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. Study of systemic lupus erythematosus full text view. This includes novel medications, as well as agents that are currently approved for other indications. There are several kinds of lupus systemic lupus erythematosus sle is the form of the disease that most people are referring to when they say lupus. In lupus, the bodys immune system, which normally functions to protect against foreign invaders, becomes. Accepted as a classic reference in the area of sle, the previous two editions cover the basic and clinical aspects of the disease. Sle is an autoimmune disease in which the immune system attacks its own tissues, causing widespread inflammation and tissue damage in the affected organs. American college of rheumatology classification criteria for. The signs and symptoms of sle vary among affected individuals, and can involve many organs and systems, including the skin, joints, kidneys, lungs, central nervous. Although both men and women of all age groups can be affected, women outnumber men almost 10 fold and.
Systemic lupus erythematosus sle is a complex disease caused by complex interactions between genes and the environment sex, age, hormones, smoking, infections, drugs, and abnormalities of both the innate and adaptive immune systems. Lupus can take 46 years and 3 providers before diagnosis. Symptoms vary between people and may be mild to severe. Emerging therapies for systemic lupus erythematosusfocus on targeting interferonalpha. The term discoid refers to a rash of distinct reddened patches covered with. Systemic lupus erythematosus sle is an autoimmune disease with a prevalence of 36. Diagnosis is based on clinical assessment supported by investigations, including the finding of autoantibodies. From a clinical point of view, sle is characterized by a great heterogeneity in terms of clinical and laboratory manifestations. To develop new classification criteria for systemic lupus erythematosus sle jointly supported by the. Treatments range from antimalarial agents to corticosteroids and. The precise immunological events that trigger the onset of clinical manifestations of sle are not yet well understood.
Slicc criteria clinical features immunologic features. Like the other rheu matic diseases, appropriate manage ment is critically dependent upon the proper assessment of disease activity. 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 the prototypic multisystem autoimmune disorder with a broad spectrum of clinical presentations. The disease has been studied in dogs as the animal model for sle in humans. Genetics and pathogenesis of systemic lupus erythematosus. Over 90 percent of sle patients are young women around childbearing age.
Systemic lupus erythematosus sle is a multisystem autoimmune disorder that has a broad spectrum of effects on the majority of organs, including the kidneys. Common symptoms include painful and swollen joints, fever, chest pain, hair loss, mouth ulcers, swollen lymph nodes, feeling tired. The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Although both men and women of all age groups can be. 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. Systemic lupus erythematosus sle statpearls ncbi bookshelf. Systemic lupus erythematosus, the most common form of lupus, is a chronic autoimmune disease that can cause severe fatigue and joint pain. Katarzyna gilekseibert, md abstract systemic lupus erythematosus sle is a chronic, compli cated and challenging disease to diagnose and treat. The etiology of lupus and the contribution of genetic. Osler diagnosed 29 patients who presented with erythema and visceral injuries as sle. The name systemic lupus erythematosus dates back to the early 20th century. The reported prevalence of systemic lupus erythematosus sle in the united states is 20 to 150 cases per 100,000. Systemic lupus erythematosus sle is a clinically and genetically heterogeneous autoimmune disease.
While there is no cure for this autoimmune disease, the goal is to control the progression of the disease through pharmaceutical therapies. Lupus was fi rst recognised as a systemic disease with visceral manifestations by moriz kaposi 18371902. The word lupus is derived from the latin word for wolf and it refers to the characteristic butterflylike rash on the face, which is similar to the white markings on a wolfs face. Systemic lupus erythematosus sle is a systemic autoimmune disease, with multisystemic involvement. Systemic lupus erythematosus sle is a progressive autoimmune disease that results in inflammation and tissue damage characterized by flares, spontaneous remission, and relapses, sle is a chronic disease sle can affect any part of the body but often results in. 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. Manifestations of systemic lupus erythematosus ncbi. Many drugs for the treatment of sle are in various stages of development. Because of its wide range of symptoms, people often confuse lupus with other health problems. In this seminar we reflect on changes in its classification criteria. Systemic lupus erythematosus sle is an autoimmune disorder that can potentially affect any organ by inducing the production of pathogenic autoantibodies directed against cell components and the depo sition of immune complexes. Listing a study does not mean it has been evaluated by the u.
