PortoSinusoidal Vascular Diseases and Systemic Sclerosis

PSVD With or Without SSc

Porto-Sinusoidal Vascular Disease, Nodular regenerative hyperplasia, Systemic sclerosis

Porto-Sinusoidal Vascular Disorders (PSVD) is a heterogeneous clinico-pathological entity characterized by alterations in small liver vessels. Nodular regenerative hyperplasia (NRH) is the most common PSVD. The most frequent liver disease associated with Systemic sclerosis (SSc) is primary biliary cholangitis reported in 2 to 22% of cases. NRH prevalence estimated to 1.4%, may be underestimated as NRH diagnosis is histologic and usually suspected in case of complications due to portal hypertension. Few data are available about NRH associated with SSc resulting in a lack of knowledge of the characteristics and outcome of these patients.

The study aims to compare the outcome of patients with MVPS associated with SSc to patients with MVPS without SSc. This work is a multicenter retrospective study comparing two groups of adult patients, one with MVPS associated with SSc and the other with MVPS without SSc. Patients with MVPS associated with SSc will be recruited from French university hospital departments of Hepatogastroenterology and Internal Medecine. Patients with MVPS without SSc will be recruited from the Beaujon Hospital database dedicated to liver vascular diseases.

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France