Abstract
It is well documented that patients with systemic lupus erythematosus (SLE) are at an increased risk of atherosclerotic cardiovascular (CV) disease. There is evidence that traditional risk factors and disease-related factors are involved in this increased risk. Less is known about CV risk and outcomes in other connective tissue diseases (CTDs). Future longitudinal observational studies may help to answer these important questions; however, because CTDs are rare, collaboration between clinicians with similar research interests is needed to ensure sufficiently large cohorts are available to address these issues.
Here, we review the evidence available for CV risk in CTDs and discuss the benefits of longitudinal observational studies in identifying CV outcomes. Structured care protocols for the management of CV risk in CTDs are lacking. We propose a target-based approach to assessing and managing CV risk in CTDs.
Introduction
Patients with inflammatory musculoskeletal diseases are at an excess risk of atherosclerotic cardiovascular disease (ACVD) compared to the general population. A meta-analysis of 24 mortality studies in patients with rheumatoid arthritis (RA) reported a weighted combined all-cause standardised mortality ratio (meta-SMR) of 1.50 (95% 1.39–1.61) with similar increases for ischaemic heart disease (meta-SMR 1.59; 95% CI 1.46–1.73) and stroke (meta-SMR 1.52; 95% CI 1.40–1.67) . The bimodal pattern of death associated with systemic lupus erythematosus (SLE) was first described in 1976 , with the first mortality peak (within 1 year of diagnosis) being mainly due to active lupus and infection and the second peak (>5 years from diagnosis) being mainly due to cardiovascular events (CVEs). In 1997, Manzi et al. reported that the risk of myocardial infarction (MI) was >50 times higher in women aged 35–44 in the University of Pittsburgh lupus cohort than in age-matched women from the Framingham Offspring cohort .
In this chapter, we summarise the most recent studies on the risk of ACVD and the risk factors for ACVD in patients with SLE, systemic sclerosis (SSc), idiopathic inflammatory myopathies (IIMs), mixed connective tissue disease (CTD) and primary Sjögren’s syndrome (pSS). We performed a Medline search up until 30 November 2015 searching for ‘cardiovascular risk’ and ‘cardiovascular mortality’ and combined each with ‘systemic lupus erythematosus’, ‘systemic sclerosis’, ‘Sjogren’s syndrome’, ‘idiopathic inflammatory myopathy’ and ‘mixed connective tissue disease’. We have focussed on reporting relevant systematic reviews and papers published in the last 5 years.
We highlight the importance of longitudinal observational studies (LOSs) in contributing to this knowledge and on the value of embedding research in routine clinical practice. Finally, we review strategies for cardiovascular (CV) risk management in patients with CTDs.
CV disease in patients with SLE
SLE is a chronic inflammatory autoimmune condition with significant morbidity and mortality. The survival rates for SLE have improved significantly in recent years . In an international study of 9547 patients from 23 centres, the all-cause SMR estimates decreased significantly between the 1970s and 2001. However, the CV SMR increased slightly . CVEs remain one of the main causes of death in SLE.
CV mortality in patients with SLE
CV mortality is increased in SLE patients compared to the general population. In a meta-analysis of 27,123 SLE patients from 12 studies published before 2011, a threefold increased risk of all-cause mortality (meta-SMR 2.98, 95% CI 2.32–3.83) and an increased risk of death from cardiovascular disease (CVD) (meta-SMR 2.72, 95% CI 2.32–3.83) were noted . None of the studies of mortality published since 2011 has had sufficient numbers of deaths to be able to report a CV-specific SMR . The most recent study followed 2740 incident cases of SLE identified using the UK primary-care-based Clinical Practice Research Datalink and then linked to the national death register . The mortality rate ratio compared to age-, sex- and practice-matched controls was 1.67 (95%CI 1.43, 1.94). Another UK-based study followed up an inception cohort of 382 SLE patients recruited from 1989 to 2010. The all-cause SMR was 2.0 (95% CI 1.5, 2.8), with CVEs being one of the most common causes of death (27%) .
