Background/Purpose: Systemic sclerosis (SSc) is a low-prevalence disease, characterized by fibrosis and vascular changes. It is the auto-immune rheumatic disease with the highest disease-related mortality and morbidity. Diagnosis of SSc is based on a set of clinical, immunological (specific antibodies) and capillaroscopic features. The progression in treatment modalities and supportive care methods yielded a better survival. However, major organ involvement such as pulmonary hypertension and diffuse interstitial lung disease still carries severe risk for mortality. The survival data and causes of death in patients with SSc is still not available in Turkey. The aim of the present study was to analyze the survival from disease onset and risk factors associated with mortality in 2 different rheumatology clinics of Turkish patients.

Methods: All patients who fulfilled the ACR 1980 criteria and classified with SSc since 2003 to 2016 were evaluated (totally 409 patients). The demographic features including autoantibodies of SSc patients and the mortality date were recorded from medical charts. Factors affecting the survival were calculated statistically by Kaplan-Meier test.

Results: Women (366; 89.5%) outnumbered men (43; 10.5%) significantly. The mean age at the time of diagnosis was 50.6+13.5 years. 241 (58.9%) patients were classified as diffuse SSc, 151 patients (36.9%) were limited SSc and 17 (4.1%) patients were diagnosed as sine scleroderma. 43 (10.5%) of the SSc patients were smoking. Prominent clinical manifestations were sclerodactily (95%), telangiectasis (42.8%), digital ulcers (38.5%), pulmonary fibrosis (40.5%), pulmonary hypertension (37.9%), dysphagia (19.7%), auto-amputation (7%), pleural effusion (5.1%), renal crisis (2.2%), inflammatory myositis (1.1%). At the time of diagnosis 94.6% of SSc patients were ANA positive; 95 (23.2%) were anti-centromere positive, 126 (30,8%) were anti-Scl-70 positive and 21 (5.1%) were anti-RNP positive. Anti-Ro antibody was positive in 34 (8.3%) and anti-La antibody was positive in 7 (1.7%) patients. The mean follow-up from the time of diagnosis was 51.7+38.6 months and the median follow-up was 48 months (1-300 months). There were a total of 54 deaths in our cohort (13.2%). Overall, 5 years survival was 85.1% and 10 years survival was 75.5%. The probability of survival was reduced in men (5-year survival: 62.1% vs 72%, not-significant, p=0.09), in patients with pulmonary fibrosis (5-year survival: 67.8% vs 83.5%, not-significant p=0.81), renal crisis (40.6% vs 80.5%, p<0.001), pulmonary hypertension (5-year survival: 50% vs 70.7%, p<0.001), coronary arter disease (54.6% vs 83.5%, p<0.001) and in patients with essential hypertension (5-year survival: 44% vs 84.7%, p=0.003) (table 1). Cox regression analysis showed that pulmonary hypertension (OR: 0.39, 95%CI: 1.74-3.5, p=0.007) at the time of diagnosis was an independent factor effecting survival in patients with SSc.

Conclusion: In our SSc patients, we observed reduced survival in patients with pulmonary hypertension, coronary arter disease and essential hypertension. Any antibody including anti-centromer and Scl-70 were not meaningful. Table 1. Survival of Systemic Sclerosis Patients According to Clinical Features

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/the-survival-and-prognostic-factors-of-patients-with-systemic-sclerosis-turkish-experience/