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).
General liver involvement in CVID is controversial among different publications, reported being at least affecting 10% of these patients 2. 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 1.
Liver involvement is mostly asymptomatic until the development of complications like chronic cholestasis, non-cirrhotic portal hypertension, and cirrhosis 1,2.
Abnormal liver function tests are usually present, particularly elevation of alkaline phosphatase (ALP).
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 1.
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.
- 1. Pecoraro, Antonio, Crescenzi, Ludovica, Varricchi, Gilda, Marone, Giancarlo, Spadaro, Giuseppe. Heterogeneity of Liver Disease in Common Variable Immunodeficiency Disorders. (2020) Frontiers in Immunology. 11: 338. doi:10.3389/fimmu.2020.00338 - Pubmed
- 2. Junmin Song, Ana Lleo, Guo Xiang Yang, Weici Zhang, Christopher L. Bowlus, M. Eric Gershwin, Patrick S. C. Leung. Common Variable Immunodeficiency and Liver Involvement. (2018) Clinical Reviews in Allergy & Immunology. 55 (3): 340. doi:10.1007/s12016-017-8638-z - Pubmed
- 3. C. Ward, M. Lucas, J. Piris, J. Collier, H. Chapel. Abnormal liver function in common variable immunodeficiency disorders due to nodular regenerative hyperplasia. (2008) Clinical & Experimental Immunology. 153 (3): 331. doi:10.1111/j.1365-2249.2008.03711.x - Pubmed