PortoSinusoidal Vascular Diseases and Systemic Sclerosis

Caen University Hospital

Status: Completed

Conditions: PSVD With or Without SSc

Study type: Observational

Funder types: Other

Identifiers

NCT06032754

MVPScS

Details and patient eligibility

About

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.

Full description

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.

Enrollment: 180 patients

Sex: All

Ages: 18+ years old

Volunteers: No Healthy Volunteers

Inclusion criteria

• PSVD (Valdig criteria)
• SSC (ACR/EULAR 2013 criteria)

Exclusion criteria

• Liver cirrhosis
• Limited scleroderma
• Autoimmune hepatitis
• Primary biliary cholangitis

Trial design

180 participants in 3 patient groups

First group : SSC with PSVD

Description:

medical file analysis of patients affected by SSC and PSVD

Second group : PSVD without SSC

Description:

medical file analysis of patients affected by PSVD only

Third group : SSC without PSVD

Description:

medical file analysis of patients affected by SSC only