At least 10% of patients with common variable immunodeficiency I (CVID) have liver involvement. Nodular regenerative hyperplasia (RNH) is a common lesion seen in the liver of CVID patients and can lead to chronic cholestasis, non-cirrhotic portal hypertension, or liver cirrhosis. Patients with liver involvement may remain asymptomatic or present with fatigue, nausea, vomiting, jaundice, pruritus, ascites, edema, hepatomegaly, splenomegaly, and esophageal varices. Clinically, some patients with CVID have been diagnosed with chronic hepatitis, autoimmune hepatitis, primary sclerosing cholangitis, hepatopulmonary syndrome secondary to cryptogenic liver disease, portal hypertension, liver cirrhosis, and even liver failure. When liver abnormalities are present, the underlying causes should be investigated. Possible causes of liver abnormalities include HCV, HBV, and HIV infections, autoimmune reactions, lymphoproliferation, malignancies, iron, copper, and fat deposition, alcohol, drug, and toxin intake. A liver biopsy should be performed in selected cases to define the etiology.
An intentional search for alterations at the liver function in outpatients with a diagnosis of CVID of the gastroenterology service. Of the total number of patients recruited with a history of common variable immunodeficiency, 75% met the criteria for nodular regenerative hyperplasia, 100% had increased alkaline phosphatase, and 50% had abnormal liver enzymes. 100% of the patients had elevated alkaline phosphatase and this parameter responded to the administration of ursodeoxycholic acid. At the 12-month follow-up, there was improvement in laboratory parameters and variceal bleeding was prevented with the use of ursodeoxycholic acid (15mg/kg/day) and beta-blocker (40mg propranolol). The presence of liver cirrhosis may be a reason wich patients do not have improvement in liver function tests, as occurred in one of our cases. The time of presentation of the liver alterations and the time of diagnosis of CVID are not related.