sistemik lupus eritematozus (definite accusative sistemik lupus eritematozusu, plural sistemik lupus eritematozuslar). (healthcare) systemic lupus erythematosus . Protein kaybettiren enteropati ile baflvuran s›rad›fl› bir sistemik lupus eritematozus vakas› sunulmaktad›r. 24 yafl›nda bayan hasta, jeneralize. erythematosus presenting with protein-losing enteropathy Protein kaybettiren enteropati ile seyreden bir sistemik lupus eritematozus vakas.
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Systemic lupus erythematosus SLEalso known simply as lupusis an autoimmune disease in which the body’s immune system mistakenly attacks healthy tissue in many parts of the body. The cause of SLE is not clear. There is no cure for SLE. Rate of SLE varies between countries from 20 to 70 perSLE is one of several diseases known as ” the great imitator ” because it often mimics or is mistaken for other illnesses.
Diagnosis can thus be elusive, with some people having unexplained symptoms of SLE for years. Common initial and chronic complaints include fevermalaisejoint painsmuscle painsand fatigue.
Because these symptoms are so often seen in association with other diseases, these signs and symptoms are not part of the diagnostic criteria for SLE.
When occurring in conjunction with other signs and symptoms see belowhowever, they are considered suggestive. While SLE can occur in both males and females, it is found far more often in women, and the symptoms associated with each sex are different.
Males tend to have more seizureskidney diseaseserositis inflammation of tissues lining the eritematouzs and heartskin problems ulpus, and peripheral neuropathy.
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The three main categories of lesions are chronic cutaneous discoid lupus, subacute cutaneous lupus, and acute cutaneous lupus. People with discoid lupus may exhibit thick, red scaly patches on the skin. Similarly, subacute cutaneous lupus manifests as red, scaly patches of skin but with distinct edges. Acute cutaneous lupus manifests as a rash. Some have the classic malar rash or butterfly rash associated with the disease. Hair lossmouth and nasal ulcers, and lesions on the skin are other possible manifestations.
The most commonly sought medical attention is for joint painwith the small joints of the hand and wrist usually affected, although all joints are at risk. More than 90 percent of those affected will experience joint or muscle eriematozus at some time during the course of their illness.
Fewer than ten percent of people with lupus arthritis will develop deformities eriteatozus the hands and feet. A possible association between rheumatoid arthritis and SLE has been suggested,  and SLE may be associated with an increased risk of bone fractures in relatively young women. People with SLE may have an association with antiphospholipid antibody syndrome  a thrombotic disorderwherein autoantibodies to phospholipids are present in their serum.
Pathology Outlines – Lupus: Systemic lupus erythematosus (SLE)
Abnormalities associated with eritrmatozus antibody syndrome include a paradoxical prolonged partial thromboplastin time which usually occurs in hemorrhagic disorders and a positive test for antiphospholipid antibodies; the combination of such findings have earned the term ” lupus anticoagulant -positive”. Another autoantibody finding in SLE is the anti-cardiolipin antibodywhich can cause a false positive test for syphilis.
SLE may cause pericarditis —inflammation of the outer lining surrounding the heart, myocarditis —inflammation of the heart muscle, or endocarditis —inflammation of the inner lining of the heart. It involves either the mitral valve or the tricuspid valve.
Atherosclerosis also occurs more often and advances more rapidly than in the general population. Inflammation of the pleurae known as pleurisy can rarely give rise to shrinking lung syndrome. Painless passage eriteamtozus blood or protein in the urine may often be the only presenting sign of kidney involvement.
Acute or chronic renal impairment may develop with lupus nephritisleading to acute or end-stage kidney failure. The histological hallmark of SLE is luous glomerulonephritis with “wire loop” abnormalities.
Neuropsychiatric syndromes can result when SLE affects the central or peripheral nervous system. The American College of Rheumatology defines 19 neuropsychiatric syndromes in systemic lupus erythematosus. A common neurological disorder people with SLE have is headache although the existence of a specific lupus headache and the optimal approach to headache in SLE egitematozus remains controversial. Neurological disorders contribute eritematizus a significant percentage of morbidity and mortality in people with lupus.
SLE causes an increased rate of fetal death in utero and spontaneous abortion miscarriage. Neonatal lupus is the occurrence of SLE symptoms in an infant born from a luups with SLE, most commonly presenting with a rash resembling discoid lupus erythematosusand sometimes with systemic abnormalities such as heart block or enlargement eritematozud the liver and eritwmatozus.
Fatigue in SLE is probably multifactorial and has been related to not only disease activity or complications such as anemia or hypothyroidismbut also to paindepressionpoor sleep quality, poor physical fitness and lack of social support.
SLE is presumably caused by a genetic susceptibility coupled with an environmental trigger which results in defects in the immune system. One of the factors associated with SLE is vitamin D deficiency.
The first mechanism may arise genetically. Research indicates SLE may have a genetic link. SLE does run in families, but eritematozua single causal gene has been identified. Lupsu, multiple genes appear to influence a person’s chance of developing lupus when triggered by environmental factors. While the genetics of SLE are not very well understood, there is growing evidence for the involvement of specific genes in this complex autoimmune disease.
Part of the complexity of this disease is due to the effects of both environment and genetics factors that may contribute to eritematozjs development. Genome-wide association studies GWAS revealed regions of linkage that were found on most chromosomes. Candidate gene loci implicated with SLE include multiple alleles from the HLA region, Fc-gamma receptorand complement erutematozus system.
Since SLE is associated with so many genetic regions, it is likely an oligogenic trait, meaning that there are several genes that control susceptibility to the disease. Cells derived from SLE patients are unable to repair DNA damages as efficiently as control cells, and it has been suggested that aberrant repair of DNA damages could lead to the development of lupus .
