Systemic Lupus Erythematosus

Abstract

Systemic lupus erythematosus (SLE) is a chronic inflammatory disease of unknown cause that can affect the skin, joints, kidneys, lungs, nervous system, serous membranes and other organs in the body, in various combinations, especially in young women, and which is characterised by immune abnormalities, in particular antibodies to nuclear constituents, on a background of genetic and environmental risk factors. The clinical course of SLE is variable and may be characterised by periods of remissions and of chronic or acute relapses.

Treatment is based on both preventive measures and alleviation of symptoms, but in particular antiinflammatory agents including nonsteroidal antiinflammatory drugs, corticosteroids and immunosuppressive medications.

Key Concepts

  • Systemic lupus erythematosus (SLE) is a chronic inflammatory disease of unknown cause that can affect the skin, joints, kidneys, lungs, nervous system, serous membranes and/or other organs of the body. Immunologic abnormalities, especially the production of a number of antinuclear antibodies, are another prominent feature of the disease. The clinical course of SLE is variable and may be characterised by periods of remissions and chronic or acute relapses. Women, especially in their 20s and 30s, are affected more frequently than men.
  • The most common pattern is a mixture of constitutional complaints with skin, musculoskeletal, mild haematologic and serologic involvement. However, some patients have predominately haematologic, renal or central nervous system manifestations. The pattern that dominates during the first few years of illness tends to prevail subsequently.
  • Fatigue, fever and weight loss are typically present at some time during the course of the disease, occurring in 50–100% of patients. Other symptoms may include myalgia and weight gain. Fever due to active disease is seen in over 50% of patients with SLE; fever may also represent infection or a drug reaction.
  • The course is marked by remissions and relapses and may vary from mild to severe. Major manifestations include:
    • Arthritis, with joint symptoms in >90% of patients
    • Mucocutaneous lesions, including skin lesions in most patients, hair loss, and oral and/or nasal ulcers, which occur in 12–45%
    • Raynaud phenomenon, which is present in 16–40%
    • Renal involvement, with histopathologic change in most patients but with clinically apparent disease in ∼50%
    • Gastrointestinal tract disease, which is more often due to complications of medications than to active SLE
    • Pulmonary diseases, including pleurisy, pleural effusion, pneumonitis, interstitial lung disease, pulmonary hypertension and alveolar haemorrhage
    • Cardiovascular diseases, including pericarditis (which is relatively common), verrucous endocarditis and increased risk of coronary artery disease
    • Neurologic diseases, including cognitive defects, organic brain syndromes, delirium, psychosis, seizures, headache, peripheral neuropathies and thromboembolic disease
    • Ophthalmologic manifestations, particularly keratoconjunctivitis sicca as well as more uncommon manifestations
    • Haematologic manifestations, including cytopenias, thrombophilia, thromboembolic disease, lymphadenopathy and splenomegaly
    • Autoantibody production
  • The onset of SLE is infrequently attributed to a single event, although most clinicians have seen patients in which the disease began shortly following some memorable occurrence. Precipitating factors may include exposure to ultraviolet light, infections, stress, surgery and pregnancy.

Keywords: autoimmune disease; rheumatic disease; immunology; genetics; corticosteroids

References

Allam R and Anders H‐J (2008) The role of innate immunity in autoimmune tissue injury. Current Opinion in Rheumatology 20: 538–544.

Barber CE and Barnabe C (2012) Another consequence of severe lupus: invasive fungal disease. Journal of Rheumatology 39: 1772.

Bernatsky S , Boivin JF , Joseph L , et al. (2006) Mortality in systemic lupus erythematosus. Arthritis and Rheumatism 54: 2550.

Boackle SA (2013) Advances in lupus genetics. Current Opinion in Rheumatology 25: 561.

Bootsma H , Spronk P , Derksen R , et al. (1995) Prevention of relapses in SLE. Lancet 345: 1595–1599.

Cervera R , Khamashta MA , Font J , et al. (2003) Morbidity and mortality in systemic lupus erythematosus during a 10‐year period. A comparison of early and late manifestations in a cohort of 1,000 patients. Medicine (Baltimore) 82: 299.

Davidson A and Aranow C (2006) Pathogenesis and treatment of systemic lupus erythematosus nephritis. Current Opinion in Rheumatology 18: 468–475.

Drenkard C , Villa AR , Garcia‐Padilla C , et al. (1996) Remission of systemic lupus erythematosus. Medicine 75: 88–98.

Duffy KN , Duffy CM and Gladman DD (1991) Infection and disease activity in SLE: a review of hospitalised patients. Journal of Rheumatology 18: 1810.

Feng X , Wu H , Grossman JM , et al. (2006) Association of increased interferon‐inducible gene expression with disease activity and lupus nephritis in patients with systemic lupus erythematosus. Arthritis and Rheumatism 54: 2951.

Graham KL and Utz PJ (2005) Sources of autoantigens in systemic lupus erythematosus. Current Opinion in Rheumatology 17: 513.

Grimaldi CM (2006) Sex and systemic lupus erythematosus: the role of the sex hormones estrogen and prolactin on the regulation of autoreactive B cells. Current Opinion in Rheumatology 18: 456–461.

