Common variable immunodeficiency (hepatic manifestations)

Hepatic manifestations of common variable immunodeficiency are not uncommon and can be primarily related to nodular regenerative hyperplasia.

For a general discussion of the underlying condition, please refer to the article on common variable immunodeficiency (CVID).

Epidemiology

General liver involvement in CVID is controversial among different publications, reported being at least affecting 10% of these patients . Primary liver parenchymal involvement in CVID is mostly characterized by nodular regenerative hyperplasia, and this has been reported in up to 87% of patients undergoing liver biopsy .

Clinical presentation

Liver involvement is mostly asymptomatic until the development of complications like chronic cholestasis, non-cirrhotic portal hypertension, and cirrhosis .

Abnormal liver function tests are usually present, particularly elevation of alkaline phosphatase (ALP).

Pathology

Hepatic intrasinusoidal inflammatory infiltrates and nodular regenerative hyperplasia are commonly found in CVID patients.

Although primary liver malignancy is not common in CVID patients, the liver can be secondarily involved in non-Hodgkin lymphoma, which is the most common type of malignancy affecting them .

Radiographic features

CVID patients can have a spectrum of presentation on imaging, ranging from a normal-appearing liver, non-specific heterogeneous parenchyma, to features of cirrhosis and portal hypertension.