Challenges in liver transplantation for common variable immunodeficiency-related liver disease: a case series and systematic review

Background Common variable immunodeficiency (CVID) is a rare primary B-cell immunodeficiency disorder associated with multisystem complications of impaired humoral immunity. CVID is characterized by impaired adaptive responses to pathogen challenge and a paradoxical predisposition to a wide range of autoimmune and neoplastic complications. Methods We present a case series describing our experience of liver transplantation in two patients with CVID and perform a comprehensive literature review of post-transplant complications and survival outcomes detailing the global experience of 30 reported cases of adult liver transplantation for common variable immunodeficiency-related liver disease (CVIDrLD). Results Liver transplantation in CVIDrLD is frequently complicated by atypical infections, autoimmune complications and graft recurrence and is associated with suboptimal long term survival outcomes post-transplantation in comparison to established listing indications for liver transplantation; this is further compounded (median survival 3 vs. 5 years) when removing patients with concomitant chronic hepatitis C infection from the survival analysis. Conclusions Liver transplantation is associated with poor overall survival outcomes and careful consideration should be given to the phenotype and severity of immunodeficiency, the presence of chronic pathogen colonisation and the extrahepatic burden of disease at the time of assessment. At present, despite careful patient selection, survival post-transplant outcomes fall below acceptable listing thresholds, necessitating a conservative approach to consideration of single organ listing in this context with current available treatments. Patients with a secondary aetiological factor for liver disease may perform better post-transplant.