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. May 02, 2016 cutaneous lupus erythematosus cle can be divided into acute cutaneous lupus, subacute cutaneous lupus, and chronic cutaneous lupus. Lupus disease definition of lupus disease by medical dictionary. Starting from 1970, several genetic studies in various sle populations have identified numerous susceptibility loci. One complication in the choice of a treattotarget outcome is the fact that some treatments for disease activity e. Oct 02, 2017 the most common form of lupus is systemic lupus erythematosus sle, the causes of which are detailed below. 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. Systemic lupus erythematosus is an autoimmune disease that affects many systems, including the skin, musculoskel etal, renal, neuropsychiatric, hematologic, cardiovascular, pulmonary. Aug 09, 2017 lupus is an autoimmune disease that can affect almost every organ in the body. The clinical course is variable, with a broad spectrum of organ manifestations. Systemic lupus erythematosus sle is a chronic autoimmune disease with varied natural history and multisystemic involvement. Disease activity in systemic lupus erythematosus sle is related to longterm organ damage 1,2.
Inflammation caused by lupus can affect many different body systems including your joints, skin, kidneys, blood cells, brain, heart and lungs. Systemic lupus erythematosus sle is a chronic inflammatory autoimmune disease that involves collagen tissue throughout the body. 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. Although both men and women of all age groups can be affected, women outnumber men almost 10 fold and. Here, we sought to confirm the efficacy of anifrolumab versus placebo in a. Disease severity is wide ranging, with most suffering milder. You may experience pain and stiffness, with or without swelling. 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. Systemic lupus erythematosus sle has variable presentation, course and prognosis. Of these, systemic lupus erythematosus also known as sle is the most common and serious form. Pathogenesis of human systemic lupus erythematosus.
Systemic lupus erythematosus larissa lisnevskaia, grainne murphy, david isenberg systemic lupus erythematosus is a remarkable and challenging disorder. Systemic lupus erythematosus is an autoimmune disease that affects many systems, including the skin, musculoskel etal, renal, neuropsychiatric, hematologic, cardiovascular, pulmonary, and. Lesions can be segmental or global, and active or chronic, with endocapillary or extracapillary proliferative lesions. In lupus, the bodys immune system, which normally functions to protect against foreign invaders, becomes hyperactive, forming antibodies that attack normal. The diagnosis and treatment of systemic lupus erythematosus. Systemic lupus erythematosus an overview sciencedirect. Systemic lupus erythematosus is a systemic autoimmune disease with a worldwide distribution. Lupus is a systemic autoimmune disease that occurs when your bodys immune system attacks your own tissues and organs.
Lyn class iv disease, the most severe tnip1 and most common subtype of il10 lupus nephritis. Systemic lupus erythematosus overview the lupus initiative. 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. Bullous systemic lupus erythematosus bsle is a rare subset of systemic lupus erythematosus sle associated with autoimmunity to type vii collagen. Th e systemic form was further established by osler in baltimore and jadassohn in vienna. Symptoms vary between people and may be mild to severe. Nacetylcysteine reduces disease activity by blocking mammalian target of rapamycin in t cells from systemic lupus erythematosus patients. Systemic lupus erythematosus, its treatment, and future. Druginduced lupus, similar to sle, which is caused by an overreaction to certain medications. Comparison of remission and lupus low disease activity state. Lupus erythematosus may manifest as systemic disease or in a purely cutaneous form also known as incomplete lupus erythematosus. Systemic lupus erythematosus genetics home reference nih. The signs and symptoms of sle vary among affected individuals, and can involve many organs and systems, including the. American college of rheumatology classification criteria for.
Type i interferons are involved in systemic lupus erythematosus sle pathogenesis. The pathogenesis is multifactorial and complex precipitating the formation of autoantibodies. The disease takes two formschronic discoid lupus erythematosus dle and the more serious, and sometimes lifethreatening, form, systemic lupus erythematosus. Systemic lupus erythematosus has many guises, but the unifying feature is the presence of antibodies against doublestranded dna in almost all patients. 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. Common areas for muscle pain and swelling include the neck, thighs, shoulders, and upper arms. 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. He also used the term lupus erythematosus and published the fi rst illustrations in his atlas of skin diseases in 1856. Symptoms of lupus can range from very mild to lifethreatening. Genomewide association study technology has led to the identification of. Sle is an autoimmune condition marked by abnormalities of b and t lymphocytes and the complement system. Jun 19, 2015 systemic lupus erythematosus sle is an autoimmune disease with a prevalence of 36. The extreme heterogeneity of the disease has led some investigators to propose that sle represents a syndrome rather than a single disease. 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.
Tumid erythematosus lupus is characterized by smooth, nonscarring, pink to violetcolored pimples papules on the skin without any other apparent skin changes, such as scarring. Type i interferon inhibitor anifrolumab in active systemic. In 23% of patients with sle, cutaneous involvement is the. Management of this disease should be individualized and should include both. 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. Common symptoms include painful and swollen joints, fever, chest pain, hair loss, mouth ulcers, swollen. An inflammatory, multisystem, autoimmune disease of unknown etiology with protean clinical and laboratory. Lupus systemic lupus erythematosus emedicinehealth. Diagnosis and management of systematic lupus erythematosus. Lupus can be difficult to diagnose because its signs and symptoms often mimic. 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 an autoimmune disease with multifactorial etiology, in which genetic and environmental factors interplay determining disease susceptibility. Sle is an autoimmune disease in which the immune system attacks its own tissues, causing widespread inflammation and tissue damage in the affected organs.
Systemic lupus erythematosus rhode island medical society. It can affect the joints, skin, brain, lungs, kidneys, and blood vessels. Systemic lupus erythematosus lupus is a chronic longlasting autoimmune disease that can affect many parts of the body. Clinical practice guideline on systemic lupus erythematosus. 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. Slicc, systemic lupus international collaborating clinicsamerican college of rheumatology damage index. Lupus also known as systemic lupus erythematosus is a disease of the immune system. One of the main factors in sle is the interaction between environmental triggers and genetic factors.
Normally, the immune system protects the body from infection. Systemic lupus erythematosus sle, is the most common type of lupus. Genetic causes according to the national resource center on lupus, no less than 50 genes have been linked to lupus. Systemic lupus erythematosus is the most common form of the disease. Full text get a printable copy pdf file of the complete article 4. 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. It is more common in african americans and people of american indian and asian descent than in white people. Sep 11, 2018 systemic lupus erythematosus sle is a chronic autoimmune disease with varied natural history and multisystemic involvement. Pdf systemic lupus erythematosus is a systemic autoimmune disease with a worldwide distribution. 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. Causes of lupus or systemic lupus erythematosus sle.
Systemic lupus erythematosus sle is an autoimmune disease that can affect many organs, including the skin, joints, the central nervous system and the kidneys. A more thorough categorization of lupus includes the following types. 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. Systemic lupus erythematosus sle is the prototypic multisystem. Katarzyna gilekseibert, md abstract systemic lupus erythematosus sle is a chronic, compli cated and challenging disease to diagnose and treat. This cpg on systemic lupus erythematosus sle is framed within this context.
The most common type of lupus is called systemic lupus erythematosus sle, which affects many parts of the body. 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. Th e figure 1 natural history of systemic lupus erythematosus. Systemic lupus erythematosus an overview sciencedirect topics. 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. Unlike healthy individuals, patients with sle have t and b cells that react to the patients own nucleic acids and binding proteins.
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