Ethnicity may play a role in the risk of CV mortality in SLE. A systematic review of five observational studies involving 4469 Chinese patients with SLE found lower rates of death from CV causes than has been documented in other ethnic groups . CVEs accounted for 11.5% of all deaths, whereas deaths from infection accounted for 33% of all deaths.
Traditionally, mortality studies have focussed on the underlying cause of death as recorded in the death certificate. A new methodology called multiple-cause-of-death analysis enables all the diagnoses recorded on the death certificate to be analysed. Two recently published studies have used this methodology in SLE. One study included 4815 death certificates from Sao Paulo, Brazil, on which SLE was listed as a cause of death between 1985 and 2005 . The most common contributory causes of death were renal failure and sepsis. Only in 2003–2007, and in individuals aged <50 years when they died, was CVD a more common cause of death than in the general population. The second study used death certificates from all 1593 adults who died in France between 2000 and 2009 and in whom SLE was mentioned on the death certificate . The mean (SD) age at death of the SLE patients was 63.5 (18.4) years. The age-standardised mortality rate fell from 4.1 per million in 2000 to 3.0 per million in 2009. Amongst patients in whom SLE was listed as the underlying cause of death, 49.5% had CVD listed as another main cause. In patients in whom SLE was listed as a non-underlying cause of death, CVD was listed as the underlying cause of death more frequently than in the general population (relative risk (RR) 1.58; 95% CI 1.42–1.76).
MI in patients with SLE
Patients with SLE have an increased risk of MI compared to the general population . Using health administrative databases from Canada between 1990 and 2007, Avina-Zubieta et al. found that the overall multivariable RR for MI in patients with physician-diagnosed SLE, compared with age- and sex-matched controls, was 2.5 (95% CI 2.1, 3.1) . The subsequent incidence of MI in 1207 patients with SLE from Taiwan was 2.10 per 1000 person-years (pyrs) compared with 0.49 in age- and sex-matched controls (adjusted hazard ratio (HR) 5.11 (95% CI 2.63, 9.92)) .
Patients with SLE have a higher prevalence of CVD even before diagnosis. The prior incidence of CVD was higher in 70 incident cases of SLE identified from The Marshfield Epidemiologic Study Area in Wisconsin, USA, than in matched controls (23% vs. 10%; OR 3.7 (1.8, 7.9)) .
A study from the US National Heart, Lung and Blood Institute Dynamic Registry of patients undergoing percutaneous coronary interventions (PCIs) found no significant differences in the mean percent of coronary artery stenosis and total occlusion in SLE versus non-SLE subjects . However, SLE patients had significantly worse CV outcomes at 1 year following PCI including a higher risk of MI (15.6% vs. 4.8%, p = 0.01) and repeat PCI (31.3% vs. 11.8%, p = 0.009). A retrospective cohort study from Taiwan found that SLE was an independent predictor of inpatient mortality after PCI (OR 3.81; 95% CI 2.02, 7.16) .
Congestive heart failure in patients with SLE
The risk of hospitalisation for congestive heart failure (CHF) in SLE appears to be substantially increased. Using the California Hospital Discharge Database, Ward found that the proportionate morbidity ratio for CHF in women with SLE compared to women without SLE was 3.22 (95% CI 2.55, 4.05) in those aged 18–44 years, 1.34 (95% CI 1.14, 1.56) in those aged 45–64 years and 1.30 (95% 1.16, 1.45) in those aged ≥65 years . The nature of CHF in SLE is likely to be multifactorial. Of 24 episodes of CHF in a large international inception cohort of SLE followed up for up to 8 years, only five (21%) were attributed to atherosclerosis . CHF is more common in patients with SLE admitted with an MI compared to controls admitted with an MI (20% vs. 15%), and the presence of CHF in SLE patients admitted with an MI is a risk factor for mortality (17.9% vs. 5.8%) .
