Background: Systemic sclerosis (SSc) is a devastating disease that has a profound impact on life expectancy, reflected by a standardised mortality ratio of 3,5. There is still limited data regarding the predictive factors for mortality in patients with SSc. Determining those factors could guide in disease management and follow up.¹

Objectives: We aimed to identify the predictive factors for death in SSc.

Methods: Patients followed in a tertiary rheumatology clinic in the last 5 years were included in this retrospective study. All of the patients met the ACR / EULAR SSc 2013 criteria. Medical records of the patients were reviewed. Follow up time was defined as the time period from the first admission of the patient to our rheumatology clinic until the date of death or the date on which the study was performed. Candidate predictive factors for mortality were tested by Kaplan-Meier (with Log rank) and Cox-regression analyses.

Results: In total 146 patients (mean age 55.6±12.3 years, female 89.7%, diffuse cutaneous type SSc 45.2%) were included in the study (Table 1). The mean age at diagnosis of study group was 48±13.7 years. The median duration of follow up was 71 (6-228) months. Fourteen (10%) patients died during follow-up. The causes of death were: pulmonary (7), renal (2) and cardiac diseases (1), infection (3) and cancer (1).

Univariate analysis revealed that age at diagnosis (p=0.028), SSc subtype (p=0.035), the presence of interstitial lung disease (p=0.002), oesophageal involvement (on computed tomography) (p=0.030), pulmonary artery systolic pressure of ≥35 mmHg (measured by transthoracic echocardiography) (p=0.004), glucocorticoid (p=0.029), hydroxychloroquine (p=0.002) and cyclophosphamide (p=0.006) usage at any time were associated with mortality (Figure 1). Multivariate analyses model formed with age at diagnosis (B: 0.055, 95% CI, 1.005-1.112; p=0.033), SSc subtype (B: 0.963, 95% CI 0.541-12.684; p=0.231), glucocorticoid (B: 1.396, 95% CI, 0.487-33.507; p=0.196) and hydroxychloroquine usage (B: -1.50, 95% CI, 0.061-0.816; p=0.023) showed that age at diagnosis and hydroxychloroquine usage were independent predictive factors for mortality in patients with SSc.

Conclusion: The results of the study revealed for the first time that apart from the age at diagnosis hydroxychloroquine might reduce mortality in patients with SSc. Further studies are needed to prove of this information.

References: [1]Elhai M, et al. Ann Rheum Dis 2017;0:1–9. doi:10.1136/annrheumdis-2017-211448

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Figure 1.

Disclosure of Interests: None declared.