Article
Pulmonary arterial hypertension with signs of venous/capillary involvement in connective tissue diseases: paradigms and paradoxes.
European respiratory review : an official journal of the European Respiratory Society - 1 Jul 2026
Zebian Ghadi, Giordano Jessica, Dorfmüller Peter, Hivin Elisa, Lamblin Nicolas, Sitbon Olivier, Humbert Marc, Sobanski Vincent, Hachulla Eric, Launay David, Pontana François, Montani David, Sanges Sébastien
Abstract excerpt
Pulmonary arterial hypertension (PAH) with features of venous and/or capillary involvement, formerly pulmonary veno-occlusive disease (PVOD), represents a rare and severe subset of pulmonary hypertension. While PVOD is typically idiopathic, heritable or drug/toxin-induced, "PVOD-like" features have long been recognised in PAH associated with connective tissue diseases (CTDs), especially systemic sclerosis (SSc)....
Read the complete abstract on PubMedTopics
- Humans
- Pulmonary Veno-Occlusive Disease
- Connective Tissue Diseases
- Phenotype