A cross-sectional study including a 180 sample of patients followed up in a Rheumatology outpatient clinic from Complejo Asistencial Universitario de León with the following diagnosis: Systemic Lupus Erythematosus (SLE), Sjogren's Syndrome (SS), Systemic Sclerosis (SSc) and Rheumatoid Arthritis (RA) fulfilling ACR/EULAR criteria matched by age, sex and disease duration were included. The inclusion criteria were: age over 18 years old and ≥5-year follow-up at the consultation. Patients who did not meet the criteria for a defined disease and overlap syndromes, deaths, and loss of follow-up were excluded.

Sociodemographic data, clinical data and analytical parameters and disease activity score at inclusion were included after reviewing medical records. A standard protocol of questionnaires, completed after a clinical interview with closed questions were performed. The items on the questionnaires were common to all diseases (including those regarding demographic data, traditional cardiovascular risk factors, and cardiovascular events). Traditional cardiovascular risk and cardiovascular events history were also collected.

Cardiovascular risk factors were established using the following World Health Organization (WHO) definitions8:

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  Arterial hypertension: systolic blood pressure (SBP) over 140mmHg or diastolic blood pressure (DBP) over 90mmHg or receiving antihypertensive treatment. Blood pressure was considered as the mean of 3 measurements.

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  Dyslipidemia: cholesterol, low-density lipoprotein, (LDL), triglycerides, and high-density lipoprotein (HDL) imbalance. Hypercholesterolemia as total cholesterol level>250mg/dl on two or more prospective occasions. Hypertriglyceridemia as serum triglyceride level>150mg/dl on two or more prospective occasions. Abnormalhigh-density lipoprotein (HDL) as <40mg/dl) and abnormal low-density lipoprotein (LDL); defined as >130mg/dl).

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  Obesity was defined as body mass index (BMI) over 30kg/m2.

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  Smoking was defined as current smoker and ex-smoker.

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  Diabetes mellitus was defined as diabetes requiring insulin/oral antidiabetic drugs treatment and/or the presence in at least two determinations of fasting glycemia higher than 126mg/dl.

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  Hyperuricemia was defined as serum uric acid concentrations over 6mg/dl for females, 7mg/dl for men or patients under uric acid lowering drugs treatment.

Cardiovascular events were evaluated retrospectively and included:

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  Cerebrovascular disease including ischemic attacks confirmed by brain CT and/or brain MRI and carotid artery disease on sonography.

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  Coronary artery disease included heart infarction defined by elevated cardiac enzyme levels and/or electrocardiogram; silent myocardial ischemia defined as myocardial ischemia in the absence of chest discomfort or other anginal equivalents; stable angina and unstable angina defined clinically during physical exertion.

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  Arrhythmia: including supraventricular arrhythmias, ventricular tachyarrhythmias and ventricular fibrillation, atrial fibrillation, and bradyarrhythmia.

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  Conduction defects: first-degree heart block, left anterior fascicular block, right and left, bundle branch block, and second- or third-degree AV block.

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  Arterial peripheral vascular disease: narrowing or blockage of the vessels that carry blood from the heart to the legs.

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  Venous thrombosis: deep vein thrombosis and pulmonary embolism.

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  Heart failure: structural and/or functional cardiac abnormality and corroborated by elevated natriuretic peptide levels and/or objective evidence of pulmonary or systemic congestion.

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  Valvular heart disease: the presence of stenosis and insufficiency/regurgitation involving the valves of the heart demonstrated by echocardiography.

According to the 2016 European Society of Cardiology guidelines, CV risk assessment was performed using the SCORE calculation. The SCORE is a simple, office-based screening tool that predicts the probability of developing CVD in the next 10 years based on sex, age, systolic blood pressure, smoking status, and the ratio of total cholesterol (TC)/high-density lipoprotein cholesterol (HDL-C). Subjects were classified as low risk (SCORE<1%), moderate risk (1% to <5%), high risk (5% to <10%), or very high risk (those with established CVD or diabetes and SCORE≥10%) for future CVD.9

