Management of liver disease and portal hypertension in common variable immunodeficiency (CVID)Key points
Summary: Patients with common variable immunodeficiency (CVID) frequently develop liver disease and associated complications, which represent an increasingly prevalent unmet medical need. The main hepatic manifestation of CVID is nodular regenerative hyperplasia (NRH), resulting in non-cirrhotic por...
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| Format: | Article |
| Language: | English |
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Elsevier
2023-11-01
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| Series: | JHEP Reports |
| Subjects: | |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2589555923002136 |
| _version_ | 1797647836529033216 |
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| author | Lukas S. Baumert Angela Shih Raymond T. Chung |
| author_facet | Lukas S. Baumert Angela Shih Raymond T. Chung |
| author_sort | Lukas S. Baumert |
| collection | DOAJ |
| description | Summary: Patients with common variable immunodeficiency (CVID) frequently develop liver disease and associated complications, which represent an increasingly prevalent unmet medical need. The main hepatic manifestation of CVID is nodular regenerative hyperplasia (NRH), resulting in non-cirrhotic portal hypertension (NCPH). Liver disease is often underdiagnosed, leading to poor outcomes and decreased survival. The increasing numbers of patients with CVID who are diagnosed late with progressive liver disease underscores the importance of appropriate clinical management and treatment of liver complications. At the same time, specific guidelines for the clinical management of CVID-related liver disease are still lacking. Here, we review the epidemiology of CVID-related liver disease, reveal new insights into NRH and NCPH biology and highlight recently uncovered opportunities for NCPH diagnostics in CVID. Finally, we focus on current management of liver disease, portal hypertension and its complications – the key challenge in patients with CVID. Specifically, we review recent data regarding the role of transjugular intrahepatic portosystemic shunt and liver transplantation in clinical management. The role for anticoagulants and immunosuppressants targeting the pathogenesis of NRH will also be discussed. We propose an updated algorithm for the diagnostic work-up and treatment of NCPH in CVID. Finally, we consider future needs and therapeutic opportunities for CVID-related liver disease. |
| first_indexed | 2024-03-11T15:22:16Z |
| format | Article |
| id | doaj.art-a06cad66b1454b1ea7a577b33d4e4f11 |
| institution | Directory Open Access Journal |
| issn | 2589-5559 |
| language | English |
| last_indexed | 2024-03-11T15:22:16Z |
| publishDate | 2023-11-01 |
| publisher | Elsevier |
| record_format | Article |
| series | JHEP Reports |
| spelling | doaj.art-a06cad66b1454b1ea7a577b33d4e4f112023-10-28T05:09:34ZengElsevierJHEP Reports2589-55592023-11-01511100882Management of liver disease and portal hypertension in common variable immunodeficiency (CVID)Key pointsLukas S. Baumert0Angela Shih1Raymond T. Chung2Liver Center, Gastrointestinal Unit, Department of Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA; Faculty of Medicine, Eberhard-Karls University of Tübingen, Tübingen, GermanyDepartment of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USALiver Center, Gastrointestinal Unit, Department of Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA; Corresponding author. Address: Liver Center, GI Division, Massachusetts General Hospital, 55 Fruit St, Boston, MA 02114, USA.Summary: Patients with common variable immunodeficiency (CVID) frequently develop liver disease and associated complications, which represent an increasingly prevalent unmet medical need. The main hepatic manifestation of CVID is nodular regenerative hyperplasia (NRH), resulting in non-cirrhotic portal hypertension (NCPH). Liver disease is often underdiagnosed, leading to poor outcomes and decreased survival. The increasing numbers of patients with CVID who are diagnosed late with progressive liver disease underscores the importance of appropriate clinical management and treatment of liver complications. At the same time, specific guidelines for the clinical management of CVID-related liver disease are still lacking. Here, we review the epidemiology of CVID-related liver disease, reveal new insights into NRH and NCPH biology and highlight recently uncovered opportunities for NCPH diagnostics in CVID. Finally, we focus on current management of liver disease, portal hypertension and its complications – the key challenge in patients with CVID. Specifically, we review recent data regarding the role of transjugular intrahepatic portosystemic shunt and liver transplantation in clinical management. The role for anticoagulants and immunosuppressants targeting the pathogenesis of NRH will also be discussed. We propose an updated algorithm for the diagnostic work-up and treatment of NCPH in CVID. Finally, we consider future needs and therapeutic opportunities for CVID-related liver disease.http://www.sciencedirect.com/science/article/pii/S2589555923002136nodular regenerative hyperplasianon-cirrhotic portal hypertensionliver transplantationTIPSalgorithmautoimmune hepatitis |
| spellingShingle | Lukas S. Baumert Angela Shih Raymond T. Chung Management of liver disease and portal hypertension in common variable immunodeficiency (CVID)Key points JHEP Reports nodular regenerative hyperplasia non-cirrhotic portal hypertension liver transplantation TIPS algorithm autoimmune hepatitis |
| title | Management of liver disease and portal hypertension in common variable immunodeficiency (CVID)Key points |
| title_full | Management of liver disease and portal hypertension in common variable immunodeficiency (CVID)Key points |
| title_fullStr | Management of liver disease and portal hypertension in common variable immunodeficiency (CVID)Key points |
| title_full_unstemmed | Management of liver disease and portal hypertension in common variable immunodeficiency (CVID)Key points |
| title_short | Management of liver disease and portal hypertension in common variable immunodeficiency (CVID)Key points |
| title_sort | management of liver disease and portal hypertension in common variable immunodeficiency cvid key points |
| topic | nodular regenerative hyperplasia non-cirrhotic portal hypertension liver transplantation TIPS algorithm autoimmune hepatitis |
| url | http://www.sciencedirect.com/science/article/pii/S2589555923002136 |
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