Reply To: ORPHANET code for PSVD

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#2718

Dear Steve and all,
Thank you for stimulating the debate.
One rational for the term “Portosinusoidal vascular disease (PSVD)” is precisely that PSVD natural history starts -like all liver diseases- with a form without portal hypertension. A term referring to portal hypertension (e.g. idiopathic portal hypertension) does not allow to take that aspect into account (and leads us to focusing on the tip of the iceberg).
Parsing these patients by associated or underlying conditions is likely a good idea, but for the moment we lack data allowing us to identify the precise groups of patients.
At ILC2021 -organised by EASL- in June, there will be a session on PSVD, with a debate on these points. I invite all VALDIG members to join this interactive session.
Best wishes
Pierre-Emmanuel