However, their specific role in the pathogenesis of cutaneous lupus remains unclear. However, research using various mouse strains of spontaneous and inducible. Due to its complex and only partly understood etiology, 1 systemic lupus erythematosus sle has been labeled the cruel mystery by the lupus foundation of america. Symptoms vary between people and may be mild to severe. The pathogenesis of systemic lupus erythematosus sle is complex and remains poorly understood. Systemic lupus erythematosus is a systemic autoimmune disease with a worldwide distribution. Pdf pathogenesis of human systemic lupus erythematosus. Lupus eritematosus sistemik, ulser oral, obat kumur prednison. The loss of tolerance with subsequent immune dysregulation is a consequence of genetic. Systemic lupus erythematosus sle australasian society of. Systemic lupus erythematosus sle is a chronic autoimmune disease with a broad spectrum of clinical presentations and manifestations that can affect virtually any organ or tissue of the body.
Cellular and molecular pathogenesis of systemic lupus. Cutaneous lupus erythematosus cle is a common manifestation among systemic. This work is a courageous account of living with a chronic illness, a powerful lesson in selfdetermination, and a personal declaration of independence. 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. May 23, 2016 what is systemic lupus erythematosus sle. Manifestasi oral lupus eritematosus sistemik dan manajemen komprehensifnya. As lupus has become a paradigm for the study of autoimmune disease, immunologists investigating autoimmunity are becoming more interested in lupus.
Systemic lupus erythematosus, the most common form of lupus, is a chronic autoimmune disease that can cause severe fatigue and joint pain. Systemic lupus erythematosus sle signs and symptoms. Systemic lupus erythematosus combines basic science with clinical science to provide a translational treatment of the disease and is a useful reference for specialists in the diagnosis and management of patients with sle, a tool for measurement of clinical activity for pharmaceutical development and basic research of the disease and a reference. This book is attractive to both the investigative community and clinicians. The genetics and molecular pathogenesis of systemic lupus erythematosus sle in populations of different ancestry. Genetics and pathogenesis of systemic lupus erythematosus and. Systemic lupus erythematosus sle symptoms can be vague and vary from person to person, and consequently diagnosis can be difficult. The precise immunological events that trigger the onset of clinical manifestations of sle are not yet well understood. An extremely complicated and multifactorial interaction among various genetic and environmental factors is probably involved. Common symptoms include painful and swollen joints, fever, chest pain, hair loss, mouth ulcers, swollen lymph nodes, feeling tired, and a red rash which is. Systemic lupus erythematosus larissa lisnevskaia, grainne murphy, david isenberg systemic lupus erythematosus is a remarkable and challenging disorder.
Giris sistemik lupus eritematozus sle, kronik, sebebi bilinmeyen, immunolojik bozukluklarla birlikte, otoimmun karakterli, bircok organ ve sistemi tutan bir bag dokusu hastal. Systemic lupus erythematosus sle is an autoimmune disease characterized by excessive autoantibody production and multiorgan involvement. Studies have revealed metabolic cellular aberrations, which underwrite cell and organ injury, as important contri. Multiple functions of b cells in the pathogenesis of systemic lupus. Jul 17, 2016 lupus erythematosus lupus erythematosus le may be seen in one of two wellrecognized forms systemic acute lupus erythematosus sle profound impact it has on many organs discoid chronic lupus erythematosus dle chronic and localised skin lesions involving the bridge of nose and adjacent cheeks without any. A characteristic feature of sle is the presence of autoantibodies against doublestranded dsdna, histones and nucleosomes, and other chromatin components. Pathogenesis 20 and clinical features 1 introduction systemic lupus erythematosus sle is the prototypic multisystem autoimmune disorder with a broad spectrum of clinical presentations encompassing almost all organs and tissues. See epidemiology and pathogenesis of systemic lupus erythematosus. Signs and symptoms of lupus gi tract abdominal pain mesenteric arteritis pancreatitis ulcers liver enlargement spleenomegaly sexual dysfunction pain fatigueweakness self esteem decreased desire psychosocial dealing with illness fear of death chronic lack of socialization body image changes rash medication. Role of innate cells neutrophils, macrophages and dendritic cells in systemic lupus erythematosus sle pathogenesis.
Familial sle as well as earlyonset juvenile sle studies make it possible to identify monogenic causes of sle. Established for forty years as the definitive reference on lupus, dubois lupus erythematosus is now in its thoroughly revised, updated seventh edition. In lupus, the bodys immune system, which normally functions to protect against foreign invaders, becomes. Apr 25, 2014 systemic lupus erythematosus sle is a systemic autoimmune disease with various clinical manifestations affecting different tissues. Systemic lupus erythematosus sle is a complex, autoimmune disorder that can have debilitating effects on various organs, including the kidneys. Sle is a polygenic disorder that can appear in individuals without a family history of the disease, yet siblings of affected individuals are much more likely to develop sle, and monozygotic twins. Lupus, technically known as systemic lupus erythematosus sle, is an autoimmune disease in. Chronic means that the signs and symptoms tend to last longer than six weeks and often for many years. An update on pathogenesis of systemic lupus erythematosus pedram ahmadpoor, nooshin dalili, mehrdad rostami systemic lupus erythematosus is a group of diseases that seem to become separate entities by etiology in the near future. The classification of glomerulonephritis in systemic lupus. Lupus comprises a range of multisystem disorders involving the deposition of aberrant immune complexes into tissues. 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.
