Background Systemic Sclerosis (SSc) is a rare, multisystemic connective tissue disease with significant morbidity. Prevalence and incidence of SSc varies worldwide (0.7–265 cases per 100,000/y1,2 and 0.06–12.2 cases per 100,000/y1 respectively). Mortality in SSc patients remains 3.5 times higher than the general population3. We sought to generate updated data on the epidemiology and burden of SSc in the Greek population.
Objectives To study the prevalence and incidence of SSc, describe the clinical characteristics and assess mortality and main causes of death in Crete (Greece) over a 5-year period (2010–2015).
Methods We conducted a cohort Study in defined geographic area (6,5% of Greek population). We reviewed demographics, clinical features, autoantibodies status and the causes of death from the Scleroderma cohort followed at the Rheumatology clinic of the University Hospital, Heraklion. The cause of death was categorized as related or not to scleroderma. Incidence and prevalence were estimated including patients living permanently in Crete who fulfilled SSc 2013 ACR/EULAR Classification Criteria for Scleroderma.
Results 72 patients (88% women, mean±SD age at diagnosis 48.5±16.7 years [range 15–87]) were identified, corresponding to a crude point prevalence of 13.8/105 (CI 95% 11–17/105) (December 2015). The Incidence rate was estimated at 0.05/105 per year (period 2010–2015). Diffuse SSc (dSSc) was present in 27.7%, limited SSc (lSSc) in 72.2%, while an overlap syndrome in 19.4% (9.7% with systemic lupus erythematosus). Frequencies of anti-Scl70 and anti-cectromere antibodies were 59.7% and 9.7%, respectively. Arthritis was present in 69.5%, lung involvement in 61% (9.7% pulmonary arterial hypertension [PAH]), whereas only a single patient developed renal crisis. 8.3% developed cancer. Case fatality rate during 2010–2015 was 9.7% (CI 95% 7.4–11.9) with an average (±SD) age of death at 65.2 (± 17.6) years. Mortality cases were related to SSc in 30.7%. The main cause of death was sepsis (30.8%) followed by PAH and cardiac arrest (15.4% each). Male gender (p<0.001) and presence of PAH (p=0.001) were related to mortality. Mean disease duration until death was 5.3 (±3.2) years in cases of SSc-related death, as compared to 18.3 (±11.0) years in deaths not related to SSc.
Conclusions SSc prevalence (13,8/105) in Crete is consistent with previous (2002) Greek studies (15,4/105)4 albeit lower compared to other European countries and USA5. Despite its rarity, the disease has significant burden. The SSc-related mortality occurs early in the disease course. Early recognition and treatment may decrease disease burden and improve the outcomes.
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Disclosure of Interest None declared