Intravenous iloprost is a first-line option for the treatment of scleroderma-related digital vasculopathy, and some studies have suggested its favourable role on disease progression. The aim of our study is to evaluate the disease progression, specifically in terms of cardiopulmonary function, in a group of consecutive patients chronically treated with intravenous iloprost. Our retrospective study enrolled 68 scleroderma patients (68 F, 54.4 ± 12.3 years) treated with iloprost for 7.1 ± 2.9 years, with a schedule of 5–6 consecutive daily infusions per month (6 h/day, 0.5–2.0 ng/kg/min). In all patients, modified Rodnan skin score (4.7 ± 5.3 vs. 3.7 ± 5.3, p < 0.0001), systolic pulmonary arterial pressure (sPAP) (30.9 ± 6.4 vs. 24.0 ± 3.2 mmHg, p < 0.0001), tricuspid annular plane systolic excursion (22.1 ± 2.4 vs. 23.8 ± 3.5 mm, p = 0.0001), pro-brain natriuretic peptide (97.2 ± 69.3 vs. 65.8 ± 31.7 pg/ml, p = 0.0005) showed statistically significant improvement from baseline. In the subgroup of patients with baseline sPAP ≥36 mmHg (n = 17), a significant sPAP reduction was observed (from 39.5 ± 3.8 to 25.1 ± 4.5 mmHg, p < 0.0001) after 7.6 ± 2.5 years of follow-up. The number of patients with digital ulcers (DUs) at follow-up was reduced from baseline (42.6 vs. 11.8%, p < 0.001), and none of the free-DU patients at baseline presented DUs at follow-up. An intensive and chronic regimen of IV iloprost administration seems to stabilize and potentially improve the long-term development of disease in SSc patients, as suggested by stabilization or significant improvement of cardiopulmonary parameters and vasculopathy.

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BackgroundCardiopulmonary involvement is recognized as a poor prognostic factor in systemic sclerosis (SSc). So, stabilizing the cardiopulmonary parameters as long as possible may represent an important therapeutic goal. Intravenous iloprost is a first-line option for the treatment of SSc-related digital vasculopathy to reduce the frequency and severity of SSc-Raynaud's phenomenon (RP) attacks and to heal active digital ulcers in patients with SSc.1 Some studies have suggested its role in preventing the incidence of most severe vascular complications in long term treated patients.2–3ObjectivesThe aim of our study is to evaluate the evolution of the disease, with particular focus on cardiopulmonary function, in a group of consecutive patients chronically treated with iloprost at the Rheumatology Unit of AOU Policlinico Vittorio Emanuele, Catania Italy, from 2006 to 2015.Methods59 SSc patients (59F, 51.8±12.1 years). At baseline, 71.2%, 20.3% and 6.8% had a limited, diffuse and early SSc, respectively, and 23% had interstitial lung disease.Iloprost was administered for a mean duration of 31.1±18.9 months, with a regimen of 6 infusions per month (6 hours/day, 0.5–2.0 ng/kg/min) to treat secondary RP, diagnosed at an average age of 45.9±14.8 years.We evaluated: skin score (SS), systolic pulmonary arterial pressure (PAPs), plane tricuspid annular systolic excursion (TAPSE), lung diffusing capacity of carbon monoxide (DLCO), forced vital capacity (FVC), alveolar volume (VA), DLCO/VA, pro-brain natriuretic peptide (pBNP), and NYHA class.We analyzed the patients as whole group and in groups divided according to the length of treatment: <1 year (n=10), 1–2 years (n=9), 2–3 years (n=21), >3 years (n=19).ResultsIn the whole group of patients, SS showed statistically significant improvement from baseline (4.6±6.2 vs 3.9±6.0, p=0.034) while the other parameters showed no significant changes: TAPSE (21.5±2.3 vs 23.1±8.1 mm), PAPs (31.3±6.4 vs 30.1±9.0 mmHg), DLCO (83.5±14.4 vs 81.1±14.2 mmol/kPa.min), FVC (108.2±15.1 vs 107.9±18.2 liters), VA (93.4±11.7 vs 92.2±13.1 liters), DLCO/VA (88.1±13.7 vs 87.6±15.8), pBNP (105.2±75.1 vs 90.8±64.0 pg/ml), and NYHA class (1.0±0.0 vs 1.1±0.4).Subgroup analysis indicates a significant improvement in the SS from >1 year follow-up on, and a significant reduction of the PAPs in the subgroup of patients treated for more than 3 years. We observed an inverse correlation (R=-0.97) between the duration of treatment and the reduction of the PAPs from baseline.ConclusionsAn intensive (6 inf/month) and chronic regimen of iv iloprost administration seems to stabilize and potentially improve the long term evolution of disease in SSc patients, as suggested by the stabilization of cardiopulmonary parameters and the significant improvement of SS and PAPs in patients treated for more than 3 years.Our results suggest a potential effect of iv iloprost on the evolution of the disease, with a role that goes beyond the mere treatment of SSc vasculopathy.ReferencesAnn Rheum Dis;68:620–8.Rheumatol Int 2005;25:250–...