Systemic lupus erythematosus an overview sciencedirect topics. Identifying appropriate therapeutic targets and pursuing these systematically has led to improved care for patients with these diseases and useful guidance for healthcare providers and administrators. We are a professional membership organization committed to improving the care of patients with rheumatic disease and advancing the rheumatology subspecialty. Oct 18, 2016 systemic lupus erythematosus sle is a chronic autoimmune disorder. Diagnostic criteria for systemic lupus erythematosus. This 20 update on the treatment of systemic lupus erythrematosus provides rationale for universal use of antimalarials even absent skin or joint manifestations, but chiefly focuses on the management options for refractory cutaneous, articular, and renal. Druginduced lupus is a lupus like disease caused by certain prescription drugs. It can affect the skin, joints, kidneys, brain, and other organs.
Systemic lupus erythematosus sle is a chronic autoimmune inflammatory disorder of unknown etiology in which there is an increased production of autoantibodies, particularly antidoublestranded dna dsdna antibodies, that promotes formation of immune complexes. It is regarded as the prototypal connective tissue disease, 1 where the key pathogenesis relates to a dysfunctional immune system that results in overproduction of various autoantibodies. Systemic lupus erythematosus sle can involve many parts of the eye, including the eyelid, ocular adnexa, sclera, cornea, uvea, retina and optic nerve. We present a case which describes a 29yearold woman with systemic lupus erythematosus who was treated aggressively with cytotoxic immunosuppression. While the clinical presentation of sle can be quite diverse because the disease can affect virtually any. Systemic lupus erythematosus powerpoint templates w. Systemic lupus erythematosus free download as powerpoint presentation. Systemic lupus erythematosus sle is a chronic autoimmune disorder. Jun 19, 2015 systemic lupus erythematosus sle is an autoimmune disease with a prevalence of 36.
This 20 update on the treatment of systemic lupus erythre matosus provides rationale for universal use of antimalarials even absent skin or joint manifestations, but chiefly focuses on the. Systemic lupus erythematosus disease activity index 2000. Suspecting an underlying inflammatory disorder, immunosuppression with oral prednisone and methotrexate was commenced. Ppt systemic lupus erythematosus powerpoint presentation. A characteristic feature of sle is the presence of autoantibodies against doublestranded dsdna, histones and nucleosomes, and other chromatin components. New therapies for systemic lupus erythematosus past.
Systemic lupus erythematosus sle is a chronic autoimmune disease characterized by the chronic activation of the immune system, leading to the formation of autoantibodies and multiorgan damage. Systemic lupus erythematosus safaa eid medical surgical and critical care. However, the care of children and adolescents with sle is very different from. Five years later and approximately 12 weeks pregnant, she is confirmed as having carcinoma of the tongue. Discoid lupus erythematosus is a chronic skin disorder red, raised rash appears on the face, scalp, or elsewhere the rash may last for days or years. Vyse, in genomic and personalized medicine second edition, 20.
Dubois lupus erythematosus and related syndromes ninth edition. It is suitable for basic scientists looking for detailed coverage of their areas of interest. These data should be considered preliminary until published in a. In this seminar we reflect on changes in its classification criteria. This 20 update on the treatment of systemic lupus erythrematosus provides rationale for universal use of antimalarials even absent skin or joint manifestations, but chiefly focuses on the management options for refractory cutaneous, articular, and renal disease and current sta. Systemic lupus erythematosus sle is associated with an increased risk of coronary heart disease chd. Systemic lupus erythematosus sle is a heterogeneous condition characterized by a wide range of symptoms, rendering its diagnosis an often difficult task. 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 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, 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. 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. Systemic lupus erythematosus sle is a chronic inflammatory autoimmune disease involving multiple systems, with a remitting or relapsing course. The initial presentation of lupus often mimics a viral syndrome. The presentation, disease course, and outcome of sle are unpredictable.
