Rheumatology (Oxford). 2022 Sep 8:keac512. doi: 10.1093/rheumatology/keac512. Online ahead of print.
ABSTRACT
OBJECTIVES: Survival and death prognostic factors of systemic sclerosis (SSc) patients varied during the last 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.
METHODS: Patients who received a diagnosis of SSc after January 1st, 2009, and were longitudinally followed up in 4 Italian Rheumatologic Centres were retrospectively assessed up to December 31st, 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 = 2.76), diffuse cutaneous involvement (HR = 3.14), older age at diagnosis (HR = 1.08), PAH (HR = 3.21), ILD-associated PH (HR = 4.11), comorbidities (HR = 3.53), and glucocorticoid treatment (HR = 2.02).
CONCLUSIONS: In the last 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.
PMID:36074979 | DOI:10.1093/rheumatology/keac512
