In January 2025, the Journal of Hepatology published a thought-provoking letter titled “Beyond Portal Hypertension: Characterizing Porto-Sinusoidal Vascular Disorder in Patients without Signs of PH.” This study sheds new light on the clinical course and prognosis of PSVD in patients who do not exhibit signs of portal hypertension (PH) at the time of diagnosis
The researchers analyzed data from 30 patients diagnosed with PSVD at the IRCCS Humanitas Research Hospital in Milan. None of the patients showed radiological or endoscopic evidence of PH, and their platelet counts remained consistently above normal thresholds. Over a median follow-up of 58.8 months, no PH-related complications such as gastrointestinal bleeding, thrombosis, or splenomegaly were observed, suggesting a stable and benign course for PSVD without PH in the medium term.
Interestingly, while autoimmune diseases were common among the cohort, none of the patients had significant exposure to drugs typically associated with PSVD, and there were no cases of thrombosis or HIV. Despite these reassuring findings, the authors caution that the potential for disease progression over longer periods, especially in patients with comorbidities, cannot be ruled out.
This study highlights the urgent need for large-scale, multicenter studies to better understand the natural history of PSVD without PH and to develop optimized management strategies for this unique patient population.
Read the full letter in the Journal of Hepatology: https://lnkd.in/e8wjpMTk
