AI Article Synopsis
- - Liver disease is a significant risk factor for higher mortality in patients with common variable immunodeficiency (CVID), mainly due to conditions like presinusoidal portal hypertension (PHTN) from nodular regenerative hyperplasia (NRH).
- - Recent studies have explored the causes and treatment options, revealing that T-cell damage leads to NRH and PHTN in CVID patients, with elevated liver stiffness and enlarged spleens as potential red flags for early detection.
- - There is a call for more research to improve the understanding of liver diseases in CVID and to establish effective treatment protocols, as current strategies are often inconsistent and based on trial and error.
Article Abstract
Purpose Of Review: Liver disease has emerged as a major risk factor for increased mortality in patients with common variable immunodeficiency (CVID). This is mostly due to presinusoidal portal hypertension (PHTN) frequently secondary to nodular regenerative hyperplasia (NRH). Its pathogenesis is still poorly understood and treatment strategies for its various stages are often guided by trial and error. This review summarizes the most recent findings in the light of previous literature.
Recent Findings: In the last 2 years, different groups have addressed pathology, diagnostics, treatment, and liver transplantation. Histological examinations seem to support the pathogenetic sequence of T-cell mediated infiltration and damage of the sinusoidal space with secondary development of NRH, pericellular fibrosis, and the manifestation of PHTN. While markers of the early phase - beyond slight elevation of cholestatic enzymes - are still missing, elevated liver stiffness and splenomegaly above 16 cm longitudinal diameter have been suggested as warning signs for PHTN in CVID patients. Data on immunosuppressive treatment of this manifestation is still very heterogeneous, but a recent report on liver transplantation was encouraging for end stage liver disease.
Summary: Liver disease deserves higher attention in the management of CVID. More studies are needed to understand its pathogenesis and optimal treatment.
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| http://dx.doi.org/10.1097/ACI.0000000000001035 | DOI Listing |
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