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.