Long-term clinical stabilization of scleroderma patients treated with a chronic and intensive IV iloprost regimen

Rheumatology International, Oct 2016

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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Long-term clinical stabilization of scleroderma patients treated with a chronic and intensive IV iloprost regimen

Rheumatol Int Long?term clinical stabilization of scleroderma patients treated with a chronic and intensive IV iloprost regimen Rosario Foti 0 1 3 4 Elisa Visalli 0 1 3 4 Giorgio Amato 0 1 3 4 Alessia Benenati 0 1 3 4 Giovanni Converso 0 1 3 4 Alberto Farina 0 1 3 4 Salvatore Bellofiore 0 1 3 4 Massimiliano Mul? 0 1 3 4 Marcella Di Gangi 0 1 3 4 0 Division of Cardiology, A.O.U. Policlinico Vittorio Emanuele , Catania , Italy 1 Rheumatology Unit, A.O.U. Policlinico Vittorio Emanuele , Catania , Italy 2 Rosario Foti 3 Division of General Thoracic Surgery, A.O.U. Policlinico Vittorio Emanuele , Catania , Italy 4 Medical Affairs Department, Italfarmaco S.p.A. , Milan , Italy 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. Raynaud's phenomenon; Scleroderma; Iloprost; Systolic pulmonary arterial pressure; Digital ulcers Introduction Scleroderma (systemic sclerosis or SSc) is a severe, chronic disease characterized by small vessel vasculopathy, autoantibodies production, and fibroblast dysfunction leading to an excessive deposition of collagen in the skin and internal organs [ 1, 2 ]. Severe Raynaud?s phenomenon (RP) is the early onset symptom in most SSc patients and may precede other clinical manifestations of the disease by many years [ 3 ]. The clinical course of the disease often involves the cardiovascular and respiratory systems, which can be severely damaged by SSc. The heart can be directly or indirectly involved, with myocardial damage, or with the involvement of other organs, especially kidneys and lungs, respectively [ 4 ]. For the respiratory system, SSc can affect lung parenchyma and pulmonary blood vessels, leading to interstitial lung disease (ILD), which may progress to pulmonary arterial hypertension (PAH). The presence of a cardio-pulmonary involvement generally leads to a poor prognosis for the patient [ 2 ]. Patients with significant internal organ involvement remain often asymptomatic until the late stages of systemic sclerosis; therefore, routine monitoring for the underlying disease and an intensive medical treatment are essential after the first diagnosis. Despite recent advances in the disease management, systemic sclerosis remains a treatable but not curable disease [ 2 ]. In the present paper, we report our experience with intravenous iloprost, a stable prostacyclin analogue indicated for the treatment of secondary RP, which may have a favourable effect on the disease progression. Iloprost shows vasodilating, anti-platelet, cytoprotective, and immunomodulating properties, with a long-lasting beneficial effect on the microcirculation [ 5 ]. The European League Against Rheumatism (EULAR) guidelines recommend iloprost as a first-line drug for the treatment of SSc-related digital vasculopathy in order to reduce the frequency and severity of SSc-RP attacks and to heal active digital ulcers (DUs) in patients with SSc, representing the gold standard in the treatment of active ulcers [ 6 ]. Moreover, recent studies have described a favourable disease course in SSc patients regularly treated with iloprost, with a low occurrence of the most severe vascular complications such as PAH, renal crisis, and digital necrosis [ 7?12 ]. The aim of the present study is to evaluate the disease progression, specifically in terms of cardiopulmonary function, in a group of consecutive SSc patients treated with iloprost, at t (...truncated)


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Rosario Foti, Elisa Visalli, Giorgio Amato, Alessia Benenati, Giovanni Converso, Alberto Farina, Salvatore Bellofiore, Massimiliano Mulè, Marcella Di Gangi. Long-term clinical stabilization of scleroderma patients treated with a chronic and intensive IV iloprost regimen, Rheumatology International, 2016, pp. 245-249, Volume 37, Issue 2, DOI: 10.1007/s00296-016-3582-4