| Recommendation/item | No. of studies evaluated | Category of evidence | Strength of statement | Mean level of agreement* |
| Prognosis. Prognostic value of: | ||||
| Clinical features | ||||
| Rashes | 4 | 4 | B | 8.6 |
| Arthritis | 4 | 4 | B | 8.7 |
| Serositis | 6 | 4 | B | 8.6 |
| Seizures/psychosis | 9 | 4 | B | 9.0 |
| Laboratory findings | ||||
| Severe anaemia | 10 | 4 | B | 8.0 |
| Leucopenia/lymphopenia | 4 | 5 | C | 8.0 |
| Thrombocytopenia | 15 | 4 | B | 8.0 |
| Serum creatinine | 20 | 4 | B | 9.2 |
| Proteinuria/urinary sediment | 24 | 4 | B | 9.3 |
| C3/C4 | 13 | 4 | B | 8.4 |
| Anti-dsDNA | 17 | 4 | B | 8.7 |
| Anti-Ro/SSA | 6 | 4 | B | 7.7 |
| Anti-La/SSB | 1 | 5 | C | 7.7 |
| Antiphospholipid | 19 | 4 | B | 8.5 |
| Anti-RNP | 3 | 4 | B | 7.6 |
| Imaging | ||||
| Brain MRI | 7 | 4 | B | 8.7 |
| Pathology | ||||
| Renal biopsy | 33 | 4 | B | 9.5 |
| Monitoring. Diagnostic ability of: | ||||
| Rashes | 1 | 5 | C | 8.8 |
| Anaemia | 1 | 4 | B | 8.3 |
| Lymphopenia | 1 | 4 | B | |
| Thrombocytopenia | 1 | 5 | C | |
| C3/C4 | 13 | 4 | B | 8.8 |
| Anti-C1q | 8 | 4 | B | 7.7 |
| Anti-dsDNA | 15 | 4 | B | 8.7 |
| Comorbidities. Increased risk for: | ||||
| Infections | 13 | 5 | C | 8.6 |
| Urinary-tract infections | 1 | 4 | B | 8.9 |
| Atherosclerosis | 14 | 4 | B | 8.8 |
| Hypertension | 7 | 4 | B | 9.4 |
| Dyslipidaemia | 7 | 4 | B | 9.2 |
| Diabetes | 3 | 5 | C | 8.9 |
| Osteoporosis | 6 | 5 | C | 9.1 |
| Avascular necrosis | 8 | 5 | C | 8.6 |
| Neoplasms | 8.7 | |||
| Non-Hodgkin’s lymphomas | 6 | 4 | B | |
| Other | 10 | 4 | B | |
| Therapy of uncomplicated SLE | ||||
| Antimalarials | 4 | 2 | A | 9.4 |
| NSAIDs | 1 | – | D | 8.8 |
| Glucocorticoids | 3 | 2 | A | 9.1 |
| Azathioprine | 1 | 4 | B | 9.3 |
| Mycophenolate mofetil | 4 | 6 | D | 6.9 |
| Methotrexate | 3 | 2 | A | 8.0 |
| Adjunct therapy in SLE | ||||
| Photoprotection | 1 | 4 | B | 9.2 |
| Smoking cessation | – | – | D | 9.3 |
| Weight control | – | – | D | |
| Exercise | – | – | D | |
| Low-dose aspirin | 1 | 4 | D† | 9.0 |
| Calcium/vitamin D | 5 | 2 | A | 9.2 |
| Biphosphonates | 2 | 2 | A | 8.5 |
| Statins | – | – | D | 8.9 |
| Antihypertensives | – | – | D | 8.9 |
| Oral contraceptives (safe use) | 2 | 2 | A | 9.1 |
| Hormone-replacement therapy | 3 | 2 | A | 9.1 |
| Diagnosis of neuropsychiatric lupus | 8.1‡ | |||
| Clinical features | ||||
| Headache (not related) | 1 | 3 | A | |
| Anxiety | 1 | 5 | C | |
| Depression | 1 | 5 | C | |
| Cognitive impairment | 3 | 4 | B | |
| Laboratory tests | ||||
| EEG | 3 | 4 | B | |
| Anti-P | 6 | 4 | B | |
| Antiphopholipid | 4 | 4 | B | |
| Neuropsychological tests | 3 | 5 | C | |
| Imaging tests | ||||
| CT | 3 | 4 | B | |
| MRI | 9 | 4 | B | |
| PET | 2 | 4 | B | |
| SPECT | 5 | 5 | C | |
| MTI | 5 | 5 | C | |
| DWI | 1 | 5 | C | |
| MRS | 3 | 5 | C | |
| T2 relaxation time | 2 | 5 | C | |
| Treatment of neuropsychiatric lupus | ||||
| Immunosuppressants (CY) in combination with glucocorticoids | 10 | 2 | A | 9.2 |
| Pregnancy | ||||
| Fertility not impaired | 4 | 5 | C | 8.8 |
| Increased lupus activity/flares | 11 | 3 | B | 8.8 |
| Increased risk for pre-eclampsia | 6 | 4 | B | 9.8 |
| Increased risk for miscarriage/stillbirth/premature delivery | 30 | 4 | B | 9.4 |
| Increased risk for intrauterine growth restriction | 6 | 5 | C | |
| Increased risk for fetal congenital heart block | 7 | 4 | B | |
| Therapy during pregnancy | ||||
| Prednisolone | 6 | 6 | D | 9.6 |
| Azathioprine | 5 | 6 | D | 9.2 |
| HCQ | 9 | 2 | A | 9.5 |
| Low-dose aspirin | 1 | 6 | D | 9.3 |
| Antiphospholipid syndrome | ||||
| Primary prevention of thrombosis/pregnancy loss | ||||
| Low-dose aspirin | – | – | D | 8.7 |
| Secondary prevention of thrombosis/pregnancy loss | ||||
| Oral anticoagulants (non-pregnant patients) | 8 | 2 | A | 9.0 |
| Unfractionated/LMW heparin and aspirin (pregnant patients) | 14 | 1 | A | 9.1 |
| Nephritis: monitoring | ||||
| Repeat renal biopsy | 6 | 4 | B | 9.5 |
| Urinary sediment | 2 | 4 | B | |
| Proteinuria | 10 | 4 | B | |
| Serum creatinine | 8 | 4 | B | |
| Anti-dsDNA | 3 | 4 | B | 8.7 |
| C3 | 2 | 4 | B | |
| Nephritis: treatment | ||||
| Combined glucocorticoids and immunosuppressants are effective against ESRD | 21 | 1 | A | 9.3 |
| MMF has similar efficancy to pulse CY in short-/medium-term trials | 8 | 2 | A | 9.2 |
| CY efficacy in long-term trials | 13 | 1 | A | 9.5 |
| End-stage renal disease in SLE | ||||
| Dialysis is safe in SLE | 7 | 3 | B | 8.8 |
| Transplantation is safe in SLE | 9 | 3 | B | |
| Transplantation is superior to dialysis | 2 | 5 | C§ | 9.4 |
*Mean level of agreement of the Task Force members on each sub-item/statement; †in elderly SLE patients, low-dose aspirin is associated with improved cognitive function (4/B); ‡this refers to the statement that “in SLE patients, the diagnostic work-up (clinical, laboratory, neuropsychological and imaging tests) of neuropsychiatric manifestations should be similar to that in the general population presenting with the same neuropsychiatric manifestations”; §non-SLE studies.