Management of a patient with common variable immunodeficiency and hepatopathy

Abstract

Background Common variable Immunodeficiency (CVID) is a primary immunodeficiency disorder and the most common form of severe antibody deficiency. Both children and adults are affected and clinical manifestations vary widely. Often, CVID manifests with infections, autoimmune phenomena or chronic lung disease, but it also frequently affects the liver. The differential diagnoses of hepatopathies in CVID patients are diverse and the characteristics of CVID patients often make it difficult to determine the correct diagnosis. Case presentation We present the case of a 39-year-old patient with CVID and elevated liver enzymes, nausea and unintended weight loss, who was referred to our clinic with the suspected diagnose of autoimmune hepatitis or immunoglobulin-induced hepatopathy. Prior, the patient had undergone an extensive diagnostic work-up including liver biopsy but viral hepatitides had only been investigated serologically – with negative antibody results. We searched for viral nucleic acid by polymerase chain reaction and detected hepatitis E virus-RNA. Antiviral therapy was started and the patient recovered quickly. Conclusion Hepatopathies in CVID patients are common with a broad spectrum of possible causes. While treating CVID patients, the distinct diagnostic and therapeutic requirements of the CVID patients should be closely considered and diagnosed by the appropriate measures.

Differential Diagnosis: Hepatic Complications in Inborn Errors of Immunity

“…Hepatitis E virus appears to have a lower prevalence with fewer documented reports within inborn errors of immunity, which may be associated with the neutralising antibody in many infused immunoglobulin products [80]. There has been a recent case report of CVID patient with acute hepatitis owing to hepatitis E, which highlights the importance of maintaining vigilance even in those established on immunoglobulin replacement [81].…”