The Charlson Comorbidity Index (CCI) assesses the presence of comorbidities by taking into account both the number and severity of pre-defined comorbid conditions: myocardial infarct, congestive heart failure, peripheral vascular disease, cerebrovascular disease, dementia, chronic pulmonary disease, connective tissue disease, peptic ulcer disease, mild liver disease, diabetes, hemiplegia, moderate or severe renal disease, diabetes with end organ damage, any tumor, leukemia, lymphoma, moderate or severe liver disease, metastatic solid tumor, AIDS. It can be used to predict short-term and long-term outcomes such as function, hospital length of stay and mortality rates. Based on the CCI score, the severity of comorbidity was categorized into three grades: mild, with CCI scores of 1–2; moderate, with CCI scores of 3–4; and severe, with CCI scores≥5.10

Disease activity was assessed in SLE, RA, SS and SSc at the time of inclusion in the study. SLEDAI (Systemic Lupus Erythematosus Disease activity index) was used for SLE, ESSDAI (EULAR Sjögren's syndrome (SS) disease activity index) for SS, DAS28 (Disease Activity Score Calculator for Rheumatoid Arthritis) for RA and EUSTAR activity index 2017 for SSc. Disease activities were graded as low (SLEDAI: 1–5, DAS28≤3.2, ESSDAI: 1–4, EUSTAR-AI<2.5), moderate (SLEDAI: 6–10; DAS28>3.2 and ≤5.1; ESSDAI: 5–13) and high (SLEDAI: ≥11; DAS28>5.1; ESSDAI>13, EUSTAR-AI≥2.5).11–14

As part of this study, an anonymous, self-administered questionnaire was distributed to the 180 patients included in our study. The questionnaire was developed by our research group, comprising physicians, researchers, and nurses, to assess patients’ perception of cardiovascular disease (CVD) risk and evaluate various dimensions: physical activity, tobacco use, alcohol use, nutrition, activities in daily living, beliefs about their diagnosis, lifestyle changes after diagnosis, and biometric measures (blood pressure, cholesterol, blood glucose, weight). The health risk questionnaire was completed during the visit, and it is included as supplementary data.

The questionnaire was divided in three parts:

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  Part 1: General information on survey participants including socio-demographic data, age of diagnosis, type of diagnosis, and current treatment with conventional/targeted synthetic disease-modifying antirheumatic drugs (c/tsDMARDs), biologic DMARDs (bDMARDs), and corticosteroids.

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  Part 2: Risk assessment in daily life, including alert symptoms related to CVD risk and personal and familial history assessed with a 5-point Likert scale. Cardiovascular alert symptoms were assessed by the questions “I feel chest pain without making a physical effort,” “I have trouble breathing when I move,” and “I feel my pulse racing without making a physical effort.”

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  Part 3: Knowledge of traditional CVD risk factors and individual health status and perceived CVD risk, assessed with a 5-point Likert scale. Physical activity was assessed by the question: “Exercising two to three times per week is part of my routine”. Lifestyle was assessed through the questions “Having a healthy lifestyle is important to me”, “I avoid foods with high salt content”, “I avoid foods with high sugar content”, “I avoid excessive alcohol consumption” and “I avoid exposure to tobacco”. Self-perceived risk was evaluated with statements like “My autoimmune disease does not increase the risk of having a heart attack if I have no other risk factors,” “Since my diagnosis, I have made significant changes to my lifestyle,” and “I do not make adjustments to my treatment without consulting my doctor first.” Categorical risk perception was assessed with a 5-point Likert scale using the question “Compared to other people of the same age and sex as you, how do you perceive your risk of developing a CVD in the future in the next ten years?” It was collapsed into three categories: low perception (including much lower risk, slightly lower, and neither lower nor higher risk), moderate perception (slightly higher), and higher than others (moderately higher and much higher).

Numerical variables were summarized using mean and standard deviations, while categorical variables were presented as absolute and relative frequencies. The prevalence of traditional cardiovascular events and cardiovascular risk factors was calculated for each disease. The associations between SCORE and risk perception, and Charlson's morbidity index and risk perception were assessed using Spearman's rank correlation, while the relationship between Charlson's comorbidity index and disease activity was examined using a Chi-square test. All data analyses were performed using R version 4.3.015 and RStudio software,16 with statistical significance determined with α=0.05.

