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. Systemic lupus erythematosus sle is a heterogenous disease largely dependent on the generation of autoreactive antibodies by b cells. Sle often flares during pregnancypostpartum, though it is less likely if sle is in remission at the time of. Overview of the pathogenesis of systemic lupus erythematosus. The precise immunological events that trigger the onset of clinical manifestations of sle are not yet well understood. 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.
In this issue, the role of some of these new players in. Immune system aberrations, as well as heritable, hormonal and environmental factors interplay in the expression of organ damage. We discuss the role of pathological factors in the development of skin damage in sle. A diverse array of genetic factors contribute to the. Immunological pathogenesis and treatment of systemic lupus. Association of systemic lupus erythematosus with c8orfblk and itgamitgax. Nonetheless, there should be no serious doubts that mycoplasma infection can lead to demyelination.
Infectious triggers, genetic background, immunological abnormalities and environmental factors are all supposed to interact for the disease development. The reported prevalence of systemic lupus erythematosus sle in the united states is 20 to 150 cases per 100,000. Diagnosis sle sle can involve practically all organ systems. Focus of the therapy is adjusted according to the location of the internal sle lesions. Shlomchik1,3 systemic lupus erythematosus sle is an autoimmune disease characterized by loss of tolerance to self nucleic acids. Tnfr1 and ifnifnr play an important role in the skin injury of sle. 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. Systemic lupus erythematosus sle signs and symptoms. In women, prevalence rates vary from 164 white to 406 african american per 100,000.
Tsokos1, systemic lupuserythematosus sle isachronicautoimmunediseaseaffecting multiple organs. Certain risk factors have been identified and shown to contribute to disease suscepti bility or activate the. The pathogenesis of systemic lupus erythematosus an update. Type 1 ifns play a central role in sle pathogenesis. However, research using various mouse strains of spontaneous and inducible. A characteristic feature of sle is the presence of autoantibodies against doublestranded dsdna, histones and nucleosomes, and other chromatin components. Systemic lupus erythematosus sle is the paradigm of systemic autoimmune diseases characterised by a wide spectrum of clinical manifestations with an unpredictable relapsingremitting course. Pregnant women have a much higher rate of developing lupus. 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. 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. Systemic lupus erythematosus sle is a complex, chronic, multisystem autoimmune in. Aug 10, 2012 the pathogenesis of systemic lupus erythematosus sle is complex and remains poorly understood. Health, general apoptosis health aspects immune response observations oxidative stress systemic lupus erythematosus development and progression.
Nambiar et al molecular pathogenesis of sle madhusoodana p. Systemic lupus erythematosus diagnosis and treatment. Sle is characterized by production of pathogenic autoantibodies. Cellular and molecular pathogenesis of systemic lupus. 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.
Aug 28, 2016 pathogenesis and pathophysiology of sle 1. Pathogenesis of human systemic lupus erythematosus. Systemic lupus erythematosus clinical presentation youtube. Sle patients, and they discount the notion that this mycoplasma species has any role to play in the pathogenesis of sle. Pathogenesis aetiology or contributing factors pathophysiology key events in development of the disease.
Review sexual disparities in the incidence and course of sle and ra sara k. Acomplexinteractionofgenetics,environment,andhormones leads. Systemic lupus erythematosus sle is an autoimmune disease caused by cells represented in the peripheral blood circulation 57. The full pathological and genetic mechanisms of this complex disease are yet to be elucidated. Sexual disparities in the incidence and course of sle and ra. New insights into the immunopathogenesis of systemic lupus. Disease severity is wide ranging, with most suffering milder. Bacterial infections and the pathogenesis of autoimmune. Sle is a prototype type iii hypersensitivity reaction. There is a high risk of developing lupus and increased maternal morality.
