TY - JOUR
T1 - Survival and prognostic factors from a multicentre large cohort of unselected Italian systemic sclerosis patients
AU - Cacciapaglia, Fabio
AU - Airò, Paolo
AU - Fornaro, Marco
AU - Trerotoli, Paolo
AU - De Lorenzis, Enrico
AU - Corrado, Addolorata
AU - Lazzaroni, Maria Grazia
AU - Natalello, Gerlando
AU - Montini, Fabio
AU - Altomare, Alberto
AU - Altomare, Annamaria
AU - Urso, Livio
AU - Verardi, Lucrezia
AU - Bosello, Silvia Laura
AU - Cantatore, Francesco Paolo
AU - Iannone, Florenzo
PY - 2023
Y1 - 2023
N2 - Objectives: Survival and death prognostic factors of SSc patients varied during the past decades. We aimed to update the 5- and 10-year survival rates and identify prognostic factors in a multicentre cohort of Italian SSc patients diagnosed after 2009. Material and methods: Patients who received a diagnosis of SSc after 1 January 2009 and were longitudinally followed up in four Italian rheumatologic centres were retrospectively assessed up to 31 December 2020. Overall survival of SSc patients was described using the Kaplan–Meier method. Predictors of mortality at 10-year follow-up were assessed by the Cox regression model. A comparison of our cohort with the Italian general population was performed by determining the standardized mortality ratio (SMR). Results: A total of 912 patients (91.6% females, 20% dcSSc) were included. Overall survival rates at 5 and 10 years were 94.4% and 89.4%, respectively. The SMR was 0.96 (95% CI 0.81, 1.13), like that expected in the Italian general population. Pulmonary arterial hypertension (PAH) and interstitial lung disease (ILD) associated with pulmonary hypertension (PH) significantly reduced survival (P < 0.0001). Main death predictors were male gender (HR ¼ 2.76), diffuse cutaneous involvement (HR ¼ 3.14), older age at diagnosis (HR ¼ 1.08), PAH (HR ¼ 3.21), ILD-associated PH (HR ¼ 4.11), comorbidities (HR ¼ 3.53) and glucocorticoid treatment (HR¼ 2.02). Conclusions: In the past decade, SSc patients have reached similar mortality of that expected in the Italian general population. Male gender, diffuse cutaneous involvement, comorbidities and PAH with or without ILD represent the main poor prognostic factors.
AB - Objectives: Survival and death prognostic factors of SSc patients varied during the past decades. We aimed to update the 5- and 10-year survival rates and identify prognostic factors in a multicentre cohort of Italian SSc patients diagnosed after 2009. Material and methods: Patients who received a diagnosis of SSc after 1 January 2009 and were longitudinally followed up in four Italian rheumatologic centres were retrospectively assessed up to 31 December 2020. Overall survival of SSc patients was described using the Kaplan–Meier method. Predictors of mortality at 10-year follow-up were assessed by the Cox regression model. A comparison of our cohort with the Italian general population was performed by determining the standardized mortality ratio (SMR). Results: A total of 912 patients (91.6% females, 20% dcSSc) were included. Overall survival rates at 5 and 10 years were 94.4% and 89.4%, respectively. The SMR was 0.96 (95% CI 0.81, 1.13), like that expected in the Italian general population. Pulmonary arterial hypertension (PAH) and interstitial lung disease (ILD) associated with pulmonary hypertension (PH) significantly reduced survival (P < 0.0001). Main death predictors were male gender (HR ¼ 2.76), diffuse cutaneous involvement (HR ¼ 3.14), older age at diagnosis (HR ¼ 1.08), PAH (HR ¼ 3.21), ILD-associated PH (HR ¼ 4.11), comorbidities (HR ¼ 3.53) and glucocorticoid treatment (HR¼ 2.02). Conclusions: In the past decade, SSc patients have reached similar mortality of that expected in the Italian general population. Male gender, diffuse cutaneous involvement, comorbidities and PAH with or without ILD represent the main poor prognostic factors.
KW - interstitial lung disease
KW - prognostic factors
KW - survival
KW - SSc
KW - pulmonary hypertension
KW - interstitial lung disease
KW - prognostic factors
KW - survival
KW - SSc
KW - pulmonary hypertension
UR - http://hdl.handle.net/10807/237505
U2 - 10.1093/rheumatology/keac512
DO - 10.1093/rheumatology/keac512
M3 - Article
SN - 1462-0324
VL - 62
SP - 1552
EP - 1558
JO - Rheumatology
JF - Rheumatology
ER -