A total of 180 patients were included in the study, with 58.9% being female. The inclusion comprised 45 patients from each disease group (SSc, RA, SLE, and SS). Clinical and sociodemographic data are presented in Table 1. The participants had a mean age of 61 years, and 51.7% were over 60 years old. The average disease duration was 11.8 years. In terms of employment, 41.1% worked in the private sector, and 40.6% had completed secondary education. The mean age for SLE patients was 57.3±18.2 years, for SSc patients was 53.5±21 years, for SS patients was 58.4±23 years, and for RA was 55.2±17 years.

The prevalence of cardiovascular risk factors in patients with SSc, SS, RA and SLE are represented in Table 2. Arterial hypertension (55%) was the most prevalent risk factor followed by dyslipidemia (37.8%) and diabetes mellitus (12.8%).

Prevalence of cardiovascular events in patients with SSc, SS, SLE and RA are summarized in Table 3. Valvular heart disease (29%), arrhythmias (24%), and congestive heart failure (14.4%), as well as deep vein thrombosis (14.4%), were the most prevalent.

72 (40%) patients had low disease activity, 86 (48%) patients had moderate activity and 22 (12%) patients had high activity. CCI score grading was mild for 87 (48.3%) patients, moderate for 57 (31.7%), and high for 36 (20%). When analyzing an association between the comorbidity index and disease activity, we found no significant association between CCI and disease activity level (p=0.62). When the analysis was performed separately for each diagnosis, consistent results were obtained, except for Sjogren's syndrome, where a significant association between these comorbidity index and disease activity was found (p=0.02).

Results from part 3 of the survey are summarized in Tables 4 and 5 included in the supplementary data.

When assessing patients’ perceptions, 164 (91.1%) agreed that maintaining a healthy lifestyle was important to them. Only 71 (39.4%) stated that exercising 2–3 times per week was part of their routine. Additionally, 134 (74.5%) and 136 (75.6%) reported avoiding foods with high salt and sugar content, while only 122 (67.7%) avoided tobacco exposure. In contrast, 149 (82.8%) avoided alcohol consumption. Furthermore, 162 (90%) emphasized the importance of following their doctor's recommendations. However, only 81 (45%) believed that their autoimmune disease could increase their risk of a heart attack, even without other risk factors. A total of 116 (64.4%) patients made significant lifestyle changes after diagnosis. Additionally, 138 (76.7%) reported not altering their treatment without consulting their doctor, while 42 (23.3%) admitted to independently increasing corticosteroid doses during a flare. Notably, 84 (46.7%) were unaware that NSAIDs pose a cardiovascular risk factor.

Regarding the perception of the risk of developing cardiovascular diseases in the next ten years, 36 (20%) considered it lower, 41 (23%) neither lower nor higher, and 56% perceived it as higher. Categorizing patients by risk level, 100 (45%) were classified as low risk, 67 (37%) as moderate risk, 23 (12.8%) as high risk, and 11 (5.6%) as very high risk.

When assessing the association between the patient's SCORE index and their perception of CV risk, we find that all diseases present a correlation between these two variables (p<0.05), except for patients with SLE (p=0.27).

Mean SCORE was 2.3±1.98 for SLE; 1.92±1.8 for RA; 1.72±0.82 for SS and 2.01±1.2 for SSc. Fig. 1 reflects the association between the SCORE index represented by the different colors (low as green, medium as purple, high as orange and very high as yellow) and the patient's perception of CV risk stratified by disease represented by the abscissa (low, medium and high). There was also a significant association between comorbidity index measured by CCI and risk perception for all the disease groups (p<0.001).

Fig. 1.

Association between SCORE index and patient's perception of CV risk stratified by disease.

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When analyzing the influence of age, working status and education in CVD risk perception, we found an association between CVD risk perception and age (p=0.01). Patients over 40 years of age exhibited a higher perception of CVD risk perception than younger patients. Meanwhile, no differences were found regarding working status (p=0.19) nor education level (p=0.06).