Rodolfi, Stefano;
Ng, Cheryl Chun Man;
Ruiz Bejerano, Ana Maria;
Kanitkar, Medha;
Ong, Voon H;
Denton, Christopher P;
(2025)
Impact of immunosuppression on development and outcome of systemic sclerosis-associated pulmonary arterial hypertension.
Clinical and Experimental Rheumatology
, 43
(8)
pp. 1492-1498.
10.55563/clinexprheumatol/x0keg5.
Preview |
Text
Rodolfi et al CER - PAH in SSc-IST-for UCL upload 2025.pdf - Accepted Version Download (399kB) | Preview |
Abstract
OBJECTIVES: Pulmonary arterial hypertension (PAH) is a frequent and severe complication of systemic sclerosis (SSc). Despite the improvements achieved in recent decades, PAH remains a cause of significant clinical burden, and treatment mainly relies on a combination of vasodilatory and anti-proliferative agents, following the same therapeutic approach used for idiopathic PAH. METHODS: We retrospectively analysed the records of 607 patients reviewed in our centre. PAH was defined as pre-capillary pulmonary hypertension (mPAP ≥ 25mmHg, PVR >3WU, PCWP <15mmHg) in absence of significant hypoxic component (patients with clinically significant airway disease, or with interstitial lung disease and FVC <70%, were excluded from PAH group). Early immunosuppression (IS) was defined as treatment within the first 5 years after SSc onset with conventional or biologic disease-modifying anti-rheumatic drugs (DMARDs), or a prednisone-equivalent dose ≥10mg/day. RESULTS: Out of 607 patients, 77 received a diagnosis of PAH. Early immunosuppression was not associated with reduced odds of developing PAH (OR 0.74, p=0.495). However, when analysing individual immunosuppressive agents, early treatment with mycophenolate mofetil was associated with a significant protective effect (OR 0.12; p=0.048). Immunosuppressive treatment was associated with a significant reduction in mortality risk (HR 0.41; p= 0.045), attributable to the effect of hydroxychloroquine, which was the only agent showing an impact on survival (HR 0.04; p= 0.004). CONCLUSIONS: In this retrospective monocentric study early treatment with mycophenolate was associated with reduced odds of developing SSc-PAH, while treatment with hydroxychloroquine showed a significant improvement in survival in patients with established SSc-PAH.
| Type: | Article |
|---|---|
| Title: | Impact of immunosuppression on development and outcome of systemic sclerosis-associated pulmonary arterial hypertension |
| Location: | Italy |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.55563/clinexprheumatol/x0keg5 |
| Publisher version: | https://doi.org/10.55563/clinexprheumatol/x0keg5 |
| Language: | English |
| Additional information: | This version is the version of record. For information on re-use, please refer to the publisher’s terms and conditions. |
| Keywords: | Humans, Scleroderma, Systemic, Retrospective Studies, Female, Male, Middle Aged, Immunosuppressive Agents, Treatment Outcome, Hypertension, Pulmonary, Aged, Risk Factors, Time Factors, Adult, Pulmonary Arterial Hypertension, Mycophenolic Acid, Arterial Pressure, Antirheumatic Agents |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Inflammation |
| URI: | https://discovery.ucl.ac.uk/id/eprint/10212082 |
Archive Staff Only
 |
View Item |