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. Genes associated with systemic lupus erythematosus gene locus gene name gene product signal transducer and 2q32. Unfortunately, this is more easily said than done, because there are few large studies to guide decisionmaking. However, it is likely that these people already had systemic lupus, with the skin rash as their main symptom. Pathophysiology of systemic lupus erythematosus free download as powerpoint presentation. The diagnosis and treatment of systemic lupus erythematosus. Treatment of systemic lupus erythematosus 20 update. Sle manifests in a unique way in each patient, and treatment should be tailored to the type and severity of organ system involvement table 221. Systemic lupus erythematosus, pathogenesis request pdf. Patients with sle present in many different ways and therefore may first encounter the medical system in a number of different clinics, including dermatology, nephrology, neurology, haematology or rheumatology, in both adult and paediatric care settings. The symptoms of druginduced lupus are similar to those of systemic lupus, but only rarely will any major organs be affected. 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. Systemic lupus erythematosusassociated neutrophilic. While this fact sheet focuses on sle, and not on less common cutaneousdiscoid lupus skin and mucosa only nor druginduced lupus temporary, a few italicized comments are made regarding the latter two forms of lupus.
Systemic lupus erythematosus 1 systemic lupus erythematosus. Sub acute cutaneous lupus erythematosus refers to skin lesions that appear on parts of the body exposed to sun. Consensus treatment plans for induction therapy of newly diagnosed proliferative lupus nephritis in juvenile systemic lupus erythematosus. Janus kinases jaks are intracellular tyrosine kinases which play a key role in the signal pathways of many cytokines, such as type l interferons ifns, interleukin6 il6, il12 and il23. Systemic lupus erythematosus sle is an autoimmune disease in which the bodys immune system mistakenly attacks healthy tissue.
One month after the initial onset of visual symptoms, the patient developed a malar rash. The slide sets may be used only by the person who downloads or purchases them. Initial presentation of childhoodonset systemic lupus erythematosus. The le cell is a neutrophil that has engulfed the antibodycoated nucleus of another neutrophil. Dubois lupus erythematosus and related syndromes has been recognized internationally as the goto clinical reference on lupus and other connective tissue diseases. Rheumatological assessment and immunological markers were supportive of a diagnosis of systemic lupus erythematosus. An overview of the disease and management options ncbi. 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. 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. It is a multisystem disease, and patients can present in vastly different ways. Systemic lupus erythematosus sle linkedin slideshare.
Founded in 1934, we are a notforprofit, global medical society that serves over 8,400 physicians, health professionals, and scientists worldwide. Systemic lupus erythematosus sle is an autoimmune, multisystemic disease. Systemic lupus erythematosus annals of internal medicine. Recently, an increasing number of reports describe a nonbullous, nonvasculitic sleassociated neutrophilic dermatosis. Sle is a challenging condition that presents unique issues in diagnosis and management. Systemic lupus erythematosus in children pediatrics clerkship. This program will include a discussion of offlabel treatment and investigational agentsnot approved by the fda for use in the us, and data that were presented in abstract form. Systemic lupus erythematosus autoimmunity ncbi bookshelf. Keratoconjunctivitis sicca is the most common manifestation. It is a valuable clinical resource for practicing clinicians from different disciplines. Systemic lupus erythematosus is a systemic autoimmune disease with a worldwide distribution. Tubulointerstitial disease has high impact on prognosis.
From basic scientific principles to practical points of clinical management, the updated 9th edition provides extensive, authoritative coverage of systemic lupus. Hence, inhibition of jak signals has provided an attractive therapeutic option in sle. Systemic lupus erythematosus sle is a heterogeneous, multisystem disease. Systemic lupus erythematosus sle is the prototypic autoimmune disease characterized by multisystem involvement and the production of an array of autoantibodies. The latestage pipeline for systemic lupus erythematosus sle and lupus nephritis ln contains biologics and smallmolecule drugs for a broad range of different targets. If you continue browsing the site, you agree to the use of cookies on this website. Risk of systemic lupus erythematosus in patients with. Consensus treatment plans for induction therapy of newly. New therapeutic strategies in systemic lupus erythematosus. 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. Systemic lupus erythematosus sle is a systemic autoimmune disease characterized by multiple clinical features and autoantibody series.
