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Hemodynamic improvement and prognostic impact of intravenous iloprost in patients with systemic sclerosis

Francesca Spano

Riccardo Scagliola

Francesco Puppo

Claudio Brunelli

Manrico Balbi

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Conference: 2017 PH Professional Network Symposium

Release Date: 10.06.2017

Presentation Type: Abstracts

File Download: 2017 PHPN Abstract 1032

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Abstract presented at the 2017 PH Professional Network Symposium held in Bethesda, MD on October 5-7, 2017

 

Purpose

Our aim was to determine if vasodilator treatment with intravenous (i.v.) iloprost for treatment and/or prevention of Raynaud’s phenomenon and digital ulcers could impact on prognosis and hemodynamic features of pulmonary circulation in patients with systemic sclerosis (SSc).

Background

Pulmonary arterial hypertension is a late, well known complication of (SSc), which significantly impacts on prognosis and quality of life. Vasodilator treatment with i.v. prostanoids have been approved for the management of Raynaud’s phenomenon and digital ulcers. Moreover, they contribute to attenuate vascular remodeling on pulmonary circulation in patients with connective tissue disease.

Methods

From January 2007 until September 2012, 28 SSc patients (18 with limited cutaneous form, 7 with diffuse cutaneous form and 3 with ‘Overlap’ syndromes) underwent right heart catheterization on the basis of high clinical and echocardiographic suspicion of pulmonary hypertension. All the patients fulfilled the diagnostic criteria for SSc proposed by the American College of Rheumatology and the European League Against Rheumatism (ACR/EULAR). Thirteen SSc patients were in treatment with monthly cycles ofi.v.iloprost (0,5-2 ng/kg/min/6 hours/cycle) for the management of Raynaud’s phenomenon and/or digital ulcers, while the other 15 did not receive any i.v. prostanoids.

Results

Both groups of SSc patients were comparable in terms of sex, age and anthropometric features. Patients treated with i.v. iloprost showed a low mean value of pulmonary vascular resistances (4.7 W.U. ± 4.0 vs 6.9 W.U. ± 4.61; p=0.07) and mean pulmonary arterial pressure (33.3 mmHg ± 12.4 vs 37.6 mmHg ± 9.1 p=0.4), although statistically nonsignificant, and a significant increase of cardiac index (3.1 l/min/m2 ± 0.6 vs 2.5 l/min/m2 ± 0.7; p= 0.03) than those untreated with i.v. prostanoids. Moreover, survival in patients treated with i.v. iloprost at 5 and 7 years from SSc diagnosis was 97.4% and 96.8% respectively, whereas untreated patients had a worse survival rate at 5 and 7 years (86.3% and 72.4% respectively). Vasodilator treatment with i.v. iloprost was associated with the greatest survival advantage both at 5 (OR 6.18; p=0.042) and 7 years (OR7.14; p=0.036) from SSc diagnosis.

Conclusion

Our dates suggest how vasodilator therapy with i.v. iloprost for the management of Raynaud’s phenomenon and/or digital ulcers may impact on hemodynamic profile and survival, suggesting a potential protective role on pulmonary circulation in patients with SSc.