The pathoaetiology of systemic lupus erythematosus sle probably involves complicated and multifactorial interactions among various genetic and environmental factors multiple genes contribute to disease susceptibility, including genes encoding complement and other components of the immune response, in addition to major histocompatibility complex class i and ii genes. The interaction of sex, hormonal milieu, and the hypothalamopituitaryadrenal axis modifies this susceptibility and the clinical. Lupus erythematosus lupus erythematosus le may be seen in one of two wellrecognized forms systemic acute lupus erythematosus sle profound impact it has on many organs discoid chronic lupus erythematosus dle chronic and localised skin lesions involving the bridge of nose and adjacent cheeks without any. Pdf systemic lupus erythematous sle is an autoimmune collagenvascular disease involving. The pathogenesis of systemic lupus erythematosus an update. Digital rights management drm the publisher has supplied this book in encrypted form, which means that you need to install free software in order to unlock and read it. Systemic lupus erythematosus sle, is the most common type of lupus.
Inflammation occurs as a result of autoantibodies attacking organ systems. Reproductive health concerns in women with systemic lupus. However, research using various mouse strains of spontaneous. A stop codon polymorphism of tolllike receptor 5 is associated with resistance to systemic lupus erythematosus. The pathogenesis of lupus nephritis maciej lech and hansjoachim anders department of nephrology, medical clinic and polyclinic iv, university of munich, munich, germany abstract lupus nephritis is an immune complex gn tha t develops as a frequent complication of sle. However, once diagnosed, a combination of prescribed treatment and lifestyle adjustments enables most people with lupus to enjoy an almost normal life. Pdf systemic lupus erythematosus sle is a chronic autoimmune disease affecting multiple organs. Information for behavioral health providers in primary care. The role of microparticles in the pathogenesis of rheumatoid arthritis and systemic lupus erythematosus. Katarzyna gilekseibert, md abstract systemic lupus erythematosus sle is a chronic, compli cated and challenging disease to diagnose and treat.
Unmet needs in the pathogenesis and treatment of systemic. Pathogenesis of systemic lupus erythematosus c c mok, c s lau. A brief insight into systemic lupus erythematosus pathogenesis. Interaction of sex, hormonal milieu, the hpa axis, and defective immune regulation, such as clearance of apoptotic cells and immune complexes, modify this susceptibility. Systemic lupus erythematosus orphanet journal of rare. The disease can affect, for example, the kidneys, skin, blood cells, and nervous system. Aug 29, 2017 systemic lupus erythematosus sle is an autoimmune rheumatic disease with a prevalence of approximately 1 in. Prevalence varies with ethnicity, but is estimated to be about 1 per overall with a female to male ratio of 10. Lupus is a chronic, autoimmune disease that can damage any part of the body skin, joints, andor organs inside the body. Although both men and women of all age groups can be affected, women outnumber men almost 10 fold and. Significant progress has been made in the past decade since the pathogenesis of systemic lupus erythematosus sle was last. It can affect the joints, skin, brain, lungs, kidneys, and blood vessels. 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.
Review pathogenesis of systemic lupus erythematosus. British society for rheumatology guideline for the. Genetics and pathogenesis of systemic lupus erythematosus. Management of paediatric lupus, renal lupus, topical treatment for cutaneous lupus, and drug treatment in pregnancy have been excluded from our literature search and guideline development. Sle or discoid lupus in women receiving hrt for more than two years when compared with nonusers. Sles an autoimmune disease that can affect any tissue or organ in the body, but most often affects the skin, kidneys, and joints.
Review this overview and discuss any symptoms you have with your doctor. Pdf oral manifestations of systemic lupus erythematous and its. Renal involvement in systemic lupus erythematosus sle. Systemic lupus erythematosus sle is an autoimmune disease with a prevalence of 36.
Read an overview of the pathophysiology of systemic lupus. 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. Systemic lupus erythematosus sle is a clinically heterogeneous disease, which is autoimmune in origin and is characterized by the presence of autoantibodies directed against nuclear antigens. The pathogenesis of sle is summarised diagrammatically in fig 1. Systemic lupus erythematosus sle is a systemic autoimmune disease with various clinical manifestations affecting different tissues. The exact pathoaetiology of systemic lupus erythematosus sle remains elusive. Systemic lupus erythematosus sle is an autoimmune rheumatic disease with a prevalence of approximately 1 in. Il17producing cells have been implicated in the pathogenesis of several autoimmune diseases including multiple sclerosis and sle 30, 31. Many of the clinical manifestations are mediated directly or indirectly by antibody formation and the creation of immune complexes. Infectious triggers, genetic background, immunological abnormalities and environmental factors are all supposed to interact for the disease development. Lupus, technically known as systemic lupus erythematosus sle, is an autoimmune disease in which the bodys immune system mistakenly attacks healthy tissue in many parts of the body. Although the term lupus erythematosus was introduced by 19thcentury physicians to describe skin lesions, it took almost 100 years to realize that the disease is systemic and spares no organ.