Systemic lupus erythematosus sle is an autoimmune disease that can affect many organs, including the skin, joints, the central nervous system and the kidneys. 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. Objective the 1980 american college of rheumatology acr classification criteria for systemic sclerosis ssc lack sensitivity for early ssc and limited cutaneous ssc. Figure 1 natural history of systemic lupus erythematosus. The clinical course is variable, with a broad spectrum of organ manifestations. Systemic lupus erythematosus sle is a chronic autoimmune disease characterised by multisystem manifestations.
Systemic lupus erythematosus is the prototypic systemic autoimmune disease characterized by heterogeneous, multisystem involvement. Rnaseq analysis reveals unique transcriptome signatures. The approval of new therapies, especially biologic drugs, for systemic lupus erythematosus sle has been scarce in comparison to rheumatoid arthritis, psoriatic arthritis and ankylosing spondylitis. Systemic lupus erythematosus sle is a chronic, occasionally lifethreatening, multisystem disorder.
Systemic lupus erythematosus sle is a chronic systemic autoimmune disease that. It describes how advances in molecular biology have increased our understanding of this disease. Systemic lupus erythematosus, fifth edition download. Overlap syndrome involving systemic lupus erythematosus and. Complications of systemic lupus erythematosus in the. Systemic lupus erythematosus an overview sciencedirect. Crocker am report 12209 a free powerpoint ppt presentation displayed as a flash slide show on id. Overview of the management and prognosis of systemic lupus. Dubois lupus erythematosus and related syndromes eigth edition, 20. Systemic lupus erythematosus systemic lupus erythematosus. We have made remarkable progress in understanding the genetics of sle over. Systemic lupus erythematosus sle, lupus is a condition in which the immune system attacks healthy cells and tissues throughout the body.
Systemic lupus erythematosus during pregnancy slideshare uses cookies to improve functionality and performance, and to provide you with relevant advertising. Pdf presentation of systemic lupus erythematosus sle in. It predominantly affects younger women, but can occur in up to 20% of pts. The present work, by a joint committee of the acr and the european league against rheumatism eular, was undertaken for the purpose of developing new classification criteria for ssc. Systemic lupus erythematosus diagnosis and management. Webmds slideshow helps you understand the symptoms of lupus, an autoimmune disorder that can affect the skin, joints, and organs. Symptoms vary between people and may be mild to severe.
Prior cases of sleassociated neutrophilic dermatosis describe an urticarial eruption involving the trunk and extremities. This book provides a comprehensive overview of the basic and clinical sciences of systemic lupus erythematosus. Dec 11, 2018 the current treatment approach for systemic lupus erythematosus sle, as outlined in the recommendations by international medical associations including eular and the acr, is mostly eminence. Neutrophilic infiltrates in the setting of systemic lupus erythematosus sle are commonly associated with bullous or vasculitic disease. The four main types of lupus are neonatal and pediatric lupus erythematosus. The inflammation triggered by sle affect many organs in the body, including skin, joints, kidneys, brain, and other organs.
Prevalence varies with ethnicity, but is estimated to be about 1 per overall with a female to male ratio of 10. Omair mbbs, sf rheum consultant rheumatologist assistant professor program director of the ksu rheumatology fellowship a free powerpoint ppt presentation displayed as a flash slide show on id. Common symptoms include painful and swollen joints, fever, chest pain, hair loss, mouth ulcers, swollen. Annals of the rheumatic diseases online first, 12 january 20.
