Systemic lupus erythematosus, abbreviated as SLE, is an autoimmune disease which primarily strikes women.
Presentation
Majority of the individuals with SLE experience joint pain, accompanied by swelling. Arthritis is also a common complaint. In addition to these, individuals also experience pain in chest, hair loss, and development of sores inside mouth, anemia, malaise, fatigue, fever and sensitivity to light. Affected individuals also develop “butterfly” skin rash. The rash gets worse, when exposed to sunlight, and it commonly appears in areas of bridge of nose and check [8].
Other symptoms of SLE vary with the type of body part that is affected. Individuals also experience numbness, headache, seizures, abdominal pain, arrhythmias, swelling in legs, difficulty in breathing and Raynaud phenomenon [9].
Workup
A preliminary physical examination is done, to carefully study the signs and symptoms of the disease. To be diagnosed with SLE, the individuals should exhibit at least 4 out of 11 common signs and symptoms of the disease.
Antinuclear antibody test is done using the indirect immunofluorescence technique. The pattern of flurorescence that is obtained suggests SLE. In addition, complete blood count, chest X-ray, urinalysis and kidney function tests are also carried out. Depending on the organ involved, other tests would also be required to arrive at a definite conclusion [10].
Treatment
Systemic lupus erythematosus cannot be cured. The symptoms can however be managed, with effective treatment regimes. For mild forms, treatment includes administration of non-steroidal anti-inflammatory drugs (NSAIDs) which are indicated for joint pain, and pleurisy. For skin rashes, corticosteroid creams are also given for topical application.
For severe forms of SLE, high doses of corticosteroids are administered along with cytotoxic drugs. Cytotoxic drugs work by blocking the cell growth and by suppressing the immune system. These drugs are recommended, when corticosteroids do not seem to produce favorable results [11].
Prognosis
The past few decades have witnessed a significant decline in the mortality rates in patients with SLE [6]. It has also been estimated, that with introduction of better treatment methods, about 80% individuals have a 15–year survival rate and more than 90% individuals have a 10-year survival rate. The prognosis of the disease depends on the severity of the condition. Individuals with mild symptoms generally do well with proper treatment. Women with SLE can successfully become pregnant and deliver a baby. However, individuals with underlying disease conditions have poor outcome [7].
Etiology
The exact cause that triggers the immune system to behave in an abnormal fashion is not clearly understood. However, several factors that can trigger the development of SLE are as follows:
- Genetic predisposition: Individuals with family history of SLE are at an increased risk of developing the same.
- Environmental stimuli such as exposure to ultraviolet rays, stress, drugs, trauma and viral agents, are known to play a role in causation of the disease.
- Hormonal involvement: The hormone estrogen can to some extent, trigger attacks of SLE in women of child bearing age [2].
Epidemiology
Annually, about 5 cases of SLE occur in 100,000 populations. According to CDC, in the US, about 1.8 to 7.6 cases occur per 100,000 persons every year. As per the statistics provided by the Lupus Foundation of America, it has been estimated that about 1.5 million Americans live with SLE [3].
SLE is a common phenomenon amongst the women population. In addition, black women are 4 times more likely to develop the autoimmune disease than white women [4].
Pathophysiology
Under normal conditions, the immune system produces antibodies that protect the body against infections. SLE occurs due to abnormalities in apoptosis, which disturbs the immune tolerance ability and speeds up cell death. T cells also have a major role to play in development of SLE. Abnormalities in T cells cause defect in signaling which in turn, leads to development of abnormalities in immune response [5].
In the condition of SLE, the body’s immune system attacks the healthy cells, considering them as invaders, and in the process destroys them. Such sequence of events causes tissue damage, giving rise to various symptoms of swelling and pain. Interplay of environment and genetic factors, are known to play a role in causation of SLE.
Prevention
Onset of SLE cannot be prevented; however, the associated complications can be kept at bay by following certain preventive steps. It is necessary, that individuals with SLE should be closely monitored for several other disease conditions. Individuals should receive all immunizations to prevent development of new disorders. In addition, they are also advised to get regularly tested for osteoporosis. SLE also predisposes an individual to develop heart diseases, and therefore preventive steps should be taken to protect the heart [12].
