Abstract

Background: Systemic sclerosis (SSc) is a heterogeneous disease with diverse clinical phenotypes. Anemia has been implicated as an independent prognosis factor for mortality and organ complications in systemic sclerosis (SSc). Aims: To determine the characteristics of SSc patients with anemia in a population of patients from Peking University People’s Hospital SSc cohort (PKUPH-SSc), and associated risk factors. Methods: Patients were recruited from PKUPH-SSc between January, 1999 and January, 2020. The diagnosis of anemia was based on the reduced hemoglobin (Hb) concentrations (<120g/L for female and <130g/L for male) during follow-up. The demographics, clinical characteristics, laboratory parameters, autoantibodies and medication were collected. Logistic regression analysis and Cox regression analysis was applied to identify the risk factors independently associated with anemia in SSc. Results: A total of 302[Office1] patients were enrolled in PKUPH-SSc cohort, 150 (49.8%) patients developed anemia, containing more female (n=136, 90.7%) [Office2] patients. Normocytic-mild anemia[Office3] (n=115, 76.7%) was the most common type of anemia in SSc. According to the age-adjusted logistic regression analysis, gender of female (OR=4.77, 95%CI 2.09-10.92, p<0.001), SSc with overlap syndrome (OR=3.42, 95%CI 1.55-7.53, p=0.002), severe skin thickness (OR=3.04, 95%CI 1.44-6.41, p=0.004), ESR elevation (OR=2.22, 95%CI 1.20-4.11, p=0.011), CRP elevation (OR=2.20, 95%CI 1.03-4.69, p=0.042) and hypocomplementemia (OR=3.58, 95%CI 1.90-6.75, p<0.001) were independent risk factor determining the SSc patients with anemia[Office4]. In addition, as shown in figure, age at onset ≥ 49 years (HR=2.54, 95%CI 1.51-4.27, p<0.001) and SRC (HR=3.27, 95%CI 1.37-7.79, p=0.008) were proved to be the independent predictors to determine the 20-year incidence of anemia in SSc, while positive anti-centromere proteins (HR=0.42, 95%CI 0.24-0.76, p=0.003) seemed to be a protective factor. In terms of anemia subgroups, positive anti-Ro52 was a potential risk factor for microcytic anemia, but a protective factor for normocytic anemia. Summary/Conclusion: Anemia is associated with distinct clinical behaviors and disease progression in SSc. Age at disease onset ≥ 49 years, negative anti-centromere proteins antibody and SRC were independent predictors for 20-year incidence of anemia in SSc patients. Monitoring anemia in these patients was a potential management to evaluate the disease progression of SSc.

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