing nodular regenerative hyperplasia (NRH), idiopathic non-cirrhotic portal hypertension (INCPH), viral hepatitis, autoimmune hepatitis and hepatic neoplasia. Early recognition and accurate diagnosis of hepatic manifestations are crucial for preventing disease progression and enabling timely management of complications. Therapeutic options remain limited and are directed toward the prevention and treatment of subsequent complications. Optimal patient care relies on a multidisciplinary approach integrating expertise from Allergy/immunology, Internal Medicine, and Hepatology