Hahn BH , Ebling F , Singh RR , et al. (2005) Cellular and molecular mechanisms of regulation of autoantibody production in lupus. Annals of the New York Academy of Sciences 1051: 433.

Heller CA and Schur PH (1985) Serological and clinical remission in SLE. Journal of Rheumatology 12: 916–918.

Hochberg MC (1997) Updating the American College of Rheumatology Revised criteria for the classification of systemic lupus erythematosus. Arthritis and Rheumatism 40: 1725.

Iaboni A , Ibanez D , Gladman DD , et al. (2006) Fatigue in SLE: contributions of disordered sleep, sleepiness, and depression. Journal of Rheumatology 33: 2453.

Kamen DA and Aranow CB (2008) Vitamin D in Systemic Lupus Erythematosus. Current Opinion in Rheumatology 20: 532–537.

Karp I , Abrahamowicz M , Fortin PR , et al. (2008) Recent corticosteroid use and recent disease activity: independent determinants of coronary heart disease risk factors in systemic lupus erythematosus? Arthritis and Rheumatism 59: 169.

King JK and Hahn BH (2007) Systemic lupus erythematosus: modern strategies for management – a moving target. Best Practice & Research. Clinical Rheumatology 21: 971–987.

Kyttaris VC , Juang YT and Tsokos GC (2005) Immune cells and cytokines in systemic lupus erythematosus: an update. Current Opinion in Rheumatology 17: 518.

Lahita RG (1990) Sex hormones and the immune system. Part 1. Human data. Baillieres Clinical Rheumatology 4: 1–12.

Lockshin MD (2006) Sex differences in autoimmune disease. Lupus 15: 753.

Migita K , Miyashita T , Maeda Y , et al. (2007) Toll‐like receptor expression in lupus peripheral blood mononuclear cells. Journal of Rheumatology 34: 493.

Nived O , Johansen PB and Sturfelt G (1993) Standardized ultraviolet_A exposure provokes skin reaction in systemic lupus erythematosus. LUPUS 2: 247.

Papadimitraki ED , Choulaki C , Koutala E , et al. (2006) Expansion of toll‐like receptor 9‐expressing B cells in active systemic lupus erythematosus: implications for the induction and maintenance of the autoimmune process. Arthritis and Rheumatism 54: 3601.

Petri M , Orbai AM , Alarcon GS , et al. (2012) Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for SLE. Arthritis and Rheumatism 64: 2677.

Pons‐Estel GJ , Alarcon GS , Scofield L , et al. (2010) Understanding the epidemiology and progression of SLE. Seminars in Arthritis and Rheumatism 39: 257.

Pugh‐Bernard AE and Cambier JC (2006) B cell receptor signalling in human systemic lupus erythematosus. Current Opinion in Rheumatology 18: 451–455.

Rahman A and Isenberg DA (2008) SLE. New England Journal of Medicine 358: 929.

Ronnblom L and Pascual V (2008) The innate immune system in SLE: type I interferons and dendritic cells. Lupus 17: 394–399.

Schur PH (1995) Genetics of systemic lupus erythematosus. Lupus 4: 425–437.

Sestak AL , Nath SK , Sawalha AH and Harley JB (2007) Current status of lupus genetics. Arthritis Research & Therapy 9: 210–219.

Solomon DH , Kavanaugh AJ and Schur PH (2002) Evidence‐based guidelines for the use of immunologic tests: antinuclear antibody testing. Arthritis and Rheumatism 47: 434–444.

Sturfelt G and Truedsson L (2005) Complement and its breakdown products in SLE. Rheumatology 44: 1227–1232.

Tieng AT and Peeva E (2008) B‐cell‐directed therapies in systemic lupus erythematosus. Seminars in Arthritis and Rheumatism 38: 218–227.

Wang J , Pan HF , Ye DQ , et al. (2008) Moderate alcohol drinking might be protective for SLE: a systematic review and meta‐analysis. Clinical Rheumatology 27: 1557.

Yaniv G , Twig G , Shor DBA , et al. (2014) A volcanic explosion of autoantibodies in systemic lupus erythematosus: A diversity of 180 antibodies found in SLE patients. Autoimmunity Reviews 14 (1): 75–79.

Yurasov S , Wardemann H , Hammersen J , et al. (2005) Defective B cell tolerance checkpoints in systemic lupus erythematosus. Journal of Experimental Medicine 201: 703.

Further Reading

Aladjem H and Schur PH (1986) In Search of the Sun. New York: Scribners.

Lahita R (2004) Systemic Lupus Erythematosus, 4th edn. New York: Churchill Livingstone.

Schur PH and Massarotti EM (eds) (2012) Lupus Erythematosus. Clinical Evaluation and Treatment. New York: Springer.

Schur PH (2014) Multiple chapters on Systemic Lupus Erythematosus in Up To Date in Rheumatology. Wolters Kluwer Health

Wallace DJ and Hahn BH (2006) Dubois' Lupus Erythematosus, 7th edn. Philadelphia: Lippincott.

Contact Editor close
Submit a note to the editor about this article by filling in the form below.

* Required Field

How to Cite close
Schur, Peter H(Jul 2015) Systemic Lupus Erythematosus. In: eLS. John Wiley & Sons Ltd, Chichester. http://www.els.net [doi: 10.1002/9780470015902.a0002147.pub3]