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Systemic lupus erythematosus (SLE)

Systemic lupus erythematosus (SLE). Presented by Rachael Haun & Avery Medina. Image From http://www.medicinenet.com/image-collection/systemic_lupus_erythematosus_1_picture/picture.htm. Introduction. “Prototype” autoimmune disease Typically seen in women of childbearing age (Mock, 2011)

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Systemic lupus erythematosus (SLE)

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  1. Systemic lupus erythematosus (SLE) Presented by Rachael Haun & Avery Medina Image From http://www.medicinenet.com/image-collection/systemic_lupus_erythematosus_1_picture/picture.htm

  2. Introduction • “Prototype” autoimmune disease • Typically seen in women of childbearing age (Mock, 2011) • Cause officially unknown • Genetic predisposition coupled with environmental trigger? • More common in African American, Hispanic, Asian & Native American populations (National Institute of Arthritis, Musculoskeletal and Skin Diseases, 2009)

  3. Introduction • Defect: Mechanisms of lupus still poorly understood • Body loses “self-tolerance” • Abnormal DNA methylation leads to over-expression of genes resulting in autoimmunity • The body forms its own antibodies to nuclear components (antigens) • Abnormal T cells • activate B cells, create autoantibodies • Exacerbate autoimmune response and autoantibody formation (Gorelik & Richardson, 2010)

  4. Introduction • Autoantibodies Image from: http://www.nature.com/nri/journal/v4/n10/fig_tab/nri1456_F1.html

  5. Brief History and Diagnostics • Characteristic “butterfly rash” first described by physician Rogerius in 13th century  • Kaposi (1872) described other systemic features: arthritis & psychosis • Current estimated incidence in US: 40-50 per 100,000 cases • Life expectancy: 5 years of diagnosis (10%) • Many deaths result from long term treatment & immunosuppression • Current diagnostic tests: American College of Rheumatology criteria (4 of 11) • Antinuclear antibody (ANA), malar rash, discoid rash on cheeks, photosensitivity (sunlight), painless mucocutaneous ulcers, nonerosive arthritis (2+ joints), renal dysfunction (proteinuria/cellular casts), neurologic disorder (seizure/psychosis), serositis (pleuritis/pericarditis), hematologic disorders (anemia, thrombocytopenia, leukopenia), immunologic dysfunction • Other tests • complete blood count; urinalysis; serum creatinine level; antiphospholipid antibodies

  6. Clinical Consequences of Untreated Disease • Can affect any bodily tissue • Can damage joints, heart, lungs, kidneys, blood vessels, skin and brain • Symptoms are unpredictable and vary • Pain, swelling, fatigue, anemia, rashes/photosensitivity, ulcers and more… (UMMC, 2011) Image from: http://www.medicinenet.com/lupus_pictures_slideshow/article.htm

  7. Clinical Consequences of Untreated Disease • 90% of those with SLE have a 10 year survival rate, 63-75% have a 20 year survival rate (UMMC, 2011) • Kept unchecked, SLE can be fatal • Why? • The body is attacking itself! Widespread damage, often to major organs Image from: http://kronstantinople.blogspot.com/2011/07/lupus-rash-quest-for-truth.html

  8. Recent Research: RAGE polymorphisms • Study conducted in 2012 at University Medical Center Groningen, Netherlands • Compared patients suffering from SLE and lupus nephritis to healthy controls • Found that RAGE polymorphisms associated with pro-inflammatory response had significant involvement with susceptibility to SLE, severity of LN symptoms

  9. References • Gorelik, G., & Richardson, B. (2010). Key role of erk pathway signaling in lupus. Autoimmunity, 43(1), 17-22. doi: 10.3109/08916930903374832 • Martens, HA, Nienhuis, HLA, Gross, S, der Steege, G van, Brouwer, E, Berden, JHM, Sévaux, RGL de, Derksen, RHWM, Voskuyl, AE, Berger, SP, Navis, GJ, Nolte, IM, Kallenberg, CGM, Bijl, M (2012). Receptor for advanced glycation end products (RAGE) polymorphisms are associated with systemic lupus erythematosus and disease severity in lupus nephritis. Lupus, 21(9). pg 959-968. • Mok, C. C. (2011). Epidemiology and survival of systemic lupus erythematosus in Hong Kong Chinese. Lupus, 20(7), 767-771. doi:10.1177/0961203310388447

  10. References • National Institute of Arthritis, Musculoskeletal and Skin Diseases. (2009, Oct.). What is lupus?. Retrieved from http://www.niams.nih.gov/Health_Info/Lupus/lupus_ff.a sp • Robinson, M, Sheets Cook, S, and Currie, LM (2011). Systemic lupus erythematosus: A genetic review for advanced practice nurses. Journal of the American Academy of Nurse Practitioners, 23(12). pg 629-637. • UMMC. (2011). Systemic lupus erythematosus. Retrieved from http://www.umm.edu/altmed/articles/systemic-lupus- 000161.htm

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