Mangment of Systemic Lupus Erythematous

Elshawehdy, Naser Mahmoud (2018-06-29)

Systemic lupus erythematosus (SLE) is a systemic inflammatory autoimmune disease predominantly affecting women. The prevalence of lupus is estimated at 12.5–78.5 cases per 100,000 population in Europe and the USA with a female:male ratio of around 9:1.1 In the UK SLE is approximately 2.5 times more common in South Asian and 5–6 times more common in Afro-Caribbean individuals.2 Due to the rarity of SLE it is difficult to accurately determine the incidence, but it has been estimated to be between 2.0 and 7.6 cases per 100,000 population/year.1 The aetiology and immunopathogenesis of SLE have been extensively reviewed elsewhere.4 This review will focus on advances in the management of SLE in terms of both the disease itself and its associated co-morbidities. SLE is one of a small number of truly multisystem disorders. The heterogeneous nature of the disease can result in delayed diagnosis and cause considerable difficulty in the design of robust clinical trials. There is no diagnostic test specific for SLE and as such the diagnosis remains a clinical one, relying on a combination of clinical and laboratory features. The 1992 Revised American College of Rheumatology (ACR) Classification Criteria, while developed to aid trial design, offer a useful aide-mémoire to the rheumatologist of some of the more common features of SLE


Neuropsychiatric events are common in patients with systemic lupus erythematosus (SLE), but less than one-third of these events can be directly attributed to SLE. Increased generalized SLE disease activity or damage, previous or concurrent major neuropsychiatric SLE (NPSLE) events, and persistently positive moderate-to-high antiphospholipid antibody titers are established risk factors, and their presence could facilitate proper attribution to the disease itself. Diagnostic evaluation is guided by the presenting manifestation; MRI is used to visualize brain or spinal pathologies. For neuropsychiatric events believed to reflect an immune or inflammatory process, or when these events occur in the context of active generalized disease, evidence (primarily from uncontrolled studies) supports the use of glucocorticoids alone or in combination with immunosuppressive therapy. Antiplatelet and/or anticoagulation therapy is recommended for NPSLE manifestations related to antiphospholipid antibodies, especially for thrombotic cerebrovascular disease. For the future, we anticipate that novel biomarkers and advanced neuroimaging tests will better define the underlying pathologic mechanisms of SLE-related neuropsychiatric disease, and help guide therapeutic decisions.

Attribution 3.0 United States
Except where otherwise noted, this item's license is described as Attribution 3.0 United States