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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Bibliographic Details
Published in:Life (Basel, Switzerland) Switzerland), 2022-11, Vol.12 (11), p.1781
Main Authors: Colaci, Michele, Aprile, Maria Letizia, Sambataro, Domenico, Sambataro, Gianluca, Malatino, Lorenzo
Format: Article
Language:English
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
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Summary: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.
ISSN:2075-1729
2075-1729
DOI:10.3390/life12111781