The therapeutic approach relies on the use of corticosteroids and other immunosuppressive agents, depending on the. Systemic lupus erythematosus orphanet journal of rare. Systemic lupus erythematosus is an autoimmune connectivetissue disorder with a wide range of clinical features, which predominantly affects women, especially from certain ethnic groups. Systemic lupus erythematosus larissa lisnevskaia, grainne murphy, david isenberg systemic lupus erythematosus is a remarkable and challenging disorder. 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. Cellular and molecular pathogenesis of systemic lupus. Systemic lupus erythematosus is an autoimmune disease that can affect many organs, including the skin, joints, the central nervous system and the kidneys.
Prevalence varies with ethnicity, but is estimated to be about 1 per overall with a female. Systemic lupus erythematosus genetics home reference nih. Abstract systemic lupus erythematosus sle is the archetypal autoimmune disease given its complex clinical and molecu lar manifestations. Discoid lupus affects only the skin and does not usually involve internal organs. Systemic lupus erythematosus british society for immunology. Disease severity is wide ranging, with most suffering milder. Systemic lupus erythematosus lupus is a chronic longlasting autoimmune disease that can affect many parts of the body. In women, prevalence rates vary from 164 white to 406 african american per 100,000. Teaching fellows in lupus project systemic lupus erythematosus. Systemic lupus erythematosus sle is a chronic, multifaceted autoimmune inflammatory disease that can affect any part of the body. Several genetic studies in various sle populations have identified numerous susceptibility loci.
Genetic factors exert an important role in determining systemic lupus erythematosus sle susceptibility, interplaying with environmental factors. Systemic lupus erythematosus page 1 systemic lupus erythematosus lupus systemic lupus erythematosus, referred to as sle or lupus, is sometimes called the great imitator. Sir william osler 18491919 was the first to use the phrase systemic lupus erythematosus. More than 90% of cases of sle occur in women, frequently starting at childbearing age. Systemic lupus erythematosus sle is an autoimmune disease that impacts many body systems, with a wide variety of signs and symptoms in each patient. 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 is a systemic autoimmune disease with a worldwide distribution. Systemic lupus erythematosus, third edition has been updated from a very successful book previously offered from another publisher.
Systemic lupus erythematosus sle is defined by its clinical features and by the almost invariable presence in the blood of antibodies directed. History of systemic lupus erythematosus sciencedirect. It can affect the joints, skin, brain, lungs, kidneys, and blood vessels. It has been considered the great imitator of many different diseases. Systemic lupus erythematosus sle sle is the prototypic systemic autoimmune disease where the dominant autoimmune response is the production of an array of autoantibodies to self antigens including nuclear components dna, rna, histones as. It is more common in african americans and people of american indian and asian descent than in white people. Acute cutaneous lupus maculopapular lupus rash, malar rash, photosensitive lupus rash, etc. During that time, organ damage can develop leading to 5 fold increased risk of death. Lupus erythematosus, also called lupus, an autoimmune disorder that causes chronic inflammation in various parts of the body.
This third edition includes new chapters and expanded coverage of central nervous. Systemic lupus erythematosus sle, is the most common type of lupus. New horizons in the genetic etiology of systemic lupus. He also used the term lupus erythematosus and published the fi rst illustrations in his atlas of skin diseases in 1856. Systemic lupus erythematosus sle is a clinically heterogeneous disease which is autoimmune in origin, and characterized by the presence of autoantibodies directed against nuclear antigens. The clinical course is variable, with a broad spectrum of organ manifestations. 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. 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. 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. Systemic lupus erythematosus, lupus activity, organ damage. The word systemic means the disease can affect many parts of the body.
There are many different pharmacologic options for treatmentmanagement of systemic lupus erythematosus sle. It is a multisystem disease, and patients can present in vastly different ways. Three main types of lupus are recognizeddiscoid, druginduced, and systemic. Alisa nobee 1, angel justiz vaillant 1, patrick eberechi akpaka 1, peter poonking 2. The diagnosis and treatment of systemic lupus erythematosus.