SLE is regarded as a prototype disease due to the significant overlap erirematozus its symptoms with other autoimmune diseases.
Drug-induced lupus erythematosus is a generally reversible condition that usually occurs in people being treated for a long-term illness.
Drug-induced lupus mimics SLE. However, symptoms of drug-induced lupus generally disappear once the medication that triggered the episode is stopped.
More than 38 medications can cause this condition, the most common of which are procainamideisoniazidhydralazinequinidineand phenytoin. Discoid cutaneous lupus is limited to skin symptoms and is diagnosed by biopsy of rash on the face, neck, scalp or arms.
One manifestation of SLE is abnormalities in apoptosisa type of programmed cell death in which aging or damaged cells are neatly disposed of luupus a part of normal growth or functioning. In SLE, the lhpus immune system produces antibodies against itself, particularly against proteins in the cell nucleus. SLE is triggered by environmental factors that are unknown. The immune system must balance between being sensitive enough to protect against infection, and become sensitized to attack the eritsmatozus own proteins autoimmunity.
During an immune reaction to a foreign stimulus, such as bacteria, virus, or allergen, immune cells eritematozuss would normally be deactivated due to their affinity for self-tissues can be abnormally activated by signaling sequences of antigen-presenting cells.
Thus eeritematozus may include viruses, bacteria, allergens IgE and other hypersensitivityand can be aggravated by environmental stimulants such as ultraviolet light and certain drug eriteatozus. These stimuli begin a reaction that leads to destruction of other cells in the body and exposure of their DNA, histonesand other proteins, particularly parts of the cell nucleus. The body’s sensitized B-lymphocyte cells will now produce antibodies against these pupus proteins.
These antibodies clump into antibody-protein complexes which stick to surfaces and damage blood vessels in critical areas of the body, such as the glomeruli of the kidney; these antibody attacks are the cause of SLE. Researchers are now identifying the individual genes, the proteins they produce, and their role in the immune system. Each protein is a link on the autoimmune chain, and researchers are trying to find drugs to break each of those links. People with SLE have intense polyclonal B-cell activation, with a population shift towards immature B cells.
T cells, which regulate B-cell responses and infiltrate target tissues, have defects in signaling, adhesion, co-stimulation, gene transcription, and alternative splicing.
In the complement system low C3 levels are associated with systemic lupus erythematosus . These cells normally engulf B cells that have undergone apoptosis after somatic hypermutation. Also, uningested apoptotic nuclei can be found outside of TBMs. This material may present a threat to the tolerization of B cells and T cells.
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Dendritic cells in the germinal center may endocytose such antigenic material and present it to T cells, activating them. Also, apoptotic chromatin and nuclei may attach to the surfaces of follicular dendritic cells and make this material available for activating other Llupus cells that may have randomly acquired self-specificity through somatic hypermutation. Impaired clearance of dying cells is a potential pathway for the development of this systemic autoimmune disease.
This includes deficient phagocytic activity and scant serum components in addition to increased apoptosis. SLE is associated with defects in apoptotic clearance, and the damaging eritemqtozus caused by apoptotic debris. When apoptotic material is not removed correctly by phagocytes, they are captured instead by antigen-presenting cells, which leads to development of antinuclear antibodies.
Monocytes isolated from whole blood of people with SLE show reduced expression of CD44 surface molecules involved in the uptake of apoptotic cells. Most of the monocytes and tingible body macrophages TBMs lupuus, which are found in the germinal centres of lymph nodeseven show a definitely different morphology; they are smaller or scarce and die earlier.
Serum components like complement factors, CRPand some glycoproteins are, furthermore, decisively important for an eritemstozus operating phagocytosis. With SLE, these components are often missing, diminished, or inefficient. Recent research has found lipus association between certain people with lupus especially those with lupus nephritis and an impairment in degrading neutrophil extracellular traps NETs. The clearance of early apoptotic cells is an important function in multicellular organisms.
It leads to a progression of the apoptosis process efitematozus finally to secondary necrosis of the cells if this ability is disturbed. Necrotic cells release nuclear fragments as potential autoantigensas well as internal danger signals, inducing maturation of dendritic cells DCssince they have lost their membranes’ integrity. Increased appearance of apoptotic cells also stimulates inefficient clearance. That leads to maturation of DCs and also to the presentation of intracellular antigens of late apoptotic or secondary necrotic cells, via MHC molecules.
Autoimmunity possibly results by the extended exposure to nuclear and intracellular autoantigens derived from late apoptotic and secondary necrotic cells. B and T cell tolerance for apoptotic cells is abrogated, and the llupus get activated by these autoantigens; inflammation and the production of autoantibodies by plasma cells is initiated.
A clearance deficiency in the skin for apoptotic cells has also been observed in people with cutaneous lupus erythematosus CLE. In healthy conditions, apoptotic lymphocytes are removed in germinal eritmatozus GC by specialized phagocytes, the tingible body macrophages TBMwhich is why no free apoptotic and potential autoantigenic material can be seen.
In some people with SLE, build up of apoptotic debris can be observed in GC because of an ineffective clearance kupus apoptotic cells.
Autoreactive B cells can accidentally emerge during somatic hypermutation and migrate into the germinal center light zone.
sistemik lupus eritematozus
Autoreactive B cells, maturated coincidentally, normally do not receive survival signals by antigen planted on follicular dendritic cells and perish by apoptosis. In the case of clearance deficiency, apoptotic nuclear debris lu;us in the light zone of GC and gets attached to FDC.
This serves as a germinal centre survival signal for autoreactive B-cells.
After migration into the mantle zone, autoreactive B cells require further survival signals from autoreactive helper T cells, which promote the maturation of autoantibody-producing plasma cells and B memory cells.