Multiple genes contribute to disease susceptibility. Multiple genes confer susceptibility to disease development. Lupus, or systemic lupus erythematosus sle, is not an infectious disease but an autoimmune disorder. The pathogenesis of lupus nephritis nephrology now. We performed a medline search of english language articles published from 1st january to 31st december 2016 using mesh terms and free text words for. Systemic lupus erythematosus sle, lupus is characterized by a global loss of selftolerance with activation of autoreactive t and b cells leading to production of pathogenic autoantibodies and tissue injury. Pathogenesis of systemic lupus erythematosus c c mok, c s lau j clin pathol2003.
Pathogenesis of human systemic lupus erythematosus. Pathogenesis of systemic lupus erythematosus journal of. Moulton,1, abel suarezfueyo,1 esra meidan,1,2 hao li, masayuki mizui,3 and george c. Women are seven to nine times more likely than men to have a diagnosis of sle, with a peak incidence during the childbearing years. Systemic lupus erythematosus sle is a rheumatic disease characterized by autoantibodies directed against selfantigens, immune complex formation, and immune dysregulation, resulting in damage to essentially any organ. Health, general apoptosis health aspects immune response observations oxidative stress systemic lupus erythematosus development and progression. Sle is an autoimmune disease in which the immune system attacks its own tissues, causing widespread inflammation and tissue damage in the affected organs. The disease has a waxing and waning course and carries significant morbidity and mortality from some of its complications. The pathophysiology of systemic lupus erythematosus includes the nervous system, digestion, heart, lungs, blood, muscles, bones, kidneys and skin. Systemic lupus erythematosus sle is the prototypic multisystem autoimmune. Systemic lupus erythematosus sle is the prototypical systemic autoimmune disease and is characterized by a clinical course of flares and remissions.
Systemic lupus erythematosus sle is a complex disease characterized by the appearance of autoantibodies against nuclear antigens and the involvement of multiple organ systems, including the kidneys. Overview of the pathogenesis of systemic lupus erythematosus. Sles an autoimmune disease that can affect any tissue or organ in the body, but most often affects. Sep 30, 2011 systemic lupus erythematosus sle is a complex disease characterized by the appearance of autoantibodies against nuclear antigens and the involvement of multiple organ systems, including the kidneys. Influence of functional interleukin 10tumor necrosis factoralpha polymorphisms on interferonalpha, il10, and regulatory t cell population in patients with systemic lupus. The diagnosis and treatment of systemic lupus erythematosus. Systemic lupus erythematosus sle signs and symptoms, pathophysiology, investigations, treatment. Systemic lupus erythematosus, fourth edition, provides an understanding of the basic mechanisms as well as the diagnostic and therapeutic aspects of lupus. It has been an increasing challenge to apply new pathogenetic insights to the interpretation of. It is a chronic condition in which the body attacks in own cells and tissues, resulting in inflammation of the skin, joints, blood vessels, brain, lungs, and kidneys. Oral manifestations of systemic lupus erythematous and its. Systemic lupus erythematosus sle is an autoimmune disease mediated by autoantibodies and immune complexes which target nearly every organ in the body. Bsr guidelines on the use of drugs in pregnant patients with rheumatic diseases including lupus have been developed in parallel with this guideline. An update on pathogenesis of systemic lupus erythematosus.
Over the last 30 years, advances in treatment such as use of corticosteroids and immunosuppressants have improved life expectancy and quality of life for patients with lupus and the key unmet needs have therefore changed. Read fulltext medical journal articles from medscape s systemic lupus erythematosus journal articles. The etiology and pathogenesis of sle are discussed in greater detail separately. An extremely complicated and multifactorial interaction among various genetic and environmental factors is probably. Systemic lupus erythematosus sle, or lupus is a systemic autoimmune disease with multiorgan inflammation. Systemic lupus erythematosus journal articles index. The pathogenesis of systemic lupus erythematosus is presented. Signs and symptoms of lupus gi tract abdominal pain mesenteric arteritis pancreatitis ulcers liver enlargement spleenomegaly sexual dysfunction pain fatigueweakness self esteem decreased desire psychosocial dealing with illness fear of death chronic lack of socialization body image changes. T cells have long been thought to play a central role in sle pathogenesis, and t cells from patients with lupus show defects in both signaling and. Mar 31, 2015 systemic lupus erythematosus sle is a complex, autoimmune disorder that can have debilitating effects on various organs, including the kidneys. Sle is a prototype type iii hypersensitivity reaction.
1085 1467 1537 1508 1217 1480 1633 1320 56 723 1100 416 229 1358 1461 165 348 1137 473 1619 800 297 355 861 906 1418 1501 1596 163 855 920 1090 630 830 410 1367