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Systemic Sclerosis and Idiopathic Portal Hypertension: Report of a Case and Review of the Literature
The presence of liver involvement in systemic sclerosis (SSc) is considered atypical, besides the eventual coexistence of other autoimmune hepatic disorders. However, the occurrence of syndromes called idiopathic portal hypertension (IPH) and regenerative nodular hyperplasia (RNH) have been anecdota...
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| Published in: | Life (Basel, Switzerland) Switzerland), 2022-11, Vol.12 (11), p.1781 |
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| Main Authors: | , , , , |
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| container_issue | 11 |
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| container_title | Life (Basel, Switzerland) |
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| creator | Colaci, Michele Aprile, Maria Letizia Sambataro, Domenico Sambataro, Gianluca Malatino, Lorenzo |
| description | The presence of liver involvement in systemic sclerosis (SSc) is considered atypical, besides the eventual coexistence of other autoimmune hepatic disorders. However, the occurrence of syndromes called idiopathic portal hypertension (IPH) and regenerative nodular hyperplasia (RNH) have been anecdotally reported in the literature. We described a case of SSc complicated by IPH and we reviewed the literature on the topic. No specific SSc pattern linked to IPH emerged, even though the reports often described IPH in patients with limited skin subset SSc. Coexistence of prothrombotic states and overlap with other hepatic diseases could facilitate IPH onset. In spite of IPH being a rare condition, the rheumatologists should consider IPH as a possible hepatic complication in SSc patients. |
| doi_str_mv | 10.3390/life12111781 |
| format | article |
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However, the occurrence of syndromes called idiopathic portal hypertension (IPH) and regenerative nodular hyperplasia (RNH) have been anecdotally reported in the literature. We described a case of SSc complicated by IPH and we reviewed the literature on the topic. No specific SSc pattern linked to IPH emerged, even though the reports often described IPH in patients with limited skin subset SSc. Coexistence of prothrombotic states and overlap with other hepatic diseases could facilitate IPH onset. In spite of IPH being a rare condition, the rheumatologists should consider IPH as a possible hepatic complication in SSc patients.</description><identifier>ISSN: 2075-1729</identifier><identifier>EISSN: 2075-1729</identifier><identifier>DOI: 10.3390/life12111781</identifier><identifier>PMID: 36362936</identifier><language>eng</language><publisher>Basel: MDPI AG</publisher><subject>Antibodies ; Ascites ; Autoimmune diseases ; Biopsy ; Case Report ; Case reports ; Case studies ; Coexistence ; Disorders ; Hepatitis ; Hyperplasia ; Hypertension ; idiopathic portal hypertension ; Literature reviews ; Liver ; Liver diseases ; Patients ; Portal hypertension ; Portal vein ; Scleroderma ; Scleroderma (Disease) ; Skin ; Skin diseases ; Systemic scleroderma ; Systemic sclerosis ; Thromboembolism ; Thrombosis ; Veins & arteries</subject><ispartof>Life (Basel, Switzerland), 2022-11, Vol.12 (11), p.1781</ispartof><rights>COPYRIGHT 2022 MDPI AG</rights><rights>2022 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). 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However, the occurrence of syndromes called idiopathic portal hypertension (IPH) and regenerative nodular hyperplasia (RNH) have been anecdotally reported in the literature. We described a case of SSc complicated by IPH and we reviewed the literature on the topic. No specific SSc pattern linked to IPH emerged, even though the reports often described IPH in patients with limited skin subset SSc. Coexistence of prothrombotic states and overlap with other hepatic diseases could facilitate IPH onset. 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| source | Publicly Available Content Database; PubMed Central |
| subjects | Antibodies Ascites Autoimmune diseases Biopsy Case Report Case reports Case studies Coexistence Disorders Hepatitis Hyperplasia Hypertension idiopathic portal hypertension Literature reviews Liver Liver diseases Patients Portal hypertension Portal vein Scleroderma Scleroderma (Disease) Skin Skin diseases Systemic scleroderma Systemic sclerosis Thromboembolism Thrombosis Veins & arteries |
| title | Systemic Sclerosis and Idiopathic Portal Hypertension: Report of a Case and Review of the Literature |
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