Porto-Sinusoidal Vascular Disorders (PSVD) is a heterogeneous clinico-pathological entity characterized by alterations of small liver vessels. The most common liver lesions include “nodular regenerative hyperplasia (NRH)” and obliterative portal venopathy (1,2). Patients with PSVD can developed portal hypertension and its complications as well as portal thrombosis in 1/3 of patients at five years (2-5). In patients with PSVD and signs of portal hypertension, 10 years survival decreases to 56 to 82% (2,3), highly related to conditions associated with PSVD including infections, immune-mediated disorders, haematological diseases, prothrombotic disorders, drugs exposure such as azathioprine, and genetic diseases (3,6-8).
Among immune-mediated disorders, systemic sclerosis (SSc) has been associated with NRH (9,10). This connective tissue disease can affect multiple organs and is characterized by cutaneous and/or visceral fibrosis, immune dysfunctions and microvascular injury including damage of endothelial cells (11). Liver may be involved. The most frequent liver disease associated with SSc is primary biliary cholangitis in 2 to 22% of cases (10,11,12). NHR prevalence estimated at 1.4%, may be underestimated as NRH diagnosis is histologic and usually suspected in case of complications due to portal hypertension (13). Available data about NRH associated with SSc are limited, as summarized in Appendix 1. In particular, little is known about patients’ outcome in this setting.
Study Population
Inclusion criteria:
Cases
Patients ≥ 18 years, with PSVD diagnosed since January 1 2000 according to VALDIG criteria (appendix 2, (1,14)) associated with SSc diagnosed since 2000 according to ACR/EULAR classification (score ≥ 9, appendix 3, (15,16)). For patients with a ACR/EULAR score < 9, diagnosis can be done on an expert opinion based on the association of organ damages and specific auto-antibodies.
Controls 1: Patients ≥ 18 years, with PSVD diagnosed since January 1 2000 according to VALDIG criteria (appendix 2, (1,14)) without SSc. These controls will be recruited from the Beaujon Hospital prospective database dedicated to liver vascular diseases.
Controls 2: Patients ≥ 18 years, with SSc diagnosed since January 1 2000 2000 according to ACR/EULAR classification (score ≥ 9, appendix 3, (15,16)) without PSVD. Controls will be recruited from the Caen Hospital prospective database dedicated to SSc.
Exclusion criteria for cases and controls
- Cirrhosis
- Localized systemic sclerosis (Morphéa)
- Biliary cholangitis and /or autoimmune hepatitis (histologic or immunologic criteria)