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Clinical Reviews in Allergy & Immunology

Erschienen in:

07.08.2017

Common Variable Immunodeficiency and Liver Involvement

verfasst von: Junmin Song, Ana Lleo, Guo Xiang Yang, Weici Zhang, Christopher L. Bowlus, M. Eric Gershwin, Patrick S. C. Leung

Erschienen in: Clinical Reviews in Allergy & Immunology | Ausgabe 3/2018

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Abstract

Common variable immunodeficiency (CVID) is a primary B-cell immunodeficiency disorder, characterized by remarkable hypogammaglobulinemia. The disease can develop at any age without gender predominance. The prevalence of CVID varies widely worldwide. The underlying causes of CVID remain largely unknown; primary B-cell dysfunctions, defects in T cells and antigen-presenting cells are involved. Although some monogenetic defects have been identified in some CVID patients, it is likely that CVID is polygenic. Patients with CVID develop recurrent and chronic infections (e.g., bacterial infections of the respiratory or gastrointestinal tract), autoimmune diseases, lymphoproliferation, malignancies, and granulomatous lesions. Interestingly, autoimmunity can be the only clinical manifestation of CVID at the time of diagnosis and may even develop prior to hypogammaglobulinemia. The diagnosis of CVID is largely based on the criteria established by European Society for Immunodeficiencies and Pan-American Group for Immunodeficiency (ESID/PAGID) and with some recent modifications. The disease can affect multiple organs, including the liver. Clinical features of CVID patients with liver involvement include abnormal liver biochemistries, primarily elevation of alkaline phosphatase (ALP), nodular regenerative hyperplasia (NRH), or liver cirrhosis and its complications. Replacement therapy with immunoglobulin (Ig) and anti-infection therapy are the primary treatment regimen for CVID patients. No specific therapy for liver involvement of CVID is currently available, and liver transplantation is an option only in select cases. The prognosis of CVID varies widely. Further understanding in the etiology and pathophysiology will facilitate early diagnosis and treatments to improve prognosis.

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Titel: Common Variable Immunodeficiency and Liver Involvement
verfasst von: Junmin Song
Ana Lleo
Guo Xiang Yang
Weici Zhang
Christopher L. Bowlus
M. Eric Gershwin
Patrick S. C. Leung
Publikationsdatum: 07.08.2017
Verlag: Springer US
Erschienen in: Clinical Reviews in Allergy & Immunology / Ausgabe 3/2018
Print ISSN: 1080-0549
Elektronische ISSN: 1559-0267
DOI: https://doi.org/10.1007/s12016-017-8638-z

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Springer Medizin

Common Variable Immunodeficiency and Liver Involvement

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