Summary
In women, systemic lupus erythematosus (SLE) usually begins during the child bearing age. SLE can affect the joints, brain, kidney and other body organs. Apart from women of child bearing age, the condition can also affect children and adults [1]. SLE is a chronic disease condition, wherein the affected individuals experience alternate bouts of mild and severe symptoms. SLE cannot be cured; however the symptoms can be well managed with an appropriate treatment regime.
Patient Information
Definition
Systemic erythematosus lupus (SLE) is an autoimmune disorder, in which the body’s immune system mistakenly attacks the healthy tissues of the body. Such a kind of phenomenon gravely affects the various organs of the body.
Cause
The exact cause of SLE is not known. However interplay of genetic, and environmental factors, is known to trigger abnormal immune response. Individuals with family history of the disease are at an increased risk of developing SLE.
Symptoms
Symptoms of SLE include onset of joint pain and swelling in majority of the cases. In addition, individuals also experience fever, fatigue, malaise, sensitivity to light and development of butterfly shaped rashes.
Diagnosis
A preliminary physical examination followed by antinuclear antibody test is done. This is followed by blood tests, and urinalysis to diagnose underlying disease conditions. Liver function and kidney function tests are also necessary.
Treatment
Mild forms of SLE are treated through non-steroidal anti-inflammatory drugs (NSAIDs) for managing joint pain and pleurisy. Corticosteroid cream is also administered to be applied on the skin rashes. In more severe cases, high dose corticosteroids are administered. If these do not work then cytotoxic drugs are given.
References
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- Cooper GS, Dooley MA, Treadwell EL, et al. Hormonal, environmental, and infectious risk factors for developing systemic lupus erythematosus. Arthritis Rheum 1998; 41:1714.
- Petri M. Epidemiology of systemic lupus erythematosus. Best Pract Res Clin Rheumatol 2002; 16:847.
- Danchenko N, Satia JA, Anthony MS. Epidemiology of systemic lupus erythematosus: a comparison of worldwide disease burden. Lupus. 2006;15(5):308-18.
- Lo MS, Tsokos GC. T cells in systemic lupus erythematosus: progress toward targeted therapy [August 2011]. The Rheumatologist [serial online].
- Trager J, Ward MM. Mortality and causes of death in systemic lupus erythematosus. Curr Opin Rheumatol. Sep 2001;13(5):345-51
- Pons-Estel GJ, Alarcón GS, Scofield L, et al. Understanding the epidemiology and progression of systemic lupus erythematosus. Semin Arthritis Rheum 2010; 39:257.
- Edworthy SM. Clinical Manifestations of Systemic Lupus Erythematosus. In: Harris ED, et al, eds. Kelley's Textbook of Rheumatology. 7th ed. Philadelphia, Pa: WB Saunders; 2005:1201-24.
- Rothfield N. Clinical features of systemic lupus erythematosus. In: Textbook of Rheumatology, Kelley WN, Harris ED, Ruddy S, Sledge CB (Eds), WB Saunders, Philadelphia 1981
- Tierney LM, McPhee SJ, Papadakis MA, eds. Current Medical Diagnosis and Treatment. 40th ed. New York, NY: McGraw-Hill; 2001:841-4.
- Bertsias G, Ioannidis JP, Boletis J, et al. EULAR recommendations for the management of systemic lupus erythematosus. Report of a Task Force of the EULAR Standing Committee for International Clinical Studies Including Therapeutics. Ann Rheum Dis 2008; 67:195.
- Schmajuk G, Yelin E, Chakravarty E, Nelson LM, Panopolis P, Yazdany J. Osteoporosis screening, prevention, and treatment in systemic lupus erythematosus: application of the systemic lupus erythematosus quality indicators. Arthritis Care Res (Hoboken). Jul 2010;62(7):993-1001.