Stroke in patients with SLE
In a recent meta-analysis of 10 population-based cohort studies, the pooled RR in patients with SLE for all types of stroke combined was 2.53 (95% CI 1.96, 3.26) . The risks of ischaemic stroke (RR 2.10; 95% CI 1.68, 2.62), intracerebral haemorrhage (RR 2.72; 95% CI 2.15, 3.44) and subarachnoid haemorrhage (RR 3.85; 95% CI 3.20, 4.64) were all increased. The RR for stroke was highest in adults aged <50 years.
Peripheral arterial disease in patients with SLE
Peripheral arterial disease (PAD) is more common in patients with SLE than in the general population . A study from the National Health Insurance Research Database of Taiwan of >10,000 SLE patients reported a ninefold higher incidence of PAD than in non-SLE patients .
Subclinical atherosclerosis in patients with SLE
Subclinical atherosclerosis may manifest as endothelial dysfunction, arterial stiffness, a high carotid intima media thickness (cIMT) and plaque or coronary artery calcification (CAC). A recent meta-analysis showed that patients with SLE have a higher cIMT and an increased prevalence of carotid plaque compared with controls . Plaque and IMT measurement on carotid ultrasound in SLE can independently predict CVEs .
Risk factors for CVD in patients with SLE
The aetiology of the premature atherosclerosis seen in SLE is incompletely understood. Traditional cardiovascular risk factors (TRFs), disease-related factors and certain treatments play a part. Tselios et al. recently published a systematic review in which they identified 101 papers exploring risk factors for the development of atherosclerosis in SLE and satisfying their inclusion criteria . The following section summarises the results of this systematic review.
Traditional risk factors for CVD in patients with SLE
Classic TRFs include increasing age, male gender, smoking status, diabetes, hypertension and dyslipidaemia. A number of these factors are more common in patients with SLE including hypertension and diabetes, but not smoking or hypercholesterolaemia . More recently described TRFs in the general population include a positive family history of CVD, high levels of high-sensitivity C-reactive protein (hs-CRP), ethnicity, low levels of physical activity, obesity and the metabolic syndrome (MetS). MetS is a clustering of CV risk factors including abdominal (central) obesity, elevated blood pressure, elevated fasting plasma glucose, high serum triglyceride levels and low high-density lipoprotein (HDL) levels. There is a higher prevalence of MetS in patients with SLE than in matched controls . There is evidence that SLE-related factors including active disease, damage and renal involvement are independently associated with MetS and that MetS is prevalent early in the course of the disease .
Among the non-modifiable TRFs, there is evidence that increasing age, postmenopausal status, male gender and a positive family history are independent risk factors for subclinical atherosclerosis and CVEs in patients with SLE .
Among the modifiable TRFs, obesity and arterial hypertension are independent risk factors for CVEs and subclinical atherosclerosis in patients with SLE . Smoking has been shown to be associated with CVEs, carotid plaques and CAC in SLE. Total cholesterol has been shown to be an independent risk factor for CVEs and subclinical atherosclerosis. Although not an independent risk factor for CVE or CAC, high low-density lipoprotein (LDL) levels were an independent predictor of increased cIMT and plaque formation . MetS is associated with increased cIMT, CAC and arterial stiffness.
Disease-specific risk factors for CVD in patients with SLE
TRFs alone do not fully explain the increased risk of CVD seen in patients with SLE either as a group or individually. Moreover, in a study from the Toronto clinic, lupus patients with CVEs had more TRFs than those without events, but they did not have a higher Framingham risk score . SLE disease activity has been shown to be an independent risk factor for CVEs and subclinical atherosclerosis . Cumulative damage, long disease duration, double-stranded DNA (dsDNA) antibodies, anti-cardiolipin antibodies and lupus anticoagulants have also been shown to be independent predictors of CVEs and subclinical atherosclerosis .
Antiphospholipid syndrome (APS) frequently coexists with SLE . Patients diagnosed with both SLE and APS represent a more severe disease phenotype with a higher risk of CVD . Both proteinuria and renal impairment are independent predictors of CVE .
