Background/Purpose: Systemic sclerosis (SSc) is a multisystem, chronic, autoimmune disease. In this hospital based study, we aimed to evaluate the prevalence and incidence of SSc in our region.

Methods: During the 2003-2015, SSc patients diagnosed in our Department were included. Our Department is the only tertiary center of Rheumatology in this northwestern part of Turkey. The diagnosis of SSc was performed according to ACR 1980 criteria. Clinical and demographical data about SSc patients were obtained from medical charts.

Results: Of the 161 patients (146F, 15M) carrying the diagnosis the diagnosis of SSc, During the study period, the mean annual incidence rate was 2.16/100,000 for SSc. The annual incidence of SSc in women was 3.98/100,000, and in men was 0.4/100,000. In second period of study (2010-2015 vs. 2003-2009), general incidence rate increased especially in males (from 0.27 to 0.53/100,000). By December 2014, the overall point prevalence of SSc in our region was 23.2/100000 in population aged ˃16 years. The point prevalence in women (42.8/100000) was higher than the point prevalence in men (4.1/100000). Among women, SSc was more prevalent in the fourth and fifth decades of life. Among men, the peak orevalence was in the sixth decade of life. The period prevalence of SSc was 25.9/100000. The period prevalence in women was 47.7/100000 and in men it was 4.8/100000. The median age at diagnosis was 49 (21–84) years for SSc patients. 123 patients (76.4%) had limited cutaneous, 35 patients (22.4%) had diffuse SSc and 2 patients (1.2%) had sine scleroderma. Interstitial lung disease was detected in 55 patients (34.2%), pulmonary hypertension in 69 patients (42.9%), digital ulcers in 46 patients (28.6), arthritis in 47 patients (29.2%) and renal crisis in 2 patients.ANA positivity was detected in 90.1% of patients. Anti-centromer was positive in 26.7% of patients, anti-Scl-70 was positive in 28% of patients, anti-Ro in 15% of patients and anti-RNP in 11.9% of patients. The median follow-up time of all SSc patients was 48 months (3-160). 20 patients with SSc were died (16 females, 4 males). 5 and 10-years survivals of patients with SSc were 93.6% and 83.1% respectively. Univariate analysis revealed that, male sex (p=0.018), age older than >65 years at the time of diagnosis (p=0.016), pulmonary hypertension (p=0.005), digital ulcer (p=0.034), diffuse cutaneous disease (p=0.032), low level FVC (p=0.023) and anti-Scl-70 positivity (p=0.05) were poor prognostic factors. Multivariate Cox regression analysis showed that, male sex (p=0.05) and pulmonary hypertension (p=0.032) were independent poor prognostic factors in our SSc patients.

Conclusion: In conclusion, we found in our study from northwestern part of European Turkey that SSc prevalence (23.2/100,000) and incidence (2.1/100,000) is similar to data form European countries and US studies.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/the-incidence-and-prevalence-of-systemic-sclerosis-in-northwestern-part-of-turkey/