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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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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| First group : SSC with PSVD | medical file analysis of patients affected by SSC and PSVD | ||
| Second group : PSVD without SSC | medical file analysis of patients affected by PSVD only | ||
| Third group : SSC without PSVD | medical file analysis of patients affected by SSC only |
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| Measure | Description | Time Frame |
|---|---|---|
| Prognosis | To compare prognosis of PSVD patients with or without SSc (less or more gastrointestinal bleeding/portal vein thrombosis) | 2 years |
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Inclusion Criteria:
Exclusion Criteria:
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3 types of patients : PSVD with SSC, PSVD without SSC, SSC without PSVD will be compared.
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Chu de Caen | Caen | Calvados | 14000 | France |
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| ID | Term |
|---|---|
| D000094724 | Idiopathic Noncirrhotic Portal Hypertension |
| D012595 | Scleroderma, Systemic |
| ID | Term |
|---|---|
| D006975 | Hypertension, Portal |
| D008107 | Liver Diseases |
| D004066 | Digestive System Diseases |
| D003240 | Connective Tissue Diseases |
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| D017437 | Skin and Connective Tissue Diseases |
| D012871 | Skin Diseases |