List of recommendations with level of evidence and grade of recommendation, agreement, cost/risk ratio
| Recommendation | Level of evidence and grade of recommendation | Agreement | Cost/risk |
|---|---|---|---|
| 1. Patient assessment. | |||
| In addition to the standard care of patients without lupus of the same age and sex, the assessment of patients with SLE must include the evaluation of: | |||
| disease activity by a validated index at each visit | |||
| organ damage annually | |||
| general quality of life by patient history and/or by a 0–10 VAS (patient global score) at each visit | 5, D | 97.6 | L/VL |
| comorbidities | |||
| drug toxicity | |||
| 2. Cardiovascular risk factors | |||
| At baseline and during follow-up at least once a year: | |||
| assess smoking, vascular events (cerebral/cardiovascular), physical activity, oral contraceptives, hormonal therapies and family history of cardiovascular disease | |||
| blood tests: blood cholesterol, glucose | 1b, B | 98.1 | L/VL |
| examine for blood pressure, body mass index (and/or waist circumference) | |||
| NB: some patients may need more frequent follow-up (ie, patients on glucocorticoids) | |||
| 3. Other comorbidities | |||
| Osteoporosis. All patients with SLE: | |||
| should be assessed for adequate calcium and vitamin D intake, regular exercise and smoking habits | |||
| should be screened and followed for osteoporosis according to existing guidelines (a) for postmenopausal women; (b) for patients on steroids, or on any other medication that may reduce BMD | 2b, C | 93.8 | M/VL |
| Cancer. Cancer screening is recommended according to the guidelines for the general population, including cervical smear tests | 2b, C | 92.3 | M/L |
| 4. Infection risk | |||
| Screening. We recommend that patients should be screened for: | |||
| HIV based on the patient's risk factors | 2b, C | 98.8 | M/VL |
| HCV, HBV based on the patient's risk factors, particularly before IS drugs including high dose glucocorticoids are given | |||
| tuberculosis, according to local guidelines, especially before IS drugs including high dose glucocorticoids are given | |||
| CMV testing should be considered during treatment in selected patients. | |||
| Vaccination. Patients with SLE are at high risk of infections, and prevention should be recommended. The administration of inactivated vaccines (especially flu and pneumococcus), following the Centers for Disease Control (CDC) guidelines for patients who are immunosuppressed, should be strongly encouraged in patients with SLE on IS drugs, preferably administered when the SLE is inactive. For other vaccinations, an individual risk/benefit analysis is recommended. | 5, D | 93.5 | M/M |
| Monitoring. At follow-up visits, continuous assessment of the risk of infection by taking into consideration the presence of | |||
| severe neutropenia (<500 cells/mm3) | |||
| severe lymphopenia (<500 cells/mm3) | 1b, B | 88.8 | M/VL |
| low IgG (<500 mg/dl) | |||
| 5. Frequency of assessments | |||
| In patients with no activity, no damage, no comorbidity we recommend assessments every 6–12 months. During these visits, preventive measures should be emphasised. | 5, D | 93.8 | M/L |
| 6. Laboratory assessment | |||
| We recommend the monitoring of the following autoantibodies and complement: | |||
| baseline: ANA, anti-dsDNA, anti-Ro, anti-La, anti-RNP, anti-Sm, anti-phospholipid, C3, C4 | |||
| re-evaluation in previously negative patients of: anti-phospholipid antibodies: prior to pregnancy, surgery, transplant and use of oestrogen-containing treatments, or in the presence of a new neurological or vascular event; anti-Ro and anti-La antibodies before pregnancy; anti-dsDNA/C3 C4 may support evidence of disease activity/remission | 2b, C | 92.3 | M/VL |
| Other laboratory assessments. At 6–12 months intervals patients with inactive disease should have: | |||
| complete blood count | 5, D | 89.5 | M/VL |
| erythrocyte sedimentation rate | |||
| C reactive protein | |||
| serum albumin | |||
| serum creatinine (or eGFR) | |||
| urinalysis and urine protein/creatinine ratio | |||
| NB: if a patient is on a specific drug treatment, monitoring for that drug is required as well | |||
| 7. Mucocutaneous involvement | |||
| Mucocutaneous lesions should be characterised, according to the existing classification systems, as to whether they may be: | |||
| LE specific | |||
| LE non-specific | |||
| LE mimickers | 5, D | 94.6 | M/L |
| drug-related | |||
| Lesions should be assessed for activity and damage, using validated indices (ie, CLASI) | |||
| 8. Kidney | |||
| Patients with a persistently abnormal urinalysis or raised serum creatinine should have urine protein/creatinine ratio (or 24 h proteinuria), urine microscopy and renal ultrasound, and be considered for referral for biopsy. | |||
| Patients with established nephropathy should have protein/creatinine ratio (or 24 h proteinuria) and immunological tests (C3, C4, anti-dsDNA), urine microscopy and blood pressure at least every 3 months for the first 2–3 years. | 1b, B | 94.2 | H/M |
| Patients with established chronic renal disease (eGFR <60 ml or stable proteinuria >0.5 mg/24 h) should be followed according to the National Kidney Foundation guidelines for chronic kidney disease. | |||
| 9. Neuropsychiatric manifestations | |||
| Patients with SLE should be monitored for the presence of neuropsychological symptoms (seizures, paresthesiae, numbness, weakness, headache, epilepsy, depression, etc) by focused history. | 2b, D | 87.7 | M/VL |
| Cognitive impairment may be assessed by evaluating attention, concentration, word finding and memory difficulties (ie, by asking the patient about problems with multitasking, with household tasks, or memory). If there is a suspicion of any cognitive impairment, then the patient should be assessed in further detail. | |||
| 10. Eye assessment | |||
| In patients treated with glucocorticoids or antimalarials, a baseline eye examination is recommended according to standard guidelines. An eye examination during follow-up is recommended: | |||
| in selected patients taking glucocorticoids (high risk of glaucoma or cataracts) | |||
| in patients on antimalarial drugs, and (a) low risk: no further testing is required until after 5 years of baseline, after the first 5 years of treatment eye assessment is recommended yearly; (b) high risk: eye assessment is recommended yearly. | 2b, D | 95.8 | M/L |
ANA, anti-nuclear antibodies; BMD, bone mineral density; CLASI, Cutaneous Lupus Disease Area and Severity index; CMV, cytomegalovirus; dsDNA, double-stranded DNA; eGFR, estimated glomerular filtration rate; H, high; HBV, hepatitis B virus; HCV, hepatitis C virus; HIV, human immunodeficiency virus; IS, immunosuppressive; L, low; LE, lupus erythematosus; M, moderate; SLE, systemic lupus erythematosus; VAS, visual analogue scale; VH, very high; VL, very low.