Learn the clinical presentation of lupus, including the genetic and environmental triggers, symptoms, cell damage and the bodys immune response. Sle is an autoimmune disorder characterized by multisystem inflammation with the generation of autoantibodies. The signs and symptoms of sle vary among affected individuals, and can involve many organs and systems, including the. 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 complex disease characterized by the appearance of autoantibodies against nuclear antigens and. Pdf pathogenesis of systemic lupus erythematosus researchgate. 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. An extremely complicated and multifactorial interaction among various genetic and environmental factors is probably involved. 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. Pathophysiology of systemic lupus erythematosus pdf. Systemic lupus erythematosus rhode island medical society. Viral disease is a sum of the effects on the host of virus replication and of the immune response. 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.
Genetics and pathogenesis of systemic lupus erythematosus and. Multiple genes contribute to disease susceptibility. Nadph oxidase inhibits the pathogenesis of systemic lupus. Systemic lupus erythematosus genetics home reference nih. Systemic lupus erythematosus is an autoimmune disease that affects many systems, including the skin, musculoskel etal, renal, neuropsychiatric, hematologic, cardiovascular, pulmonary, and. The disease has a waxing and waning course and carries significant morbidity and mortality from some of its complications. 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 brief insight into systemic lupus erythematosus pathogenesis. Pathogenesis of skin injury of systemic lupus erythematosus. 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. It can be life threatening when major organs are involved. The exact pathoaetiology of systemic lupus erythematosus sle remains elusive. Sle is a chronic multisystem disorder that most commonly affects women during their reproductive years.
The demyelinating neuropathy known as guillainbarre gb. The pathogenesis then progresses into the tissues of skin, nerves, and blood. Systemic lupus erythematosus sle, or lupus is a systemic autoimmune disease with multiorgan inflammation. An extremely complicated and multifactorial interaction among. American college of rheumatology provisional criteria for. Familial sle as well as earlyonset juvenile sle studies make it possible to identify monogenic causes of sle. 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. Pdf the exact pathoaetiology of systemic lupus erythematosus sle remains elusive. 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. Emerging concepts in the molecular pathogenesis of systemic lupus erythematosus m. The source of autoantigen that drives disease onset and progression is unclear. Systemic lupus erythematosus sle is a complex autoim mune disease with variable clinical features 1,2. The result of this is slow developing inflammation of the joints. The basic pathological features of sle are that of inflammation and blood vessel abnormalities, which include band or occlusive vasculopathy, vasculitis, and.
Pdf pathogenesis of human systemic lupus erythematosus. Emerging concepts in the molecular pathogenesis of systemic. Systemic lupus erythematosus pathogenesis and pathophysiology 2. Viral pathogenesis this lecture will define and discuss the basic principles of viral pathogenesis, the entire process by which viruses cause disease. Cellular and molecular pathogenesis of systemic lupus erythematosus. 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. The second most common clinical expression in lupus patients is skin damage that the pathogenesis remains unclear. Systemic lupus erythematosis sle is a complex and clinically heterogeneous autoimmune disease. The pathogenesis of lupus nephritis involves a variety of pathogenic mechanisms. Brigham and womens hospital, division of rheumatology, immunology and allergy, harvard medical school, 75 francis. 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. 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. Moulton,1, abel suarezfueyo,1 esra meidan,1,2 hao li, masayuki mizui,3 and george c. Evolution of disease burden over five years in a multicenter inception systemic lupus erythematosus cohort.
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. 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. Systemic lupus erythematosus sle is a systemic autoimmune disease with unpredictable disease course, intermingled with periods of remission and exacerbation. 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. This is a pdf file of an unedited manuscript that has. Tsokos department of cellular injury, walter reed army institute of research, building 503, robert grant avenue, silver spring, md 20910. 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. Sle manifesta tions are associated with multiple autoantibodies, ensuing immune complex formation and deposition, and other immune processes 2,3. What is the pathogenesis of systemic lupus erythematosus sle. Nevertheless, infections related to immune suppression, cardiovascular disease, and renal.
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