In this multicenter, retrospective study we evaluate the clinico-epidemiologic and prognostic features of a large Italian systemic sclerosis (SSc) series (1,012 patients, 897 females and 115 males; mean age at presentation, 50.5 yr ± 13.8 SD; mean follow-up, 7.1 yr ± 5.7 SD) recruited between 1955 and 1999 at 3 university-based rheumatology units, from the north (University of Padova), center (University of Pisa), and south (University of Napoli) of Italy. Limited cutaneous SSc was the most frequent subset with the best prognosis independent of the classification used, based on skin sclerosis extent (2- or 3-subset models). The percentages of various organ involvement significantly increased at the last patient evaluation. The progression of the disease during follow-up was mirrored by the constant decrease in the cumulative survival rates (Kaplan-Meier method) calculated at the 10th and 20th year from diagnosis (69.2% and 45.5%, respectively, p < .00001); the observed SSc survival rates were significantly lower than those expected in the Italian general population (p < .00001). Among SSc patients, significantly worse prognosis was observed in the diffuse cutaneous subset (p < .00001), in male gender (p < .00001), and in patients with lung (p < .00001), heart (p < .00001), and renal involvement (p < .00001). A shorter duration of Raynaud phenomenon before the scleroderma onset was correlated with worse outcome (p < .0164). With regards to serologic markers, the presence or absence of anti-centromere antibody was an important prognostic indicator (85.9% vs 72.7% 10th-year survival, respectively; p < .0004). Univariate and multivariate analysis by Cox proportional hazard regression model further confirmed the results of survival study: the mortality risk was significantly increased in male patients; in patients with diffuse cutaneous SSc; in patients with lung, heart, and kidney involvement; and in patients with abnormally high erythrocyte sedimentation rate (ESR) (> 25 mm/h) evaluated at patient enrollment. Thirty percent of patients died during the follow-up period; the most frequent causes of death were cardiac (36%) and lung (24%) involvement, and cancer (15%). Deaths were definitely or possibly related to SSc in 36% and 52% of cases, respectively. Renal involvement was a relatively rare complication in Italian SSc patients; comparable features were observed in other SSc populations from the Mediterranean area. Patients recruited after 1985 showed a significantly better 10th-year survival rate compared with subjects referred before 1985 (76.8% vs 60.6%, p < .0001). Comparable survival rates have been reported in recent studies on SSc series from other countries. This finding could be related to the wider recruitment of mild-to-moderate clinical variants at specialist centers, which better reflects the entire scleroderma spectrum, and, not secondarily, to the possible contribution of recently available therapies.
