Systemic lupus erythematosus in children and adolescents. Sle is a multifactorial disease with unknown exact etiology, however, several. Alexander muacevic and john r adler asad ali, 1 zohaib sayyed, 2 muhammad atif ameer, 3 abdul wahab arif, 4 fnu kiran, 5 ayesha iftikhar, 6 waleed iftikhar, 7 malik qistas ahmad, 8 muhammad bilal malik, 9 vijay kumar, 10 arjan dass. Systemic lupus erythematosus sle is a systemic autoimmune disease. It may show only single organ sign or multiple system sign at the onset. The reported prevalence of systemic lupus erythematosus sle in the united states is 20 to 150 cases per 100,000. This can damage your joints, skin, blood vessels and organs. Sle is an inflammatory autoimmune disorder characterized by autoantibodies to nuclear antigens. It may involve only a single organ, but in its due course, it usually involves multiple organs of the body. He also used the term lupus erythematosus and published the fi rst illustrations in his atlas of skin diseases in 1856. Despite these major advances, there is a paucity of specific targeted therapies for sle. Systemic lupus erythematosus sle is an autoimmune disease in which the bodys immune system attacks its own healthy tissue. Systemic lupus erythematosus is a systemic autoimmune disease with a worldwide distribution. Understanding the epidemiology and progression of systemic.
It can affect the joints, skin, brain, lungs, kidneys, and blood vessels. Systemic lupus erythematosus sle or lupus is a complex and severe rheumatic disease with exceedingly diverse clinical manifestations. The pathophysiology of systemic lupus erythematosus includes the nervous system, digestion, heart, lungs, blood, muscles, bones, kidneys and skin. Moreover, the presentation of patients with the disease can be as diverse as the many systems in the body that it can affect. In women, prevalence rates vary from 164 white to 406 african american per 100,000 2. A number of candidate genes have been identified which increase the risk of developing cle. This book is attractive to both the investigative community and clinicians.
During its course periods of fl ares intercept periods of. Although there is no definate cause of systemic lupus erythematosus giannouli 2006, there is thought to be several factors increasing the likelihood of developing systemic lupus erythematous they include genetics where members of the family have lupus this condition will often increase the childs chances of developing it hemminki 2009. Th e systemic form was further established by osler in baltimore and jadassohn in vienna. Accepted as a classic reference in the area of sle, the previous two editions cover the basic and clinical aspects of the disease. It describes how advances in molecular biology have increased our understanding of this disease. Lupus can be difficult to diagnose because its signs and symptoms often mimic. Aug 28, 2016 pathogenesis and pathophysiology of sle 1. Pathophysiology of cutaneous lupus erythematosus arthritis.
In lupus, the bodys immune system, which normally functions to protect against foreign invaders, becomes hyperactive, forming antibodies that attack normal tissues and organs, including the skin. Unmet medical needs in systemic lupus erythematosus. Due to the chronic and heterogenous nature of the condition, clinical trials of novel therapeutics or treatment strategies are challenging, with some. Lupus is a systemic autoimmune disease that occurs when your bodys immune system attacks your own tissues and organs. Mar 18, 2020 systemic lupus erythematosus sle is an autoimmune disorder characterized by antibodies to nuclear and cytoplasmic antigens, multisystem inflammation, protean clinical manifestations, and a relapsing and remitting course. Systemic lupus erythematosus, third edition has been updated from a very successful book previously offered from another publisher. Systemic lupus erythematosus lupus is a chronic longlasting autoimmune disease that can affect many parts of the body.
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 disease in which the immune system attacks its own tissues, causing widespread inflammation and tissue damage in the affected organs. An overview of the disease pathology and its management monitoring editor. Systemic lupus erythematosus sle is a complex, autoimmune disorder that can have debilitating effects on various organs, including the kidneys. It is characterized by an immune system dysregulation resulting in the production of various autoantibodies and is considered a multifactorial disease with evidence of genetic susceptibility 1. The pathogenesis of systemic lupus erythematosus an update. Pathophysiology is defined as the study the biological and physical. Review this overview and discuss any symptoms you have with your doctor. While systemic lupus can affect any area of the body.
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. Association of systemic lupus erythematosus with c8orfblk and itgamitgax. Systemic lupus erythematosus, fourth edition, provides an understanding of the basic mechanisms as well as the diagnostic and therapeutic aspects of lupus. Understanding the etiology of sle and environmental factors that can initiate or exacerbate the disease may make it possible to avoid those triggers. As lupus has become a paradigm for the study of autoimmune disease, immunologists investigating autoimmunity are. Pathophysiology of lupus pathophysiology of sle dr garys. Systemic lupus erythematosus an overview sciencedirect. In systemic lupus erythematosus sle your immune system attacks healthy cells and tissues by mistake. Lupus is an autoimmune disease, which means that the immune system erroneously acts against its own healthy tissues. It generally involves one organ system at the onset, but can affect other organ systems subsequently. It is suitable for basic scientists looking for detailed coverage of their areas of interest. The prevalence and severity vary with sex, race, ethnicity, and socioeconomic factors. Systemic lupus erythematosus sle, which is simply known as lupus, is an autoimmune disease in which the immune system of the body erroneously onslaughts tissues in various parts of the body which are healthy 1, 2. Systemic lupus erythematosus current medical diagnosis and.
