Pulmonary Veno-Occlusive Disease in Systemic Sclerosis: A Scoping Review of the Literature.
5 affiliations
- 1Rheumatology Unit, Department of Medicine-DIMED, Padova University Hospital, 35128 Padova, Italy.
- 2Cardiology Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, Padova University Hospital, 35128 Padova, Italy.
- 3Unit of Advanced Clinical and Translational Imaging, Department of Cardiac, Thoracic, Vascular Sciences and Public Health-DCTV, University of Padova, 35128 Padova, Italy.
- 4Department of Surgery, Oncology and Gastroenterology (DiSCOG), Padova University Hospital, 35128 Padova, Italy.
- 5Department of Cardiac, Thoracic, Vascular Sciences and Public Health, Padova University Hospital, 35128 Padova, Italy.
Abstract
Background: Patients with systemic sclerosis (SSc) may be affected by pulmonary veno-occlusive disease (PVOD), a poorly characterized form of group I pulmonary arterial hypertension (PAH). This scoping review aimed to systematically map the literature regarding SSc-PVOD to identify key clinical features, knowledge gaps, and future research areas. Methods: A systematic literature search was conducted (January 1970 to May 2026) across PubMed, Scopus, and Cochrane databases following the PRISMA-ScR guidelines. We included adult patients with right heart catheterization (RHC)-confirmed SSc-PAH and a concurrent/subsequent diagnosis of PVOD established via pulmonary edema following vasodilators, highly suggestive chest HRCT findings, or histopathology. Results: A total of 20 articles were included, encompassing 63 SSc-PVOD patients (87.2% females). Chest HRCT revealed ≥2 highly suggestive abnormalities (centrilobular ground-glass opacities, interlobular septal thickening, mediastinal lymphadenopathy) in 46/54 patients. Almost all patients had mPAP > 35 mmHg at diagnosis, and 12/25 had DLCO < 30%. Lung tissue was obtained in 13 patients (11 post-mortem); predominant histological findings were venous luminal narrowing (11/13), arteriole fibrosis (9/13), and thickened interalveolar septae (9/13). Notably, 20 out of 24 patients treated with pulmonary vasodilators developed pulmonary edema. Overall, two patients underwent lung transplantation, and 33 (52%) died. Conclusions: SSc-PVOD mimics classic SSc-PAH at RHC but carries a high risk of PAH-treatment-induced pulmonary edema. Early non-invasive recognition is vital to avoid drug-related harm and prompt transplant referral. Novel anti-remodeling therapies should be explored.
Related
- Systemic sclerosis markedly impairs health-related quality of life: a single-centre tertiary cohort study with literature-based comparison to leading causes of global disease burden.
- Sotatercept-related peripheral microvascular alterations in systemic sclerosis–associated pulmonary arterial hypertension: insights from nailfold capillaroscopy
- Case Report: Upfront sotatercept-based triple therapy in high-risk systemic sclerosis-associated pulmonary arterial hypertension
- Expert Perspectives: Defining and Managing Progressive Pulmonary Fibrosis in Systemic Sclerosis
- Endothelial and platelet dysfunction in systemic sclerosis with pulmonary hypertension
BibTeX
@article{carta2026pulmonary,
title = {{Pulmonary Veno-Occlusive Disease in Systemic Sclerosis: A Scoping Review of the Literature.}},
author = {Francesco Carta and Beatrice Moccaldi and Giulia Famoso and Chiara Giraudo and Anna Cuberli and Marco Binda and Andrea Benini and Martina Perazzolo Marra and Roberta Ramonda and Laura De Michieli and Elisabetta Zanatta},
journal = {Journal of cardiovascular development and disease},
year = {2026},
month = oct,
doi = {10.3390/jcdd13100506},
url = {https://europepmc.org/article/MED/42930902},
note = {CC BY},
}
RIS
TY - JOUR
TI - Pulmonary Veno-Occlusive Disease in Systemic Sclerosis: A Scoping Review of the Literature.
AU - Francesco Carta
AU - Beatrice Moccaldi
AU - Giulia Famoso
AU - Chiara Giraudo
AU - Anna Cuberli
AU - Marco Binda
AU - Andrea Benini
AU - Martina Perazzolo Marra
AU - Roberta Ramonda
AU - Laura De Michieli
AU - Elisabetta Zanatta
PY - 2026
DA - 2026/10/08
JO - Journal of cardiovascular development and disease
DO - 10.3390/jcdd13100506
UR - https://europepmc.org/article/MED/42930902
AB - Background: Patients with systemic sclerosis (SSc) may be affected by pulmonary veno-occlusive disease (PVOD), a poorly characterized form of group I pulmonary arterial hypertension (PAH). This scoping review aimed to systematically map the literature regarding SSc-PVOD to identify key clinical features, knowledge gaps, and future research areas. Methods: A systematic literature search was conducted (January 1970 to May 2026) across PubMed, Scopus, and Cochrane databases following the PRISMA-ScR guidelines. We included adult patients with right heart catheterization (RHC)-confirmed SSc-PAH and a concurrent/subsequent diagnosis of PVOD established via pulmonary edema following vasodilators, highly suggestive chest HRCT findings, or histopathology. Results: A total of 20 articles were included, encompassing 63 SSc-PVOD patients (87.2% females). Chest HRCT revealed ≥2 highly suggestive abnormalities (centrilobular ground-glass opacities, interlobular septal thickening, mediastinal lymphadenopathy) in 46/54 patients. Almost all patients had mPAP > 35 mmHg at diagnosis, and 12/25 had DLCO < 30%. Lung tissue was obtained in 13 patients (11 post-mortem); predominant histological findings were venous luminal narrowing (11/13), arteriole fibrosis (9/13), and thickened interalveolar septae (9/13). Notably, 20 out of 24 patients treated with pulmonary vasodilators developed pulmonary edema. Overall, two patients underwent lung transplantation, and 33 (52%) died. Conclusions: SSc-PVOD mimics classic SSc-PAH at RHC but carries a high risk of PAH-treatment-induced pulmonary edema. Early non-invasive recognition is vital to avoid drug-related harm and prompt transplant referral. Novel anti-remodeling therapies should be explored.
SN - 2308-3425
ER -