Systemic lupus erythematosus.
Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect many organs, including the skin, joints, the central nervous system and the kidneys. Women of childbearing age and certain racial groups are typically predisposed to developing the condition. Rare, inherited, single-gene complement deficiencies are strongly associated with SLE, but the disease is inherited in a polygenic manner in most patients. Genetic interactions with environmental factors, particularly UV light exposure, Epstein-Barr virus infection and hormonal factors, might initiate the disease, resulting in immune dysregulation at the level of cytokines, T cells, B cells and macrophages. Diagnosis is primarily clinical and remains challenging because of the heterogeneity of SLE. Classification criteria have aided clinical trials, but, despite this, only one drug (that is, belimumab) has been approved for use in SLE in the past 60 years. The 10-year mortality has improved and toxic adverse effects of older medications such as cyclophosphamide and glucocorticoids have been partially offset by newer drugs such as mycophenolate mofetil and glucocorticoid-sparing regimes. However, further improvements have been hampered by the adverse effects of renal and neuropsychiatric involvement and late diagnosis. Adding to this burden is the increased risk of premature cardiovascular disease in SLE together with the risk of infection made worse by immunosuppressive therapy. Challenges remain with treatment-resistant disease and symptoms such as fatigue. Newer therapies may bring hope of better outcomes, and the refinement to stem cell and genetic techniques might offer a cure in the future.
Department of Rheumatology, St. George's, University of London, Cranmer Terrace, London SW17 0RE, UK.
Arvind
A
Kaul
Rheumatology Research Group, Institute of Inflammation and Ageing, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK.
Caroline
C
Gordon
Mary Kirkland Center for Lupus Research, Hospital for Special Surgery, New York, New York, USA.
Mary K
MK
Crow
University of Toronto Lupus Clinic, Toronto Western Hospital, Centre for Prognosis Studies in the Rheumatic Diseases, Toronto, Ontario, Canada.
Zahi
Z
Touma
University of Toronto Lupus Clinic, Toronto Western Hospital, Centre for Prognosis Studies in the Rheumatic Diseases, Toronto, Ontario, Canada.
Murray B
MB
Urowitz
Unit for Clinical Therapy Research, Inflammatory Diseases (ClinTRID), Karolinska Institutet, Stockholm, Sweden.
Ronald
R
van Vollenhoven
Autoimmune Diseases Research Unit, Department of Internal Medicine, BioCruces Health Research Institute, Hospital Universitario Cruces, University of the Basque Country, Bizkaia, Spain.
Guillermo
G
Ruiz-Irastorza
The London Lupus Centre, London Bridge Hospital, London, UK.
Graham
G
Hughes
D000293:Adolescent; D000328:Adult; D061067:Antibodies, Monoclonal, Humanized / Q000494:pharmacology / Q000627:therapeutic use; D018771:Arthralgia / Q000209:etiology; D001402:B-Lymphocytes / Q000276:immunology; D015897:Comorbidity; D064420:Drug-Related Side Effects and Adverse Reactions / Q000150:complications; D020077:Endogenous Retroviruses / Q000472:pathogenicity; D020031:Epstein-Barr Virus Infections / Q000150:complications; D005221:Fatigue / Q000209:etiology; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000494:pharmacology / Q000627:therapeutic use; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000453:epidemiology / Q000503:physiopathology*; D011788:Quality of Life / Q000523:psychology; D012307:Risk Factors; D012907:Smoking / Q000009:adverse effects; D013601:T-Lymphocytes / Q000276:immunology; D014466:Ultraviolet Rays / Q000009:adverse effects
D061067:Antibodies, Monoclonal, Humanized; D007166:Immunosuppressive Agents; C511911:belimumab