Treatment-related factors and the risk of CVD in patients with SLE
The negative effects of corticosteroids on CVE, hypertension, lipid profile and weight are well known. High cumulative steroid dose predicts plaque formation in patients with SLE . Current steroid use, independent of disease activity, also predicts CVE .
The protective role of hydroxychloroquine has been demonstrated in a number of studies. In a Chinese SLE cohort, lack of use of hydroxychloroquine was an independent risk factor for CVD . In a prospective study in the Toronto lupus cohort, patients who developed coronary artery disease (CAD) were significantly less likely to have been treated with hydroxychloroquine than those who did not develop CAD .
CV disease in patients with SLE
SLE is a chronic inflammatory autoimmune condition with significant morbidity and mortality. The survival rates for SLE have improved significantly in recent years . In an international study of 9547 patients from 23 centres, the all-cause SMR estimates decreased significantly between the 1970s and 2001. However, the CV SMR increased slightly . CVEs remain one of the main causes of death in SLE.
CV mortality in patients with SLE
CV mortality is increased in SLE patients compared to the general population. In a meta-analysis of 27,123 SLE patients from 12 studies published before 2011, a threefold increased risk of all-cause mortality (meta-SMR 2.98, 95% CI 2.32–3.83) and an increased risk of death from cardiovascular disease (CVD) (meta-SMR 2.72, 95% CI 2.32–3.83) were noted . None of the studies of mortality published since 2011 has had sufficient numbers of deaths to be able to report a CV-specific SMR . The most recent study followed 2740 incident cases of SLE identified using the UK primary-care-based Clinical Practice Research Datalink and then linked to the national death register . The mortality rate ratio compared to age-, sex- and practice-matched controls was 1.67 (95%CI 1.43, 1.94). Another UK-based study followed up an inception cohort of 382 SLE patients recruited from 1989 to 2010. The all-cause SMR was 2.0 (95% CI 1.5, 2.8), with CVEs being one of the most common causes of death (27%) .
Ethnicity may play a role in the risk of CV mortality in SLE. A systematic review of five observational studies involving 4469 Chinese patients with SLE found lower rates of death from CV causes than has been documented in other ethnic groups . CVEs accounted for 11.5% of all deaths, whereas deaths from infection accounted for 33% of all deaths.
Traditionally, mortality studies have focussed on the underlying cause of death as recorded in the death certificate. A new methodology called multiple-cause-of-death analysis enables all the diagnoses recorded on the death certificate to be analysed. Two recently published studies have used this methodology in SLE. One study included 4815 death certificates from Sao Paulo, Brazil, on which SLE was listed as a cause of death between 1985 and 2005 . The most common contributory causes of death were renal failure and sepsis. Only in 2003–2007, and in individuals aged <50 years when they died, was CVD a more common cause of death than in the general population. The second study used death certificates from all 1593 adults who died in France between 2000 and 2009 and in whom SLE was mentioned on the death certificate . The mean (SD) age at death of the SLE patients was 63.5 (18.4) years. The age-standardised mortality rate fell from 4.1 per million in 2000 to 3.0 per million in 2009. Amongst patients in whom SLE was listed as the underlying cause of death, 49.5% had CVD listed as another main cause. In patients in whom SLE was listed as a non-underlying cause of death, CVD was listed as the underlying cause of death more frequently than in the general population (relative risk (RR) 1.58; 95% CI 1.42–1.76).
MI in patients with SLE
Patients with SLE have an increased risk of MI compared to the general population . Using health administrative databases from Canada between 1990 and 2007, Avina-Zubieta et al. found that the overall multivariable RR for MI in patients with physician-diagnosed SLE, compared with age- and sex-matched controls, was 2.5 (95% CI 2.1, 3.1) . The subsequent incidence of MI in 1207 patients with SLE from Taiwan was 2.10 per 1000 person-years (pyrs) compared with 0.49 in age- and sex-matched controls (adjusted hazard ratio (HR) 5.11 (95% CI 2.63, 9.92)) .