Pathogenesis aetiology or contributing factors pathophysiology key events in development of the disease 3. More than 90% of cases of sle occur in women, frequently starting at childbearing age. Recent data have provided enhanced understanding of these interactions and the mechanism by which they cause disease. Systemic lupus erythematosus sle, is the most common type of lupus. Systemic lupus erythematosus sle is considered a disease primarily of young women, but can occur in anyone. There are various types of rashes in systemic lupus erythematosus sle, the. Introduction systemic lupus erythematosus sle is a multi system connective tissue disorder characterized by the presence of numerous auto antibodies, circulating immune complexes and widespread immunologically determined tissue damage. Systemic lupus erythematosus is an autoimmune disease in which the bodys.
Systemic lupus erythematosus overview the lupus initiative. Systemic lupus erythematosus is an autoimmune disease that affects many systems, including the skin, musculoskel etal, renal, neuropsychiatric, hematologic, cardiovascular, pulmonary. Katarzyna gilekseibert, md abstract systemic lupus erythematosus sle is a chronic, compli cated and challenging disease to diagnose and treat. Read an overview of the pathophysiology of systemic lupus. An extremely complicated and multifactorial interaction among.
Review pathogenesis of systemic lupus erythematosus. Systemic lupus erythematosus sle is the prototypic multisystem autoimmune disorder with a broad spectrum of clinical presentations. Systemic lupus erythematosus is the most common form of the disease. It usually follows a chronic course and hence can also be termed as a chronic disease.
Systemic lupus erythematosus sle, or lupus is a systemic autoimmune disease with multiorgan inflammation. Although both men and women of all age groups can be affected, women outnumber men almost 10 fold and. Genetics and pathogenesis of systemic lupus erythematosus and. Systemic lupus erythematosus sle statpearls ncbi bookshelf. Systemic lupus erythematosus pathogenesis and pathophysiology 2. Bernstein, in comprehensive pediatric hospital medicine, 2007. Systemic lupus erythematosus united states changing health care systems and rheumatic disease, ed. This book provides a comprehensive overview of the basic and clinical sciences of systemic lupus erythematosus. Pdf pathogenesis of systemic lupus erythematosus researchgate.
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. Pdf the exact pathoaetiology of systemic lupus erythematosus sle remains elusive. Evolution of disease burden over five years in a multicenter inception systemic lupus erythematosus cohort. Systemic lupus erythematosus sle is a systemic autoimmune disease with a broad range of clinical manifestations. Pathogenesis of human systemic lupus erythematosus cell press. Systemic lupus erythematosus may also occur in the form of periarticular inflammation that affects the tendon sheaths, the illness causes the patient to experience coexisting fibromyalgia that results to poor sleep, chronic disease, inactivity, mood problems and depression knott 2012. Pathophysiology of lupus is currently being studied in all areas of the disease and many believe it could be a way to abolish the disease. 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. Pathophysiology of druginduced systemic lupus erythematosus. It is a valuable clinical resource for practicing clinicians from different disciplines. Many of its clinical manifestations are secondary to the trapping of antigenantibody complexes in capillaries of visceral structures or to autoantibodymediated destruction of host cells eg, thrombocytopenia.
Lupus was fi rst recognised as a systemic disease with visceral manifestations by moriz kaposi 18371902. This book is distributed under the terms of the creative commons. Common symptoms include painful and swollen joints, fever, chest pain, hair loss, mouth ulcers, swollen. Systemic lupus erythematosus sle is an autoimmune disease that affects many organ systems, is more prevalent in females, and has no known etiology. More than ninety distinguished contributing authorstwenty of them new to this editionprovide comprehensive coverage of every aspect of cutaneous and systemic lupus erythematosus, including definitions, pathogenesis, autoantibodies. Aug 10, 2015 the pathophysiology of cutaneous lupus erythematosus cle encompasses the complex interactions between genetics, the environment, and cells and their products. Systemic lupus systemic lupus erythematosus a multisystem autoimmune disease etiology unknown most common in women, young to most common in women, young to middleaged highly variable clinical presentation highly variable clinical presentation in individual patients harvardmit division of health sciences and technology. Symptoms vary between people and may be mild to severe. Finally, a key role of il10 in curbing autoantibody production and.
The most common type, sle, affects many parts of the body. This book provides a comprehensive overview of the basic and clinical sciences of systemic. Systemic lupus erythematosus sle is a chronic autoimmune inflammatory disorder of unknown etiology in which there is an increased production of. Genetics and pathogenesis of systemic lupus erythematosus. Overall improvements in medical care including the availability of antibiotics, antihypertensives, and renal replacement therapy coupled with the judicious use of glucocorticoid, antimalarial, and immunosuppressive drugs have led to improved survival of sle. It is more common in african americans and people of american indian and asian descent than in white people. Systemic lupus erythematosus sle is an autoimmune disease caused by cells represented in the peripheral blood circulation 57. Advanced handbook of systemic lupus erythematosus laurent. Barondess page images at nap items below if any are from related and broader terms. Established for forty years as the definitive reference on lupus, dubois lupus erythematosus is now in its thoroughly revised, updated seventh edition. This book will provide an introduction to the epidemiology, etiology and. Jun 09, 2004 systemic lupus erythematosus, fourth edition, provides an understanding of the basic mechanisms as well as the diagnostic and therapeutic aspects of lupus. Pathogenesis of systemic lupus erythematosus c c mok, c s lau j clin pathol2003. Mar 31, 2015 systemic lupus erythematosus sle is a complex, autoimmune disorder that can have debilitating effects on various organs, including the kidneys.
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