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. Pathogenesis aetiology or contributing factors pathophysiology key events in development of the disease. Systemic lupus erythematosus diagnosis and treatment. An extremely complicated and multifactorial interaction among. Further insight into the pathogenesis of sle and ln may lead to new targets for therapy in the future. Systemic lupus erythematosus sle is a heterogenous disease largely dependent on the generation of autoreactive antibodies by b cells. A brief insight into systemic lupus erythematosus pathogenesis. Learn the clinical presentation of lupus, including the genetic and environmental triggers, symptoms, cell damage and the bodys immune response. Systemic lupus erythematosis sle is a complex and clinically heterogeneous autoimmune disease. Nasopharynx infection of the nasal mucosa, sometimes presenting as a nasal polyp, may reveal a diffuse histiocytic infiltrate that may be misinterpreted as a form of histiocytosis, but upon fite staining, reveals abundant acidfast organisms figure 4. The exact pathoaetiology of systemic lupus erythematosus sle remains elusive. The result of this is slow developing inflammation of the joints. Systemic lupus erythematosus sle is a complex disease characterized by the appearance of autoantibodies against nuclear antigens and.
Disease severity is wide ranging, with most suffering milder. There may be an underlying difference in pathogenesis and possibly genetics between familial and sporadic ln, which can be used in studying the pathogenesis of ln. Certain risk factors have been identified and shown to contribute to disease suscepti bility or activate the. Systemic lupus erythematosus rhode island medical society.
Systemic lupus erythematosus sle is the paradigm of systemic autoimmune diseases characterised by a wide spectrum of clinical manifestations with an unpredictable relapsingremitting course. Pathogenesis of human systemic lupus erythematosus. Belimumab is a monoclonal antibody against bcell activating factor baff and the first biological drug to be approved for the treatment of sle in more than 50 years. The pathogenesis of lupus nephritis involves a variety of pathogenic mechanisms. Cellular and molecular pathogenesis of systemic lupus. New insights into the immunopathogenesis of systemic lupus. An extremely complicated and multifactorial interaction among various genetic and environmental factors is probably involved. 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. Skin deposited igg is a crucial pathologic factor in the development of skin damage in sle. Systemic lupus erythematosus sle is a systemic autoimmune disease with unpredictable disease course, intermingled with periods of remission and exacerbation.
It can be life threatening when major organs are involved. Acomplexinteractionofgenetics,environment,andhormones leads. However, research using various mouse strains of spontaneous and inducible. The full pathological and genetic mechanisms of this complex disease are yet to be elucidated. Systemic lupus erythematosus sle, or lupus is a systemic autoimmune disease with multiorgan inflammation. A characteristic feature of sle is the presence of autoantibodies against doublestranded dsdna, histones and nucleosomes, and other chromatin components. Bacterial infections and the pathogenesis of autoimmune. When disease commences early in life rather than during adulthood, it has a less favorable prognosis, particularly due to multiorgan and kidney involvement 3,4. Nevertheless, infections related to immune suppression, cardiovascular disease, and renal. Systemic lupus erythematosus sle is a complex autoim mune disease with variable clinical features 1,2. Systemic lupus erythematosus pathogenesis and pathophysiology 2. Sle is a chronic multisystem disorder that most commonly affects women during their reproductive years. The disease has a waxing and waning course and carries significant morbidity and mortality from some of its complications.
Pathophysiology of systemic lupus erythematosus pdf. Shlomchik1,3 systemic lupus erythematosus sle is an autoimmune disease characterized by loss of tolerance to self nucleic acids. Sexual disparities in the incidence and course of sle and ra. Type 1 ifns play a central role in sle pathogenesis. Overview of the pathogenesis of systemic lupus erythematosus. Tnfr1 and ifnifnr play an important role in the skin injury of sle. This is a pdf file of an unedited manuscript that has. American college of rheumatology provisional criteria for. Moulton,1, abel suarezfueyo,1 esra meidan,1,2 hao li, masayuki mizui,3 and george c. Systemic lupus erythematosus genetics home reference nih. Sle patients, and they discount the notion that this mycoplasma species has any role to play in the pathogenesis of sle. Pregnant women have a much higher rate of developing lupus.
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 reported prevalence of systemic lupus erythematosus sle in the united states is 20 to 150 cases per 100,000. Mar 31, 2015 systemic lupus erythematosus sle is a multisystem autoimmune disorder that has a broad spectrum of effects on the majority of organs, including the kidneys. A diverse array of genetic factors contribute to the. The signs and symptoms of sle vary among affected individuals, and can involve many organs and systems, including the.
Systemic lupus erythematosus sle is a complex, chronic, multisystem autoimmune in. 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. Sle is an autoimmune disorder characterized by multisystem inflammation with the generation of autoantibodies. Focus of the therapy is adjusted according to the location of the internal sle lesions. Systemic lupus erythematosus sle is an autoimmune disease caused by cells represented in the peripheral blood circulation 57. Systemic lupus erythematosus sle is a chronic systemic autoimmune disease with variable clinical presentation frequently affecting the skin, joints, haemopoietic system, kidneys, lungs and central nervous system. To develop new classification criteria for systemic lupus erythematosus sle jointly supported by the european league against rheumatism eular and the american college of rheumatology acr. Genetics and pathogenesis of systemic lupus erythematosus and. Pdf the exact pathoaetiology of systemic lupus erythematosus sle remains elusive. Viral pathogenesis this lecture will define and discuss the basic principles of viral pathogenesis, the entire process by which viruses cause disease.
