Table 1 Summary of the statements and recommendations on the management of systemic lupus erythematosus based on evidence and expert opinion
General management
    In patients with SLE, new clinical signs (rashes, arthritis, serositis, neurological manifestations and seizures/psychosis), routine laboratory (CBC, serum creatinine, proteinuria and urinary sediment), and immunological tests (serum C3, anti-dsDNA, anti-Ro/SSA, anti-La/SSB, antiphospholipid, anti-RNP), may provide prognostic information for the outcome in general and involvement of major organs, and thus should be considered in the evaluation of these patients. Confirmation by imaging (brain MRI), and pathology (renal biopsy) may add prognostic information and should be considered in selected patients.
    New clinical manifestations such as number and type of skin lesions, or arthritis, serositis, and neurological manifestations (seizures/psychosis), laboratory tests (CBC), immunological tests (serum C3/C4, anti-C1q, anti-dsDNA), and validated global activity indices have diagnostic ability for monitoring for lupus activity and flares, and may be used in the monitoring of lupus patients.
    SLE patients are at increased risk for certain co-morbidities, due to the disease and/or its treatment. These co-morbidities include infections (urinary-tract infections, other infections), atherosclerosis, hypertension, dyslipidaemias, diabetes, osteoporosis, avascular necrosis, malignancies (especially non-Hodgkin’s lymphoma). Minimisation of risk factors together with a high-index of suspicion, prompt evaluation, and diligent follow-up of these patients is recommended.
In the treatment of SLE without major organ manifestations, antimalarials and/or glucocorticoids are of benefit and may be used. NSAIDs may be used judiciously for limited periods of time at patients at low risk for their complications. In non-responsive patients or patients not being able to reduce steroids below doses acceptable for chronic use, immunosuppressive agents such as azathioprine, mycophenolate mofetil and methotrexate should also be considered.
Adjunct therapy
    Photo-protection may be beneficial in patients with skin manifestations and should be considered. Lifestyle modifications (smoking cessation, weight control, exercise) are likely to be beneficial for patient outcomes and should be encouraged. Depending on the individual medication and the clinical situation, other agents (low-dose aspirin, calcium/vitamin D, biphosphonates, statins, antihypertensives (including angiotensin converting enzyme inhibitors)) should be considered. Oestrogens (oral contraceptives, hormone-replacement therapy) may be used, but accompanying risks should be assessed.
Neuropsychiatric lupus
    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.
    SLE patients with major neuropsychiatric manifestations considered to be of inflammatory origin (optic neuritis, acute confusional state/coma, cranial or peripheral neuropathy, psychosis, and transverse myelitis/myelopathy) may benefit from immunosuppressive therapy.
Pregnancy in lupus
Pregnancy affects mothers with SLE and their offspring in several ways.
    (a) Mother. There is no significant difference in fertility in lupus patients. Pregnancy may increase lupus disease activity, but these flares are usually mild. Patients with lupus nephritis and antiphospholipid antibodies are more at risk of developing pre-eclampsia and should be monitored more closely.
    (b) Fetus. SLE may affect the fetus in several ways, especially if the mother has a history of lupus nephritis, antiphospholipid, anti-Ro and/or anti-La antibodies. These conditions are associated with an increase in the risk of miscarriage, stillbirth, premature delivery, intrauterine growth restriction and fetal congenital heart block. Prednisolone, azathioprine, hydroxychloroquine and low-dose aspirin may be used in lupus pregnancies. At present, evidence suggests that mycophenolate mofetil, cyclophosphamide and methotrexate must be avoided.
Antiphospholipid syndrome
In patients with SLE and antiphospholipid antibodies, low-dose aspirin may be considered for primary prevention of thrombosis and pregnancy loss. Other risk factors for thrombosis should also be assessed. Oestrogen-containing drugs increase the risk for thrombosis. In non-pregnant patients with SLE and APS-associated thrombosis, long-term anticoagulation with oral anticoagulants is effective for secondary prevention of thrombosis. In pregnant patients with SLE and antiphospholipid syndrome combined unfractionated or LMW heparin and aspirin reduce pregnancy loss and thrombosis and should be considered.
Lupus nephritis
    Renal biopsy, urine sediment analysis, proteinuria, and kidney function may have independent predictive ability for clinical outcome in therapy of lupus nephritis but need to be interpreted in conjunction. Changes in immunological tests (anti-dsDNA, serum C3) have only limited ability to predict the response to treatment and may be used only as supplemental information.
    In patients with proliferative lupus nephritis, glucocorticoids in combination with immunosuppressive agents are effective against progression to end-stage renal disease. Long-term efficacy has been demonstrated only for cyclophosphamide-based regimens, which are however associated with considerable adverse effects. In short- and medium-term trials, mycophenolate mofetil has demonstrated at least similar efficacy compared with pulse cyclophosphamide and a more favourable toxicity profile: failure to respond by 6 months should evoke discussions for intensification of therapy. Flares following remission are not uncommon and require diligent follow-up.
End-stage renal disease
    Dialysis and transplantation in SLE have rates for long-term patient and graft-survival comparable with those observed in non-diabetic non-SLE patients, with transplantation being the method of choice.