Common variable immunodeficiency (CVID) is one of the most prevalent primary immunodeficiency disorders, characterized by an alteration in the maturation of B lymphocytes. Patients with this condition are redisposed to a higher risk of infections. Despite being an immune deficiency disorder, the prevalence of autoimmune disorders is reported in more than 20% of patients. The likelihood of patients’ gastrointestinal tract being affected is relatively low, close to 6%. We present the case of a 22-year-old man with a history of CVID without medical treatment, who presented with upper gastrointestinal bleeding secondary to esophageal varices due to cirrhotic portal hypertension. Infectious and toxic causes of cirrhosis were ruled out. Histological changes compatible with autoimmune hepatitis (AIH) were documented by liver biopsy. The diagnosis of autoimmune diseases is a challenge in the presence of IDCV, we highlight the importance of establishing a timely diagnosis and an intentional search for these conditions to offer timely treatment and avoid late complications.
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