title: Long-term clinical stabilization of scleroderma patients treated with a chronic and intensive IV iloprost regimen.
abstract: 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.
authors: Rheumatology Unit, A.O.U. Policlinico Vittorio Emanuele, Catania, Italy. rosfoti@tiscali.it.
authors: Rosario
authors: R
authors: Foti
authors: Rheumatology Unit, A.O.U. Policlinico Vittorio Emanuele, Catania, Italy.
authors: Elisa
authors: E
authors: Visalli
authors: Rheumatology Unit, A.O.U. Policlinico Vittorio Emanuele, Catania, Italy.
authors: Giorgio
authors: G
authors: Amato
authors: Rheumatology Unit, A.O.U. Policlinico Vittorio Emanuele, Catania, Italy.
authors: Alessia
authors: A
authors: Benenati
authors: Rheumatology Unit, A.O.U. Policlinico Vittorio Emanuele, Catania, Italy.
authors: Giovanni
authors: G
authors: Converso
authors: Medical Affairs Department, Italfarmaco S.p.A., Milan, Italy.
authors: Alberto
authors: A
authors: Farina
authors: Division of General Thoracic Surgery, A.O.U. Policlinico Vittorio Emanuele, Catania, Italy.
authors: Salvatore
authors: S
authors: Bellofiore
authors: Division of Cardiology, A.O.U. Policlinico Vittorio Emanuele, Catania, Italy.
authors: Massimiliano
authors: M
authors: Mulè
authors: Rheumatology Unit, A.O.U. Policlinico Vittorio Emanuele, Catania, Italy.
authors: Marcella
authors: M
authors: Di Gangi
mesh_terms: D000328:Adult; D000368:Aged; D018450:Disease Progression; D005260:Female; D006801:Humans; D016285:Iloprost / Q000008:administration & dosage / Q000627:therapeutic use*; D007262:Infusions, Intravenous; D008875:Middle Aged; D011928:Raynaud Disease / Q000188:drug therapy* / Q000209:etiology; D012595:Scleroderma, Systemic / Q000150:complications / Q000188:drug therapy*; D012883:Skin Ulcer / Q000188:drug therapy* / Q000209:etiology; D016896:Treatment Outcome; D014665:Vasodilator Agents / Q000008:administration & dosage / Q000627:therapeutic use*
chemical_list: D014665:Vasodilator Agents; D016285:Iloprost
keywords: Digital ulcers; Iloprost; Raynaud’s phenomenon; Scleroderma; Systolic pulmonary arterial pressure