Patients with SLE have a higher prevalence of CVD even before diagnosis. The prior incidence of CVD was higher in 70 incident cases of SLE identified from The Marshfield Epidemiologic Study Area in Wisconsin, USA, than in matched controls (23% vs. 10%; OR 3.7 (1.8, 7.9)) .
A study from the US National Heart, Lung and Blood Institute Dynamic Registry of patients undergoing percutaneous coronary interventions (PCIs) found no significant differences in the mean percent of coronary artery stenosis and total occlusion in SLE versus non-SLE subjects . However, SLE patients had significantly worse CV outcomes at 1 year following PCI including a higher risk of MI (15.6% vs. 4.8%, p = 0.01) and repeat PCI (31.3% vs. 11.8%, p = 0.009). A retrospective cohort study from Taiwan found that SLE was an independent predictor of inpatient mortality after PCI (OR 3.81; 95% CI 2.02, 7.16) .
Congestive heart failure in patients with SLE
The risk of hospitalisation for congestive heart failure (CHF) in SLE appears to be substantially increased. Using the California Hospital Discharge Database, Ward found that the proportionate morbidity ratio for CHF in women with SLE compared to women without SLE was 3.22 (95% CI 2.55, 4.05) in those aged 18–44 years, 1.34 (95% CI 1.14, 1.56) in those aged 45–64 years and 1.30 (95% 1.16, 1.45) in those aged ≥65 years . The nature of CHF in SLE is likely to be multifactorial. Of 24 episodes of CHF in a large international inception cohort of SLE followed up for up to 8 years, only five (21%) were attributed to atherosclerosis . CHF is more common in patients with SLE admitted with an MI compared to controls admitted with an MI (20% vs. 15%), and the presence of CHF in SLE patients admitted with an MI is a risk factor for mortality (17.9% vs. 5.8%) .
Stroke in patients with SLE
In a recent meta-analysis of 10 population-based cohort studies, the pooled RR in patients with SLE for all types of stroke combined was 2.53 (95% CI 1.96, 3.26) . The risks of ischaemic stroke (RR 2.10; 95% CI 1.68, 2.62), intracerebral haemorrhage (RR 2.72; 95% CI 2.15, 3.44) and subarachnoid haemorrhage (RR 3.85; 95% CI 3.20, 4.64) were all increased. The RR for stroke was highest in adults aged <50 years.
Peripheral arterial disease in patients with SLE
Peripheral arterial disease (PAD) is more common in patients with SLE than in the general population . A study from the National Health Insurance Research Database of Taiwan of >10,000 SLE patients reported a ninefold higher incidence of PAD than in non-SLE patients .
Subclinical atherosclerosis in patients with SLE
Subclinical atherosclerosis may manifest as endothelial dysfunction, arterial stiffness, a high carotid intima media thickness (cIMT) and plaque or coronary artery calcification (CAC). A recent meta-analysis showed that patients with SLE have a higher cIMT and an increased prevalence of carotid plaque compared with controls . Plaque and IMT measurement on carotid ultrasound in SLE can independently predict CVEs .
Risk factors for CVD in patients with SLE
The aetiology of the premature atherosclerosis seen in SLE is incompletely understood. Traditional cardiovascular risk factors (TRFs), disease-related factors and certain treatments play a part. Tselios et al. recently published a systematic review in which they identified 101 papers exploring risk factors for the development of atherosclerosis in SLE and satisfying their inclusion criteria . The following section summarises the results of this systematic review.
Traditional risk factors for CVD in patients with SLE
Classic TRFs include increasing age, male gender, smoking status, diabetes, hypertension and dyslipidaemia. A number of these factors are more common in patients with SLE including hypertension and diabetes, but not smoking or hypercholesterolaemia . More recently described TRFs in the general population include a positive family history of CVD, high levels of high-sensitivity C-reactive protein (hs-CRP), ethnicity, low levels of physical activity, obesity and the metabolic syndrome (MetS). MetS is a clustering of CV risk factors including abdominal (central) obesity, elevated blood pressure, elevated fasting plasma glucose, high serum triglyceride levels and low high-density lipoprotein (HDL) levels. There is a higher prevalence of MetS in patients with SLE than in matched controls . There is evidence that SLE-related factors including active disease, damage and renal involvement are independently associated with MetS and that MetS is prevalent early in the course of the disease .
