Lupus Guidelines Focus on 12 Recommendations
▸ Pregnancy in lupus. There is no significant difference in fertility in lupus patients. Pregnancy may increase disease activity, but flares are usually mild. Lupus nephritis patients and patients with antiphospholipid antibodies are at increased risk of developing preeclampsia. SLE may affect the fetus, especially if the mother has a history of lupus nephritis and antiphospholipid, anti-Ro, and/or anti-La antibodies. These conditions are associated with an increased risk of miscarriage, stillbirth, premature delivery, intrauterine growth restriction, and fetal heart block. Prednisolone, azathioprine, hydroxychloroquine, and low-dose aspirin may be used in lupus pregnancies. Mycophenolate mofetil, cyclophosphamide, and methotrexate must all be avoided.
▸ Antiphospholipid syndrome in lupus. In patients with SLE and antiphospholipid antibodies, low-dose aspirin may be considered for primary prevention of thrombosis and pregnancy loss. Other risk factors should be assessed; estrogen-containing drugs raise thrombosis risk. In nonpregnant patients with SLE and APS-associated thrombosis, long-term anticoagulation with oral anticoagulants is effective for secondary thrombosis prevention. In pregnant patients with SLE and antiphospholipid syndrome, combined unfractionated or low molecular weight heparin and aspirin cut pregnancy loss and thrombosis.
▸ Lupus nephritis: diagnosis and disease monitoring. 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 with each other. Changes in immunologic tests (anti-ds DNA, serum C3) have only a limited ability to predict patient response to treatment and should be used only supplementally.
▸ Lupus nephritis: treatment. In patients with proliferative lupus nephritis, glucocorticoids plus immunosuppressive agents are effective against progression to end-stage renal disease. Long-term efficacy has been demonstrated only for cyclophosphamide-based regimens, which have considerable adverse effects. In short- and medium-term trials, mycophenolate mofetil has demonstrated at least similar efficacy, compared with pulse cyclophosphamide, and has a more favorable 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 comparable rates for long-term patient and graft survival as those observed in nondiabetic non-SLE patients.
Despite the many manifestations of lupus, the new guidelines lack differential diagnostic criteria. DR. STEEN
Additional Lupus Research Is Needed
The EULAR task force that authored the new SLE guidelines has proposed a research agenda to correct for the current dearth of randomized controlled trials establishing optimal management of lupus. Areas that the task force highlighted for future investigation included:
▸ The validity of renal biopsy, urinary sediment analysis, tests for proteinuria, and immunologic tests as surrogate markers in the treatment of lupus nephritis.
▸ The relative impact on disease susceptibility and severity of environmental factors like sun exposure, smoking, diet, and genetics.
▸ Mechanisms by which to identify patients at higher risk for SLE.
▸ Diagnostic criteria with improved sensitivity and specificity as well as criteria to identify distinct SLE subpopulations.
▸ Reliable diagnostic algorithms for neuropsychiatric lupus.
▸ The indications and optimal targets for autologous stem cell therapy in SLE, as well as the major indications for biologic therapies like B-cell depletion, inhibition of B-cell differentiation, costimulation blockade, and toleragens.
▸ Treatment options for resistant disease that involve major and nonmajor organs.
▸ The mechanisms of a lupus flare, along with the best way to manage flares.
▸ The epidemiology, management, and long-term outcome of geriatric, pediatric, and adolescent SLE.
