Sle is an autoimmune disease in which the immune system attacks its own tissues, causing widespread inflammation and tissue damage in the affected organs. Normally, the immune system protects the body from infection. He also used the term lupus erythematosus and published the fi rst illustrations in his atlas of skin diseases in 1856. Unlike healthy individuals, patients with sle have t and b cells that react to the patients own nucleic acids and binding proteins. Systemic lupus erythematosus sle is an autoimmune disease with multifactorial etiology, in which genetic and environmental factors interplay determining disease susceptibility. Systemic lupus erythematosus is the most common form of the disease. You may experience pain and stiffness, with or without swelling.
Type i interferon inhibitor anifrolumab in active systemic. Systemic lupus erythematosus sle is an autoimmune disease that can affect many organs, including the skin, joints, the central nervous system and the kidneys. Its diversity of clinical features is matched by the complexity of the factors genetic, hormonal, and environmental that cause it, and the array of autoantibodies with which it is associated. Systemic lupus erythematosus sle is a complex autoim mune disease with variable clinical features 1,2. Th e figure 1 natural history of systemic lupus erythematosus. Management of this disease should be individualized and should include both. The wide acceptance and popularity of the first eular recommendations for its management, published in 2008,1 prompted the subsequent development of specific recommendations regarding monitoring, neuropsychiatric and renal disease, as well as for pregnancy and womens health in lupus. Lupus disease definition of lupus disease by medical dictionary. Pathogenesis of human systemic lupus erythematosus. The extreme heterogeneity of the disease has led some investigators to propose that sle represents a syndrome rather than a single disease. This cpg on systemic lupus erythematosus sle is framed within this context. Systemic lupus erythematosus sle, also known simply as lupus, is an autoimmune disease in which the bodys immune system mistakenly attacks healthy tissue in many parts of the body. Mar 29, 2019 systemic lupus erythematosus sle, or lupus, is an autoimmune disease in which a persons immune system attacks various organs or cells of the body, causing damage and dysfunction. Systemic lupus erythematosus genetics home reference nih.
Systemic lupus erythematosus sle, is the most common type of lupus. Systemic lupus erythematosus rhode island medical society. American college of rheumatology classification criteria for. Systemic lupus erythematosus, the most common form of lupus, is a chronic autoimmune disease that can cause severe fatigue and joint pain. It is more common in african americans and people of american indian and asian descent than in white people. Bullous systemic lupus erythematosus bsle is a rare subset of systemic lupus erythematosus sle associated with autoimmunity to type vii collagen. Seek medical care for lupus if you have rapid swelling of one of your extremities, fever over 102 f, or acute abdominal pain or chest pain. Lesions can be segmental or global, and active or chronic, with endocapillary or extracapillary proliferative lesions.
In a phase 2 trial, anifrolumab, a human monoclonal antibody to type i interferon receptor subunit 1, suppressed interferon gene signatures and substantially reduced sle disease activity. Cutaneous lupus, which causes a rash or lesion on the skin, usually when exposed to sunlight. Lupus is a systemic autoimmune disease that occurs when your bodys immune system attacks your own tissues and organs. Systemic lupus erythematosus sle is an autoimmune disease with a prevalence of 36. Common symptoms include painful and swollen joints, fever, chest pain, hair loss, mouth ulcers, swollen. In lupus, the bodys immune system, which normally functions to protect against foreign invaders, becomes hyperactive, forming antibodies that attack normal.
A more thorough categorization of lupus includes the following types. Katarzyna gilekseibert, md abstract systemic lupus erythematosus sle is a chronic, compli cated and challenging disease to diagnose and treat. Systemic lupus erythematosus, its treatment, and future. Sle is a systemic autoimmune disease whose clinical manifestations, course and. Nacetylcysteine reduces disease activity by blocking mammalian target of rapamycin in t cells from systemic lupus erythematosus patients. Although both men and women of all age groups can be affected, women outnumber men almost 10 fold and. The disease takes two formschronic discoid lupus erythematosus dle and the more serious, and sometimes lifethreatening, form, systemic lupus erythematosus. Although the pathogenesis of sle is still unclear, multiple genetic, environmental and hormonal factors are thought to contribute and interact in the development of the disease 1. Systemic lupus erythematosus sle is a chronic autoimmune disease with varied natural history and multisystemic involvement.
Dle features red, raised bumps in the skin, usually on the face and scalp and often a butterfly rash across the bridge of the nose and hair loss alopecia. It can affect the joints, skin, brain, lungs, kidneys, and blood vessels. Symptoms vary between people and may be mild to severe. Aug 19, 2019 systemic lupus erythematosus sle is a chronic autoimmune disease that is challenging to diagnose and manage owing to its clinical heterogeneity and unknown cause. A fever higher than 100 degrees fahrenheit affects many people with lupus. Starting from 1970, several genetic studies in various sle populations have identified numerous susceptibility loci. The pathogenesis is multifactorial and complex precipitating the formation of autoantibodies.