Among the non-modifiable TRFs, there is evidence that increasing age, postmenopausal status, male gender and a positive family history are independent risk factors for subclinical atherosclerosis and CVEs in patients with SLE .
Among the modifiable TRFs, obesity and arterial hypertension are independent risk factors for CVEs and subclinical atherosclerosis in patients with SLE . Smoking has been shown to be associated with CVEs, carotid plaques and CAC in SLE. Total cholesterol has been shown to be an independent risk factor for CVEs and subclinical atherosclerosis. Although not an independent risk factor for CVE or CAC, high low-density lipoprotein (LDL) levels were an independent predictor of increased cIMT and plaque formation . MetS is associated with increased cIMT, CAC and arterial stiffness.
Disease-specific risk factors for CVD in patients with SLE
TRFs alone do not fully explain the increased risk of CVD seen in patients with SLE either as a group or individually. Moreover, in a study from the Toronto clinic, lupus patients with CVEs had more TRFs than those without events, but they did not have a higher Framingham risk score . SLE disease activity has been shown to be an independent risk factor for CVEs and subclinical atherosclerosis . Cumulative damage, long disease duration, double-stranded DNA (dsDNA) antibodies, anti-cardiolipin antibodies and lupus anticoagulants have also been shown to be independent predictors of CVEs and subclinical atherosclerosis .
Antiphospholipid syndrome (APS) frequently coexists with SLE . Patients diagnosed with both SLE and APS represent a more severe disease phenotype with a higher risk of CVD . Both proteinuria and renal impairment are independent predictors of CVE .
Treatment-related factors and the risk of CVD in patients with SLE
The negative effects of corticosteroids on CVE, hypertension, lipid profile and weight are well known. High cumulative steroid dose predicts plaque formation in patients with SLE . Current steroid use, independent of disease activity, also predicts CVE .
The protective role of hydroxychloroquine has been demonstrated in a number of studies. In a Chinese SLE cohort, lack of use of hydroxychloroquine was an independent risk factor for CVD . In a prospective study in the Toronto lupus cohort, patients who developed coronary artery disease (CAD) were significantly less likely to have been treated with hydroxychloroquine than those who did not develop CAD .
CVD in patients with SSc
SSc is an autoimmune CTD characterised by multiorgan fibrosis, vasculopathy triggered by endothelial injury as well as autoantibody production. Inflammation plays a less prominent role in SSc than in SLE. SSc may directly affect the heart leading to myocardial fibrosis, conduction defects, left ventricular systolic and diastolic dysfunction and pericardial disease . Heart disease may also occur secondary to pulmonary hypertension in SSc. Although microvascular disease is the hallmark of SSc, there is growing evidence that macrovascular damage, resulting in ACVD, contributes to excess morbidity and mortality .
CV mortality in patients with SSc
A meta-analysis including 9239 SSc patients from 17 studies published before 2005 reported an all-cause meta-SMR of 2.72 (95% CI 1.93, 3.83) . CVD-specific SMR was not reported. In the EULAR Scleroderma Trials and Research (EUSTAR) database, 55% of 234 deaths in SSc were directly attributable to the disease and 41% to non-SSc causes; 26% of the deaths were directly attributed to SSc and 29% of the non-SSc causes of death were due to CVD .
There is evidence of a higher case fatality for MI but not for stroke in patients with SSc compared to patients without CTD. Using the US Nationwide Inpatient Survey, Dave et al. found that 5.5% of 61,734 admissions in patients with SSc were for a CVD diagnosis or procedure compared with 4.9% of SLE admissions, 7.1% of RA admissions and 6.7% of control admissions. Patients with SSc and MI were more likely to die during that admission than patients with SLE and MI, patients with RA and MI or controls with MI .