There is a high risk of developing lupus and increased maternal morality. In this issue, the role of some of these new players in. Pdf pathogenesis of systemic lupus erythematosus researchgate. Pathogenesis of skin injury of systemic lupus erythematosus. Systemic lupus erythematosus sle is a chronic, multisystem autoimmune disorder that can affect nearly every major organ system, causing inflammation, tissue injury, organ damage, and, ultimately, organ dysfunction. Association of systemic lupus erythematosus with c8orfblk and itgamitgax. Viral disease is a sum of the effects on the host of virus replication and of the immune response. The pathogenesis then progresses into the tissues of skin, nerves, and blood. Sle manifesta tions are associated with multiple autoantibodies, ensuing immune complex formation and deposition, and other immune processes 2,3.
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. Infectious triggers, genetic background, immunological abnormalities and environmental factors are all supposed to interact for the disease development. Immune system aberrations, as well as heritable, hormonal and environmental factors interplay in the expression of organ damage. In women, prevalence rates vary from 164 white to 406 african american per 100,000. Sle often flares during pregnancypostpartum, though it is less likely if sle is in remission at the time of. Nonetheless, there should be no serious doubts that mycoplasma infection can lead to demyelination. Sle is an autoimmune disease in which environmental triggers in genetically susceptible individuals results in the activation of the innate and adaptive immune response, leading to a loss of tolerance to ubiquitous selfantigens. Review sexual disparities in the incidence and course of sle and ra sara k. Tsokos department of cellular injury, walter reed army institute of research, building 503, robert grant avenue, silver spring, md 20910. Nadph oxidase inhibits the pathogenesis of systemic lupus. Systemic lupus erythematosus clinical presentation youtube. Type 1 ifns regulate multiple players of the innate natural killer nk cells, monocytes, myeloid dendritic cells mdcs, neutrophils, humoral b cells, cellmediated t cells immune systems, and also promote cell death.
Brigham and womens hospital, division of rheumatology, immunology and allergy, harvard medical school, 75 francis. 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. Immunological pathogenesis and treatment of systemic lupus. Diagnosis sle sle can involve practically all organ systems. The pathogenesis of systemic lupus erythematosus an update. The source of autoantigen that drives disease onset and progression is unclear. Pdf pathogenesis of human systemic lupus erythematosus. Familial sle as well as earlyonset juvenile sle studies make it possible to identify monogenic causes of sle. Systemic lupus erythematosus sle signs and symptoms. We discuss the role of pathological factors in the development of skin damage in sle. Sle is characterized by production of pathogenic autoantibodies. The demyelinating neuropathy known as guillainbarre gb.
Nadph oxidase inhibits the pathogenesis of systemic lupus erythematosus allison m. Tsokos1, systemic lupuserythematosus sle isachronicautoimmunediseaseaffecting multiple organs. The precise immunological events that trigger the onset of clinical manifestations of sle are not yet well understood. Sle is a prototype type iii hypersensitivity reaction. Apr 25, 2014 systemic lupus erythematosus sle is a systemic autoimmune disease with various clinical manifestations affecting different tissues. We use cookies to offer you a better experience, personalize content, tailor advertising, provide social media features, and better understand the use of our services.
Emerging concepts in the molecular pathogenesis of systemic lupus erythematosus m. Systemic lupus erythematosus is an autoimmune disease that affects many systems, including the skin, musculoskel etal, renal, neuropsychiatric, hematologic, cardiovascular, pulmonary, and. Nambiar et al molecular pathogenesis of sle madhusoodana p. 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. As one of the common places between foreign antigens and autoantigens, molecular mimicry is believed to play a vital role in the pathogenesis of autoimmune. Multiple genes contribute to disease susceptibility. The second most common clinical expression in lupus patients is skin damage that the pathogenesis remains unclear. Emerging concepts in the molecular pathogenesis of systemic. Health, general apoptosis health aspects immune response observations oxidative stress systemic lupus erythematosus development and progression. Aug 10, 2012 the pathogenesis of systemic lupus erythematosus sle is complex and remains poorly understood.
What is the pathogenesis of systemic lupus erythematosus sle. Systemic lupus erythematosus sle is an autoimmune disease with manifestations derived from the involvement of multiple organs including the kidneys, joints, nervous system and hematopoietic organs. Aug 28, 2016 pathogenesis and pathophysiology of sle 1. Evolution of disease burden over five years in a multicenter inception systemic lupus erythematosus cohort. The basic pathological features of sle are that of inflammation and blood vessel abnormalities, which include band or occlusive vasculopathy, vasculitis, and. Cellular and molecular pathogenesis of systemic lupus erythematosus.
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