It may affect virtually any organ or structure of the body, especially the skin, kidneys, joints, heart, gastrointestinal tract, brain, and serous membranes membranous linings of organs, joints, and cavities of the body. Genetic causes according to the national resource center on lupus, no less than 50 genes have been linked to lupus. Lupus erythematosus may manifest as systemic disease or in a purely cutaneous form also known as incomplete lupus erythematosus. Systemic lupus erythematosus lupus is a chronic longlasting autoimmune disease that can affect many parts of the body. Lupus was fi rst recognised as a systemic disease with visceral manifestations by moriz kaposi 18371902. Systemic lupus erythematosus has many guises, but the unifying feature is the presence of antibodies against doublestranded dna in almost all patients. Systemic lupus erythematosus overview the lupus initiative. Systemic lupus erythematosus is a systemic autoimmune disease with a worldwide distribution. Slicc, systemic lupus international collaborating clinicsamerican college of rheumatology damage index.
The diagnosis and treatment of systemic lupus erythematosus. Systemic lupus erythematosus is an autoimmune disease that affects many systems, including the skin, musculoskel etal, renal, neuropsychiatric, hematologic, cardiovascular, pulmonary, and. Sle is an autoimmune condition marked by abnormalities of b and t lymphocytes and the complement system. Lupus erythematosus tumidus genetic and rare diseases. Systemic lupus erythematosus an overview sciencedirect topics. The clinical course is variable, with a broad spectrum of organ manifestations. The signs and symptoms of sle vary among affected individuals, and can involve many organs and systems, including the. Disease severity is wide ranging, with most suffering milder. Several previous studies have shown that the risk of ischaemic and haemorrhagic stroke is significantly higher in sle when compared to the general population, particularly in young individuals, representing one of the principal causes of death in these patients. Lupus also known as systemic lupus erythematosus is a disease of the immune system.
Full text get a printable copy pdf file of the complete article 4. Systemic lupus erythematosus sle is the prototypic multisystem. Causes of lupus or systemic lupus erythematosus sle. Systemic lupus erythematosus sle is a chronic inflammatory autoimmune disease that involves collagen tissue throughout the body. Clinical practice guideline on systemic lupus erythematosus.
Here, we sought to confirm the efficacy of anifrolumab versus placebo in a. An inflammatory, multisystem, autoimmune disease of unknown etiology with protean clinical and laboratory. One of the main factors in sle is the interaction between environmental triggers and genetic factors. May 02, 2016 cutaneous lupus erythematosus cle can be divided into acute cutaneous lupus, subacute cutaneous lupus, and chronic cutaneous lupus. Comparison of remission and lupus low disease activity state. Genomewide association study technology has led to the identification of. In 23% of patients with sle, cutaneous involvement is the. Disease activity in systemic lupus erythematosus sle is related to longterm organ damage 1,2. Pdf systemic lupus erythematosus is a systemic autoimmune disease with a worldwide distribution.
One complication in the choice of a treattotarget outcome is the fact that some treatments for disease activity e. Of these, systemic lupus erythematosus also known as sle is the most common and serious form. Lupus systemic lupus erythematosus emedicinehealth. May 06, 2020 systemic lupus erythematosus sle is an autoimmune disorder characterized by antibodies to nuclear and cytoplasmic antigens, multisystem inflammation, protean clinical manifestations, and a. The most common type of lupus is called systemic lupus erythematosus sle, which affects many parts of the body. Lyn class iv disease, the most severe tnip1 and most common subtype of il10 lupus nephritis. Common areas for muscle pain and swelling include the neck, thighs, shoulders, and upper arms. Sle is a chronic disease of variable severity with a waxing and waning course, with signifi cant morbidity that can be fatalif not treated earlyin some patients fi gure 1.
Systemic lupus erythematosus sle is a chronic multisystem disease characterized by diffuse vasculitis and inflammation of connective tissues that may lead to widespread organ damage affecting the kidneys, brain, and joints. Oct 02, 2017 the most common form of lupus is systemic lupus erythematosus sle, the causes of which are detailed below. Diagnosis and management of systematic lupus erythematosus. Lupus can be difficult to diagnose because its signs and symptoms often mimic. Systemic lupus erythematosus an overview sciencedirect. Systemic lupus erythematosus larissa lisnevskaia, grainne murphy, david isenberg systemic lupus erythematosus is a remarkable and challenging disorder. Jun 19, 2015 systemic lupus erythematosus sle is an autoimmune disease with a prevalence of 36. Systemic lupus erythematosus sle is a chronic disease that causes inflammation in connective tissues, such as cartilage and the lining of blood vessels, which provide strength and flexibility to structures throughout the body.
Th e systemic form was further established by osler in baltimore and jadassohn in vienna. Sep 11, 2018 systemic lupus erythematosus sle is a chronic autoimmune disease with varied natural history and multisystemic involvement. Druginduced lupus, similar to sle, which is caused by an overreaction to certain medications. Inflammation caused by lupus can affect many different body systems including your joints, skin, kidneys, blood cells, brain, heart and lungs. Aug 15, 2016 systemic lupus erythematosus, the most common form of lupus, is a chronic autoimmune disease that can cause severe fatigue and joint pain. Type i interferons are involved in systemic lupus erythematosus sle pathogenesis. Symptoms of lupus can range from very mild to lifethreatening. Systemic lupus erythematosus sle has variable presentation, course and prognosis. Aug 09, 2017 lupus is an autoimmune disease that can affect almost every organ in the body. Systemic lupus erythematosus is an autoimmune disease that affects many systems, including the skin, musculoskel etal, renal, neuropsychiatric, hematologic, cardiovascular, pulmonary.
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