Atherosclerotic CVD in patients with SSc
A number of large studies have reported an increased risk of ACVD in patients with SSc compared to the general population. A study of 850 patients with SSc from the Australian Scleroderma Cohort Study reported an odds ratio (OR) for self-reported coronary heart disease, after adjusting for TRFs, of 3.2 (95% CI: 2.3–4.5) compared with a control group from the Australian Diabetes, Obesity and Lifestyle Study and an OR of 2.0 (95% CI: 2.3–4.5) when compared with a cohort from the National Health Survey . In a study of 865 patients with SSc identified from a UK primary care database, the HR, after adjusting for TRFs and steroid use, was 1.8 (95% CI: 1.07–3.05) for MI, 2.61 (95% CI: 1.54–4.44) for stroke and 4.35 (95% CI: 2.74–6.93) for PAD compared to age- and sex-matched controls . The adjusted HR for MI between 1344 incident cases of SSc and 13,440 controls from Taiwan was 2.45 (95% CI 1.60, 3.75) .
Subclinical atherosclerosis in patients with SSc
A recent systematic review and meta-analysis reported on 31 studies examining angiography, cIMT, carotid plaque, computed tomography or magnetic resonance imaging, flow-mediated dilatation, ankle-brachial index or autopsy in patients with SSc and controls . Compared to controls, patients with SSc had a higher prevalence of coronary atherosclerosis, peripheral vascular disease and cerebrovascular calcification.
Risk factors for CVD in patients with SSc
TRFs have been less extensively studied in SSc than in SLE. Overall, there appear to be fewer differences between SSc patients and general population controls than is the case for SLE. In the study from the UK database , the SSc cohort had a lower frequency of overweight and obesity than matched controls, but a higher frequency of past smoking and oral corticosteroid use. The frequency of hypertension, dyslipidaemia and diabetes was similar in the SSc group and matched controls. Hypercholesterolaemia, diabetes and obesity were less prevalent in the Australian Scleroderma Cohort than in community-based controls, whereas past smoking was more prevalent. The prevalence of hypertension was similar .
CVD in patients with IIMs
IIMs such as dermatomyositis and polymyositis are rare autoimmune diseases characterised by inflammation of skeletal muscle, proximal muscle weakness and elevated muscle enzymes. IIM can directly affect the heart causing myocarditis, myocardial fibrosis and arrhythmias . Until recently, evidence on the risk of ACVD in IIM has been scarce.
The all-cause SMR was 1.75 (95% CI 1.41, 2.15) in 370 patients diagnosed with IIM since 1980 from the South Australian Myositis Database . CVD accounted for 30.5% of deaths. A meta-analysis, published in 2014, identified four LOSs that had examined CAD as an outcome in 13,201 IIM patients . There was a significantly increased risk of CAD in patients with IIM compared with controls (pooled risk ratio 2.24 (95% CI: 1.02, 4.92)). In a more recent study from Taiwan, the risk of acute coronary syndrome was significantly higher in patients with IIM than in controls (adjusted hazard ratio 1.98; 95% CI: 1.17–3.35) .
There is evidence of an increased risk of ischaemic stroke in patients with dermatomyositis. In a study of 907 patients with dermatomyositis from Taiwan, the adjusted HR for ischaemic stroke over the first 2 years of disease was 1.67 (95% CI: 1.19, 2.34) compared to controls . A study using a health administrative database from Quebec followed up 607 patients with IIM for a mean of 4 years. The stroke rate (5.2/1000 pyrs) was similar to that reported in patients with RA . In this study, dyslipidaemia and hypertension were associated with arterial events, whereas immunosuppressive therapy was inversely associated.
There is some evidence that TRFs are more prevalent in patients with IIM than in the general population. A cross-sectional study of 84 patients with dermatomyositis identified 47% with MetS compared to 7% of healthy controls . Diabetes was also more prevalent in the dermatomyositis group. Other cross-sectional studies have found higher rates of obesity, hypertension, diabetes and dyslipidaemia in patients with IIM compared with controls . High CAC scores on cardiac computed tomography were also more frequent in the IIM cohort .
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