ORPHANET code for PSVD

[Pierre-Emmanuel Rautou]

Dear all,

We are happy to inform you that “Portosinusoidal vascular disease” is now part of the Orphanet nomenclature and classification of rare hepatic diseases, along with its 3 histopathological subtypes:
(Disorder) Portosinusoidal vascular disease ORPHA:596937 — FR: Maladie vasculaire portosinusoïdale
(Subtype)__________Hepatoportal sclerosis ORPHA:64743 (synonym: Obliterative portal venopathy) — FR: Veinopathie portale oblitérante
(Subtype)__________Nodular regenerative hyperplasia of the liver ORPHA:48372 — FR: Hyperplasie nodulaire régénérative du foie
(Subtype)__________Incomplete septal cirrhosis ORPHA:596941 (synonym: Incomplete septal fibrosis) — FR: Cirrhose septale incomplète

It will facilitate exchange about this disease.

All the best
Pierre-Emmmanuel

[Akash Shukla]

Great News and congratulations to all !

[Stephen Caldwell]

Hi All

This format isn’t very intuitive especially for an old guy. I think I may have muddled through to actually send a message. I think its good that we undertake some further parsing of the nomenclature to address this occasional but uncommon group of illnesses that I think are best are best united by the presence of non-cirrhotic portal hypertension. I wonder if its not best to parse these further by associated or underlying conditions.

Best regards
Steve

SH Caldwell, MD, FAASLD
Professor Hepatology
University of Virginia
Charlottesville, VA

[Pierre-Emmanuel Rautou]

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

[Andrea De Gottardi]

Dear All,
Thank you very much for this further step! Looking forward to critically discussing with you at ILC2021 how to improve our understanding of this condition.
Kindest regards
Andrea

[Author]

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