title: Severe vascular complications in patients affected by systemic sclerosis cyclically treated with iloprost.
abstract: The objective of this study was to evaluate the incidence of the most severe vascular complications, such as pulmonary arterial hypertension, scleroderma renal crisis, and digital necrosis requiring amputation, in a monocentric group of systemic sclerosis (SSc) patients cyclically treated with intravenous iloprost. We reviewed the record-charts of 115 patients affected by SSc (18 men and 97 women, mean age 58.9.1 ± 14.2 years) regularly receiving iloprost for at least 3 years; the mean duration of the treatment was 98.8 ± 37.5 months (a total of 946.8 years of therapy). Demographic and clinical features were recorded. None of the patients died of SSc-associated vascular complications. After iloprost administration digital gangrene requiring amputation developed in 2 patients who had concomitant peripheral arterial disease (a total of 3 episodes; annual incidence of 0.31 for 100 years of iloprost therapy). Four patients were diagnosed with pulmonary arterial hypertension during iloprost treatment (annual incidence of 0.42 for 100 years of drug therapy); in none of the cases did the complication show a progressive course. No cases of scleroderma renal crisis were observed. With the limits of an observational study and in the absence of a control group, our experience suggests that prolonged cyclic iloprost therapy may limit the incidence/progression of severe digital and visceral SSc-vasculopathy.
authors: Rheumatology Unit, Department of Medicine, Piazzale Scuro, 37134 Verona, Italy. paola.caramaschi@ospedaleuniverona.it
authors: Paola
authors: P
authors: Caramaschi
authors: Alessandra
authors: A
authors: Dalla Gassa
authors: Daniele
authors: D
authors: Prati
authors: Giovanni
authors: G
authors: Barausse
authors: Ilaria
authors: I
authors: Tinazzi
authors: Viviana
authors: V
authors: Ravagnani
authors: Silvia
authors: S
authors: Confente
authors: Domenico
authors: D
authors: Biasi
mesh_terms: D000328:Adult; D000368:Aged; D000671:Amputation, Surgical; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D005383:Finger Injuries / Q000601:surgery; D005734:Gangrene / Q000209:etiology / Q000517:prevention & control / Q000601:surgery; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology / Q000517:prevention & control; D016285:Iloprost / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D058729:Peripheral Arterial Disease / Q000188:drug therapy / Q000209:etiology; D012595:Scleroderma, Systemic / Q000150:complications / Q000188:drug therapy*; D012720:Severity of Illness Index; D014034:Toes / Q000601:surgery; D014665:Vasodilator Agents / Q000627:therapeutic use*
chemical_list: D014665:Vasodilator Agents; D016285:Iloprost