It can be a burden to the skin, joints, internal organs, and nervous system. It has been considered the great imitator of many different diseases. Systemic lupus erythematosus is one of the connectivetissue diseases whose specific cause or causes remain undetermined. Systemic lupus erythematosus, lahita rg ed, john wiley and sons, new york 1987. Systemic lupus erythematosus sle is a systemic autoimmune disease with a broad range of clinical manifestations.
Two case reports these two cases illustrate the need to consider myocarditis as an uncommon presenting manifestation of systemic lupus erythematosus. Common manifestations may include arthralgias and arthritis, raynaud syndrome, malar and other rashes, pleuritis or pericarditis, renal or central nervous system involvement, and hematologic cytopenias. The cause of the cardiac problems that develop in patients with systemic lupus erythematosus, especially those who are taking corticosteroids. The disease has been studied in dogs as the animal model for sle in humans. Reviewing the patients history revealed that she started experiencing anxiety. This is not a condition i had heard of before i, therefore, questioned her more about it. 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.
The prevalence of systemic sclerosis is lower, however, varying significantly between different studies and countries. 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. In this chapter, we take stock of the impact of systemic lupus erythematosus on the. Type i interferons are involved in systemic lupus erythematosus sle pathogenesis. We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail.
Slicc systemic lupus international collaborating clinics. In the mechanisms of disease article on systemic lupus erythematosus sle, tsokos dec. The word systemic means the disease can affect many parts of the body. Jun 16, 2016 systemic lupus erythematosus sle is an autoimmune disease that can affect many organs, including the skin, joints, the central nervous system and the kidneys. Here, we sought to confirm the efficacy of anifrolumab versus placebo in a. More than 90% of cases of sle occur in women, frequently starting at childbearing age. In the chapter, we present evidence on employment because it plays a crucial. Systemic lupus erythematosus an overview sciencedirect. Systemic lupus erythematosus is systemic autoimmune disease causing significant morbidity and mortality worldwide, particularly in the women of childbearing age. 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 sle is a chronic, occasionally lifethreatening, multisystem disorder. Systemic lupus erythematosus has many guises, but the unifying feature is the presence of antibodies against doublestranded dna in almost all patients. 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. Systemic lupus erythematosus sle is an autoimmune disease with a prevalence of 36.
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. Analysis of the pathologic changes in 120 patients. Genetic variants differentially associated with rheumatoid arthritis and systemic lupus erythematosus reveal the diseasespecific biology. It describes how advances in molecular biology have increased our understanding of this disease. The clinical course is variable, with a broad spectrum of organ manifestations. Three main types of lupus are recognizeddiscoid, druginduced, and systemic. Systemic lupus erythematosus treatment prognosis of sle. Systemic lupus erythematosus is a remarkable and challenging disorder. 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. Systemic lupus erythematosus sle is an autoimmune disease with a strong genetic component and is characterized by chronic inflammation and the production of antinuclear autoantibodies. Although systemic lupus erythematosus is associated with premature myocardial in. It is typically associated with antinuclear antibodies, in particular antidoublestranded dna antibodies, which form immune complexes and can cause multiorgan damage by activating various cell types in genetically susceptible. Discoid lupus affects only the skin and does not usually involve internal organs.
Jun 19, 2015 systemic lupus erythematosus sle is an autoimmune disease with a prevalence of 36. Systemic lupus erythematosus is a systemic autoimmune disease with a worldwide distribution. Mar 27, 2006 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. In this seminar we reflect on changes in its classification criteria. 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 multisystem autoimmune connective tissue disorder with various clinical presentations. Patients may present with a wide array of symptoms, signs, and laboratory findings and have a variable prognosis that depends upon the disease severity and type of organ involvement. In this journal of autoimmunity special issue the most recent findings in the field of sle are addressed. Systemic lupus erythematosus, referred to as sle or lupus, is a chronic longterm disease that causes systemic inflammation which affects multiple organs. This is a pdf file of an unedited manuscript that has. 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. Belimumab for systemic lupus erythematosus nejm dois.