Teasing out the details of this transformation shows how different ways of ordering medical knowledge and practice have framed a series of radically different disease. Systemic lupus erythematosus larissa lisnevskaia, grainne murphy, david isenberg systemic lupus erythematosus is a remarkable and challenging disorder. Purtscherlike retinopathy as a first manifestation of. The principle of treatingtotarget has been successfully applied to many diseases outside rheumatology and more recently to rheumatoid arthritis. Sle is a prototype type iii hypersensitivity reaction. Ocular manifestations in systemic lupus erythematosus. Teasing out the details of this transformation shows how different ways of ordering medical knowledge and practice have framed a series of radically. How did a painfully erosive rash, likened to cancer and then tuberculosis, become a chronic systemic disorder. Dubois lupus erythematosus and related syndromes ninth. Pubmedmedline and scopus databases were searched through june 20 using the terms lupus nephritis, refractory, induction, systemic lupus erythematosus, diffuse proliferative glomerulonephritis, rituximab, cyclosporine, tacrolimus, leflunomide, mizoribine, intravenous immunoglobulin, and belimumab. Systemic lupus erythematosus orphanet journal of rare. Emerging high throughput sequencing technologies have been proved to be a useful platform to understand the complex and dynamic disease processes. The presentation of sle can be complex, considering the number of organ.
Sle affects almost almost every system in the body,with varying degrees of severity. Systemic lupus erythematosus powerpoint presentation. Ocular manifestations of sle are common and may lead to permanent blindness from the underlying disease or therapeutic side effects. Efficacy and safety of epratuzumab in patients with moderatesevere active systemic lupus erythematosus. Lupus comes from a latin word that means wolfan apt metaphor for the history of this savage and elusive condition. Clinical features in individual patients are highly variable, ranging from skin and joint involvement to organthreatening and lifethreatening disease. The rate ratio for myocardial infarction in women with sle 35 to 44 years of age was found to be 52 times that of a comparative population. This 20 update on the treatment of systemic lupus erythre matosus provides rationale for universal use of antimalarials even absent skin or joint. Slicc systemic lupus international collaborating clinicsamerican college of. Systemic lupus erythematosus, pregnancy and carcinoma of. Report by bulletin of the nyu hospital for joint diseases. Health, general antimalarials dosage and administration aspirin belimumab drug approval infliximab lupus drug therapy practice guidelines medicine rituximab skin systemic lupus erythematosus.
Systemic lupus erythematosus shams elmostabal family. Although both men and women of all age groups can be. Presentation of systemic lupus erythematosus sle in emergency department. Sle patients categorised based on autoantibody specificities are reported to have. Apr 25, 2014 systemic lupus erythematosus sle is a systemic autoimmune disease with various clinical manifestations affecting different tissues. Systemic lupus erythematosus sle patients exhibit immense heterogeneity which is challenging from the diagnostic perspective. Introduction systemic lupus erythematosus is a chronic, multisystem, inflammatory, autoimmune disorder characterized by formation of autoantibodies directed against selfantigens and immunecomplex formation resulting in damage to essentially any organ. Prevalence of systemic lupus erythematosus and systemic sclerosis in the first nations population of alberta, canada.
Systemic lupus erythematosus sle is a syndrome of unknown aetiology most commonly affecting young women. Systemic lupus erythematosus and lupus nephritis nature. Although both men and women of all age groups can be affected, women outnumber men almost 10 fold and. Racgp systemic lupus erythmatosus when to consider and. Systemic lupus erythematosus also referred to as sle or lupus date of publication. Prevalence varies with ethnicity, but is estimated to be about 1 per overall with a female. Epratuzumab for systemic lupus erythematosus dj wallace. 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. Systemic lupus erythematosus sle is an autoimmune disease characterized by complex immunological pathogenesis and diverse clinical features, as a consequence of multisystem inflammation. Systemic lupus erythematosus sle is a multisystem autoimmune disorder.
Omair mbbs, sf rheum consultant rheumatologist assistant professor program director of the ksu rheumatology fellowship a free powerpoint ppt presentation displayed as a flash slide show on. Systemic lupus erythematosus powerpoint presentation free. Download systemic lupus erythematosus ppt for free. Not wishing to consider termination of her pregnancy, she underwent surgical resection, which.