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Systemic Disease and Portal Hypertension

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Abstract

Purpose of Review

The development of portal hypertension is typically a consequence of liver cirrhosis due mainly to primary liver disorders, whereas non-cirrhotic portal hypertension (NCPH) can be a complication of systemic, primarily extrahepatic diseases. Our purpose was to review the various systemic disorders leading to portal hypertension and provide a pathway for diagnosis and management.

Recent Findings

Non-cirrhotic portal hypertension is a heterogeneous group of liver disorders primarily of vascular origin that may manifest as portal hypertension. The diagnosis of NCPH in the setting of systemic diseases is challenging and a liver biopsy may be required to confirm the diagnosis. Etiologies include those of vascular origin, autoimmune disorders, drug exposures, and infections.

Summary

Complications of portal hypertension in the setting of systemic diseases are similar to patients having cirrhosis and should be addressed similarly while addressing the underlying systemic disorder if possible

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PK made substantial contributions to the conception or design of the work; or the acquisition, analysis, or interpretation of data; drafted the work or revised it critically for important intellectual content; approved the version to be published; and agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. TK made substantial contributions to the acquisition, analysis, or interpretation of data; drafted the work or revised it critically for important intellectual content; approved the version to be published; and agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

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Bhatti, T.K., Kwo, P.Y. Systemic Disease and Portal Hypertension. Curr Hepatology Rep 23, 162–173 (2024). https://doi.org/10.1007/s11901-024-00645-8

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