Adapted from epidemiology and classification of systemic lupus erythematosus. Aug 16, 2017 there are many different pharmacologic options for treatmentmanagement of systemic lupus erythematosus sle. This book provides a comprehensive overview of the basic and clinical sciences of systemic lupus erythematosus. Pathogenesis and clinical features 477 disease starts with a preclinical phase characterised by autoantibodies common to other systemic autoimmune diseases and proceeds with a more diseasespecifi c clinically overt autoimmune phase bertsias et al 2010a. The term discoid refers to a rash of distinct reddened. It is a valuable clinical resource for practicing clinicians from different disciplines. Systemic lupus erythematosus journal articles index. In addition to affecting the skin and joints, it can affect other organs in the body such as the kidneys, the tissue lining the lungs pleura, heart pericardium, and brain. It is prevalent among young women with a peak age of onset between the late teens and early 40s and a female to male ratio of 9. Disease severity is wide ranging, with most suffering milder. Systematic lupus erythematosus sle is a chronic disease with a significantly improved life expectancy due to early recognition, diagnosis, monitoring, and therapy of patients. 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. Multiple dermatofibromas in patients with systemic lupus.
Thank you for your interest in spreading the word about the bmj. It is suitable for basic scientists looking for detailed coverage of their areas of interest. It is a multisystem disease, and patients can present in vastly different ways. Review indepth clinical information, latest medical news, and guidelines on lupus, systemic lupus erythematosus sle. Systemic lupus erythematosus sle musculoskeletal and. Genetic variants differentially associated with rheumatoid. Systemic lupus erythematosus sle is defined by its clinical features and by the almost invariable presence in the blood of antibodies directed against one or more components of cell nuclei. When questioned about her medical history, she stated she had a condition called systemic lupus erythematosus. Get a printable copy pdf file of the complete article 477k. Correspondence from the new england journal of medicine anifrolumab in systemic lupus erythematosus. Healthrelated quality of life and employment among persons with. A 20yearold woman with sle presents with disease flares and receives belimumab, a monoclonal antibody that binds to bcell activating. Mifepristone pretreatment for the medical management of early pregnancy loss.
During its course periods of fl ares intercept periods of remission culminating in disease and therapy. Katarzyna gilekseibert, md abstract systemic lupus erythematosus sle is a chronic, compli cated and challenging disease to diagnose and treat. The symptoms of systemic lupus erythematosus will vary from patient to patient as the signs and symptoms displayed will depend on the body part or system that is being affected by the systemic lupus erythematosus arthritis research uk 2014. Epidemiology of connective tissue disorders rheumatology. 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. Lupus erythematosus, also called lupus, an autoimmune disorder that causes chronic inflammation in various parts of the body. Acute lupus erythematosus disseminatus report of a case nejm.
This debilitating and sometimes lifethreatening condition affects an estimated 5 million people worldwide. Systemic lupus erythematosus sle or lupus is an autoimmune disease that can impact multiple systems in the body. 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 sle is a chronic inflammatory autoimmune disease that involves collagen tissue throughout the body. Systemic lupus erythematosus larissa lisnevskaia, grainne murphy, david isenberg systemic lupus erythematosus is a remarkable and challenging disorder. Systemic lupus erythematosus is a chronic, multisystem, inflammatory disorder of autoimmune etiology, occurring predominantly in young women. Vasculitis espanol american college of rheumatology.
Systemic lupus erythematosus orphanet journal of rare. It is more common in certain ethnic groups, such as people with african or asian ancestry. 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 an overview sciencedirect topics. The diagnosis and treatment of systemic lupus erythematosus. Prevalence varies with ethnicity, but is estimated to be about 1 per overall with a female to male ratio of 10. May 29, 2014 systemic lupus erythematosus is a remarkable and challenging disorder. Type i interferon inhibitor anifrolumab in active systemic.1066 444 1572 1518 541 1649 394 1407 1052 1019 40 1096 513 22 1304 1100 1113 30 302 1527 1011 825 1579 657 1546 104 937 475 1125 907 948 1070 1453 1047 834 763 871 457 870 1496