EULAR overarching principles and recommendations for the management of CVR in gout, vasculitis, SSc, myositis, MCTD, SS, SLE, and APS
| Overarching principles | LoA* (SD) |
| A. Clinicians should be aware of increased CVR in patients with RMDs including gout, vasculitis, SSc, myositis, MCTD, SS, SLE and APS. For all RMDs, reduction of disease activity is likely to lessen CVR. | 9.92 (0.39) |
| B. Rheumatologists are responsible for CVR assessment and management in collaboration with primary care providers, internists or cardiologists and other healthcare providers. | 9.55 (1.12) |
| C. CVR factor screening should be performed regularly in all individuals with RMDs. Risk management should include screening for and strict control of CVR factors (smoking cessation, management of blood pressure, lipids and diabetes). CVR assessment is recommended within 6 months of diagnosis and repeated based on individual patient characteristics and risk levels. | 9.55 (0.84) |
| D. Patient education and counselling on CVR, treatment adherence and lifestyle modifications, such as healthy diet and regular physical activity, are important in the management of CVR in these patients. | 9.88 (0.42) |
| Recommendations for gout, vasculitis, SSc, myositis, MCTD and SS | |
| 1. In patients with gout, vasculitis, SSc, myositis, MCTD and SS, we recommend thorough assessment of traditional CVR factors. The use of cardiovascular prediction tools for the general population is recommended. (LoE: 5, GoR‡: D) | 9.48 (0.84) |
| 2. For ANCA-associated vasculitis the Framingham score may underestimate the CVR. Information from the EUVAS model may supplement modifiable Framingham risk factors and is recommended to take into account. (LoE: 2b, GoR: D) | 8.59 (1.50) |
| 3. In patients with gout, vasculitis, SSc, myositis, MCTD and SS, blood pressure management should follow recommendations used in the general population. (LoE: 5, GoR: D) | 9.66 (0.62) |
| 4. In patients with gout, diuretics should be avoided. (LoE: 5, GoR: D) | 8.88 (2.06) |
| 5. In patients with SSc beta blockers should be avoided. (LoE: 5, GoR: D) | 8.92 (2.11) |
| 6. In patients with gout, vasculitis, SSc, myositis, MCTD and SS, lipid management should follow recommendations used in the general population. (LoE: 5, GoR: D) | 9.48 (1.08) |
| 7. In patients with gout, vasculitis, SSc, myositis, MCTD and SS, standard use of platelet inhibitors for primary prevention is not recommended. Treatment with platelet inhibitors should follow recommendations used in the general population. (LoE: 2b/5, GoR: D) | 9.37 (1.14) |
| 8. In patients with gout, we recommend a serum uric acid level below 0.36 mmol/L (6 mg/dL) to potentially lower the risk on cardiovascular events and cardiovascular mortality. (LoE: 2b, GoR: C) | 9.03 (1.34) |
| 9. In patients with gout there is no preference for a particular urate-lowering therapy from the cardiovascular point of view. (LoE: 1b, GoR: B) | 9.14 (1.35) |
| 10. In patients with ANCA-associated vasculitis, remission induction and remission maintenance will also reduce CVR. (LoE: 2b, GoR: D) | 9.07 (1.35) |
| 11. In patients with giant-cell arteritis an optimal glucocorticoid regimen that balances the risk of relapse and glucocorticoid use side effects may also reduce CVR. (LoE: 2b, GoR: D) | 9.14 (1.06) |
| Recommendations for SLE and the APS | |
| 1. In patients with SLE and/or APS, a thorough assessment of traditional CVR factors and disease-related risk factors is recommended to guide risk factor modification. (LoE: 2b, GoR: D) | 9.88 (0.32) |
| 2A. In patients with SLE, lower levels of blood pressures are associated with lower rates of cardiovascular events and a blood pressure target of <130/80 mm Hg should be considered. (LoE: 2b, GoR: C) | 9.70 (0.54) |
| 2B. In patients with lupus nephritis, ACE inhibitors or angiotensin receptor blockers are recommended for all patients with urine protein-to-creatinine ratio >500 mg/g or arterial hypertension. (LoE: 5, GoR: D) | 9.51 (0.64) |
| 2C. In patients with APS, blood pressure management should follow recommendations used in the general population. (LoE: 5, GoR: D) | 9.81 (0.39) |
| 3. In patients with SLE and/or APS, lipid treatment should follow recommendations used in the general population. (LoE: 5, GoR: D) | 9.70 (0.54) |
| 4A. Patients with SLE may be candidates for preventative strategies as in the general population, including low-dose aspirin, based on their individual CVR profile. (LoE: 2b, GoR: D) | 9.29 (1.37) |
| 4B. In asymptomatic aPL carriers (not fulfilling any vascular or obstetric APS classification criteria) with a high-risk aPL profile with or without traditional risk factors, prophylactic treatment with low-dose aspirin (75–100 mg daily) is recommended. (LoE: 2a, GoR: B) In patients with SLE and no history of thrombosis or pregnancy complications: (1) with high-risk aPL profile, prophylactic treatment with low-dose aspirin is recommended (LoE: 2a, GoR: B); (2) with low-risk aPL profile, prophylactic treatment with low-dose aspirin may be considered. (LoE: 2b, GoR: C) | 9.44 (0.97) |
| 5. In patients with SLE, low disease activity should be maintained to also reduce CVR. (LoE: 2b, GoR: B) | 9.59 (1.11) |
| 6. In patients with SLE, treatment with the lowest possible corticosteroid dose is recommended to minimise any potential cardiovascular harm. (LoE: 2b, GoR: C) | 9.59 (0.79) |
| 7. In patients with SLE, no specific immunosuppressive medication can be recommended for the purpose of lowering the risk of cardiovascular events. (LoE: 2b, GoR: C) | 9.44 (0.89) |
| 8. In patients with SLE, treatment with hydroxychloroquine (which is recommended for all patients unless contraindicated) should be considered to also reduce the risk of cardiovascular events. (LoE: 2b, GoR: B) | 9.66 (0.73) |
*LoA, level of agreement; numbers in column indicate the mean (SD) of the LoA among task force members.
†LoE, level of evidence: 1a: systematic review of RCTs; 1b: individual RCT; 2a: systematic review of cohort studies; 2b: individual cohort study (and low-quality RCT); 3a: systematic review of case–control studies; 3b: individual case–control study; 4: case series and poor-quality cohort and case–control studies; 5: expert opinion without explicit critical appraisal, or based on physiology, bench research or ‘first principles’.
‡GoR, grade of recommendation: A: consistent level 1 studies; B: consistent level 2 or 3 studies, or extrapolations from level 1 studies; C: level 4 studies or extrapolations from level 2 or 3 studies; D: level 5 evidence or troublingly inconsistent or inconclusive studies of any level.
ACE, angiotensin-converting enzyme; ANCA, antineutrophil cytoplasmic antibodies; aPL, antiphospholipid antibodies; APS, antiphospholipid syndrome; CVR, cardiovascular risk; EULAR, European League against Rheumatism; EUVAS, European Vasculitis Society; MCTD, mixed connective tissue disease; RMDs, rheumatic and musculoskeletal diseases; SLE, systemic lupus erythematosus; SS, Sjögren’s syndrome; SSc, systemic sclerosis.