Background Systemic sclerosis (SSc) is a systemic autoimmune disease with a variety of clinical findings that may shorten the life. Autoantibody tests and digital capilloroscopy facilitated to diagnose this life threating disease. 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.
Objectives We aimed to evaluate the factors affecting survival in SSc in 2 different rheumatology clinics.
Methods All patients who fulfilled the ACR 1980 criteria and classified with SSc since 2003 to 2014 were evaluated (totally 277 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 (249; 89.9%) outnumbered men (28; 10.1%) significantly. The mean age at the time of diagnosis was 50.7+13.8 years old. 159 (57.4%) patients were classified as diffuse SSc, 110 patients (39.7%) were limited SSc and 8 patients were diagnosed as sine scleroderma (2.9%). 40 (14.4%) of the SSc patients were smoking. Prominent clinical manifestations were sclerodactily (94%), telangiectasis (49.8%), digital ulcers (37.5%), pulmonary fibrosis (35.5%), pulmonary hypertension (33.9%), dysphagia (22.7%), auto-amputation (9%), pleural effusion (6.1%), renal crisis (2.5%), inflammatory myositis (1.8%). At the time of diagnosis 90.6% of SSc patients were ANA positive; 63 (22.7%) were anti-centromere positive, 86 (31%) were anti-Scl-70 positive and 19 (6.9%) were anti-RNP positive. Anti-Ro antibody was positive in 24 (8.7%) and anti-La antibody was positive in 6 (2.2%) patients. The mean follow-up from the time of diagnosis was 42.4+31.6 months and the median follow-up was 36 months (1-132 months). There were a total of 47 deaths in our cohort (17%).
Overall, 5 years survival was 80.1% and 10 years survival was 73.5%. The probability of survival was reduced in men (5-year survival: 62% vs 82%, not-significant, p=0.09), in patients with pulmonary fibrosis (5-year survival: 69.8% vs. 85.5%, p=0.008), renal crisis (28.6% vs 81.5%, p<0.001), pulmonary hypertension (5-year survival: 58% vs 89.7%, p<0.001) and in patients with essential hypertension (5-year survival: 36% vs 90.7%, p<0.001).
Cox regression analysis showed that male sex (OR: 2.38, 95%CI: 1.09-5.3, p=0.03), pulmonary hypertension (OR: 0.29, 95%CI: 1.64-3.4, p=0.001) and essential hypertension (OR: 5.07, 95%CI: 2.4-10.09, p<0.001) at the time of diagnosis was independent factors effecting survival in patients with SSc.
Conclusions In our SSc patients, we observed reduced survival in male SSc patients and patients with pulmonary hypertension and essential hypertension. Any antibody including anti-centromer and Scl-70 were not meaningful.
Disclosure of Interest None declared
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