Nevertheless, infections related to immune suppression, cardiovascular disease, and renal. Sexual disparities in the incidence and course of sle and ra. Overview of the pathogenesis of systemic lupus erythematosus. Disease severity is wide ranging, with most suffering milder. However, research using various mouse strains of spontaneous and inducible.
Moulton,1, abel suarezfueyo,1 esra meidan,1,2 hao li, masayuki mizui,3 and george c. 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 source of autoantigen that drives disease onset and progression is unclear.
Pdf the exact pathoaetiology of systemic lupus erythematosus sle remains elusive. Acomplexinteractionofgenetics,environment,andhormones leads. Nadph oxidase inhibits the pathogenesis of systemic lupus. It can be life threatening when major organs are involved. Shlomchik1,3 systemic lupus erythematosus sle is an autoimmune disease characterized by loss of tolerance to self nucleic acids. American college of rheumatology provisional criteria for.
Systemic lupus erythematosis sle is a complex and clinically heterogeneous autoimmune disease. Pathogenesis of human systemic lupus erythematosus. Pathogenesis of skin injury of systemic lupus erythematosus. Pathophysiology of systemic lupus erythematosus pdf. The reported prevalence of systemic lupus erythematosus sle in the united states is 20 to 150 cases per 100,000. Learn the clinical presentation of lupus, including the genetic and environmental triggers, symptoms, cell damage and the bodys immune response. Nadph oxidase inhibits the pathogenesis of systemic lupus erythematosus allison m. The result of this is slow developing inflammation of the joints.
Tsokos department of cellular injury, walter reed army institute of research, building 503, robert grant avenue, silver spring, md 20910. 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 signs and symptoms of sle vary among affected individuals, and can involve many organs and systems, including the. 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. Immune system aberrations, as well as heritable, hormonal and environmental factors interplay in the expression of organ damage.
A brief insight into systemic lupus erythematosus pathogenesis. 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 an autoimmune disease caused by cells represented in the peripheral blood circulation 57. There is a high risk of developing lupus and increased maternal morality. Pdf pathogenesis of systemic lupus erythematosus researchgate. The pathogenesis then progresses into the tissues of skin, nerves, and blood. 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. Bacterial infections and the pathogenesis of autoimmune.
An extremely complicated and multifactorial interaction among various genetic and environmental factors is probably involved. Systemic lupus erythematosus diagnosis and treatment. Systemic lupus erythematosus clinical presentation youtube. 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. Systemic lupus erythematosus sle is a heterogenous disease largely dependent on the generation of autoreactive antibodies by b cells. Review sexual disparities in the incidence and course of sle and ra sara k. Viral pathogenesis this lecture will define and discuss the basic principles of viral pathogenesis, the entire process by which viruses cause disease. Sle is a chronic multisystem disorder that most commonly affects women during their reproductive years. 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.
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. Pathogenesis aetiology or contributing factors pathophysiology key events in development of the disease. In women, prevalence rates vary from 164 white to 406 african american per 100,000. Emerging concepts in the molecular pathogenesis of systemic lupus erythematosus m.
Skin deposited igg is a crucial pathologic factor in the development of skin damage in sle. 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. 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. Brigham and womens hospital, division of rheumatology, immunology and allergy, harvard medical school, 75 francis. New insights into the immunopathogenesis of systemic lupus. Aug 28, 2016 pathogenesis and pathophysiology of sle 1. The exact pathoaetiology of systemic lupus erythematosus sle remains elusive. Pregnant women have a much higher rate of developing lupus. The second most common clinical expression in lupus patients is skin damage that the pathogenesis remains unclear. Focus of the therapy is adjusted according to the location of the internal sle lesions. Systemic lupus erythematosus sle is a systemic autoimmune disease with unpredictable disease course, intermingled with periods of remission and exacerbation. Diagnosis sle sle can involve practically all organ systems.
This is a pdf file of an unedited manuscript that has. Sle is an autoimmune disorder characterized by multisystem inflammation with the generation of autoantibodies. We discuss the role of pathological factors in the development of skin damage in sle. 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. A characteristic feature of sle is the presence of autoantibodies against doublestranded dsdna, histones and nucleosomes, and other chromatin components. Nambiar et al molecular pathogenesis of sle madhusoodana p.
Systemic lupus erythematosus sle signs and symptoms. Certain risk factors have been identified and shown to contribute to disease suscepti bility or activate the. Sle often flares during pregnancypostpartum, though it is less likely if sle is in remission at the time of. What is the pathogenesis of systemic lupus erythematosus sle. Tnfr1 and ifnifnr play an important role in the skin injury of sle. Systemic lupus erythematosus is an autoimmune disease that affects many systems, including the skin, musculoskel etal, renal, neuropsychiatric, hematologic, cardiovascular, pulmonary, and. Systemic lupus erythematosus rhode island medical society. Pdf pathogenesis of human systemic lupus erythematosus. Health, general apoptosis health aspects immune response observations oxidative stress systemic lupus erythematosus development and progression. Systemic lupus erythematosus sle, or lupus is a systemic autoimmune disease with multiorgan inflammation. An extremely complicated and multifactorial interaction among. Sle is characterized by production of pathogenic autoantibodies. Apr 25, 2014 systemic lupus erythematosus sle is a systemic autoimmune disease with various clinical manifestations affecting different tissues.
Viral disease is a sum of the effects on the host of virus replication and of the immune response. In this issue, the role of some of these new players in. Type 1 ifns play a central role in sle pathogenesis. Systemic lupus erythematosus sle is a complex, chronic, multisystem autoimmune in. The precise immunological events that trigger the onset of clinical manifestations of sle are not yet well understood. Familial sle as well as earlyonset juvenile sle studies make it possible to identify monogenic causes of sle. Systemic lupus erythematosus genetics home reference nih. 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. Emerging concepts in the molecular pathogenesis of systemic. Cellular and molecular pathogenesis of systemic lupus. Systemic lupus erythematosus sle is a complex autoim mune disease with variable clinical features 1,2. Sle is a prototype type iii hypersensitivity reaction. 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.
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 complex disease characterized by the appearance of autoantibodies against nuclear antigens and. Sle patients, and they discount the notion that this mycoplasma species has any role to play in the pathogenesis of sle. Systemic lupus erythematosus sle is the paradigm of systemic autoimmune diseases characterised by a wide spectrum of clinical manifestations with an unpredictable relapsingremitting course. 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. Multiple genes contribute to disease susceptibility. The pathogenesis of lupus nephritis involves a variety of pathogenic mechanisms. The demyelinating neuropathy known as guillainbarre gb. Genetics and pathogenesis of systemic lupus erythematosus and. The pathogenesis of systemic lupus erythematosus an update. 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. 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. 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. Infectious triggers, genetic background, immunological abnormalities and environmental factors are all supposed to interact for the disease development. The full pathological and genetic mechanisms of this complex disease are yet to be elucidated. Nonetheless, there should be no serious doubts that mycoplasma infection can lead to demyelination.
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. Immunological pathogenesis and treatment of systemic lupus. Aug 10, 2012 the pathogenesis of systemic lupus erythematosus sle is complex and remains poorly understood. 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.