Research & Reviews: Journal of Medical and Health Sciences
MenuISSN: 2319-9865
ISSN: 2319-9865
Birla Institute of Technology Mesra, Ranchi, India
Received: 05/05/2015 Accepted: 02/06/2015
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Systemic Lupus Erythematosus, DNA, Complement System, B-cells, autoantibodies
Systemic Lupus Erythematosus is an autoimmune disease [1]. It has various characteristics that are categorized as complications in the human body. It is mainly affected due to genetic, environmental and hormonal factors [2]. The most common lupus disease that has been found to occur in 20-30% of the patients is Lupus Nephritis which again has been divided into six classes [3].
It has been categorized by the presence of various pathogenic autoantibodies as well as inflammatory reactions in the body [2]. These autoantibodies are produced by abnormally self reacting B-cells. These B cells do not get matured in the presence of SLE [19]. Their activation is promoted by B-cells Activating factors which get reduced when the patient is associated with SLE [4].
Systemic Lupus Erythematosus is mainly diagnosed traditionally. The presence of anti double stranded DNA and hypo-complement in the blood serum is used to analyze the presence of systemic lupus in the human body. They are analyzed with their quantity which helps the doctors to find the level of autoimmunity raised inside an individual’s body. However, Now-a day, the researchers have found this method a bit in efficient. According to the paper Li and Zhang, Complement System plays a major role in the procedure of analysis and diagnosis of SLE in the body. Complement activation leads to the production of several complement products [18]. Complement Activation product CD4 produced after the activation of CD complement has been seen to act as a biomarker in an autoimmune diseased body [5].
SLE may affect several organs in the human body, which includes liver, eyes, heart, skin, joints, kidneys, lungs, nervous system, blood elements, and serosal membranes etc [6].
One such example is ocular complication, in which SLE patients have been found with eye disease. SLE can affect any part of the eye including eye lid, orbit, lacrimal system, ocular surface, etc. The most common and severe diseases that have been caused due to SLE are dry eye syndrome and retinopathy. The other eye diseases such as Conjunctiva, Corneal Disease, Episclera, Scleral Disease, Retinal Vasculitis, etc. These ocular complications can be prevented, cured and treated. These can be promptly treated with high dose of systemic corticosteroid and immunosuppressive therapy. These complications can be prevented if they are treated in their earlier stage. The earlier diagnosis, treatment and referral to an ophthalmologist help the patients to get rid of this disease in a better way [6].
Another organ that is affected with SLE is liver. The percentage of liver dysfunction during SLE ranges from 16-60%. The liver related disorders that are found in patients with SLE are Autoimmune Hepatitis, Primary Biliary Cirrhosis, Primary Sclerosing Cholangitis, etc [2]. It is very difficult to recognize the causes of liver dysfunction and liver abnormalities in patients with SLE. Through the survey, it has been revealed that these liver diseases may end up with liver failure or liver cancer. Hence, further treatment and curing methods have to be implemented for the eradication of such issues. For its treatment, several rational models have been built of humoral autoimmunity [7].
Avascular Necrosis, also called as Osteo Necrosis is a common bone disease that has been observed to avail in the patients with Systemic Lupus Erythematosus and Antiphospholipid Syndrome [8]. In this disease, the bone tissues die due to insufficient supply of blood to the bones. This may occur due to dislocation of bones or a fracture. In this disease, the bones are broken leading to ultimately a bone replacement surgery. This generally occurs in longer bones like femoral and humeral bones. SLE can also get associated to other musculoskeletal diseases like neuro-muscular disorders. One such example is spinal muscular atrophy [9].
Women, in their age of 20s and 30s have been found to get frequently affected by SLE [10]. Various experimental studies have revealed that women with SLE get affected in their reproductive system. The ovarian function may get hampered by the cause of autoimmune ovarian disease. The females with SLE have to suffer from various complications if they are in their reproductive age [21]. Firstly, the ovarian reserve is reduced leading to reproductive abnormalities. After then, when individuals are treated with chemotherapy agents then, the toxicity of the compounds may lead to infertility. Ultimately, these abnormalities may lead to fetal loss and adverse effect on ovulation procedure. Hence, these patients should follow several recommendations, if they want to preserve their fertility. If the SLE disease is in moderate condition, the, the patients must refer to the reproductive endocrinologist for their fertility preservation options. The pregnant woman should go for regular check up including blood pressure, renal function, blood content, etc. The use of toxic chemical agents should be avoided to reduce the SLE associated complications.
In a research executed by Williams EM et al. it was found that American and African patients with SLE were reported to be connected with mental distress and depression [22]. This psychological stress was due to the treatments taken during SLE, or may be due to direct involvement of Central Nervous system during its diagnosis. It was found that SLE patients were encountered with depression, anxiety, panic disorders, sleep disorders, several phobias, etc [11]. Various Medical programs and counseling courses were organized for these patients which helped these patients to recover from the psychological stress in a highly productive manner.
SLE may also consequence into cardiovascular complications. A very common example is Libman Sacks endocarditis [20]. In this an abnormal growth of mitral valve starts which may also lead to the blockage of several blood vessels. This abnormal growth occurs due to the increased content of antiphospholipid antibodies [12].
In SLE patients the t-cells are affected with their function. The SLE pathogenesis generally leads to T-cells dysfunction. Konya and Kyttaris have found that there are novel drugs like fostamatinib and anti-IL-17 antibodies which can target these T-cells and help them to activate again with their functionalities [13].
From a review, it was revealed that SLE patients may also get Obesity and its related disorders. These disorders include cardiovascular diseases, diabetes, etc. Weight loss in such individuals has been seen to reduce lupus in the victims. The bariatric surgery has been proved to be a great way of reducing lupus in obese individuals [14].
Systemic Lupus mainly causes death of the healthy cells inside an individual’s body. These are caused due to the production of autoantibodies in the blood. This leads to the destruction of body’s own proteins. In systemic lupus erythematosus, mainly internal organs are affected. These include, heart, lungs, blood vessels, etc. this leads to inflammation in the body which are seen as rashes. In this stage, when blood vessels get affected, the inflammation caused due to arterial infection. This condition is atherosclerosis [15]. This leads to cardiovascular manifestations resulting in heart stroke and arrest [16]. Aortic Atherosclerosis is also one of the common types of atherosclerosis disease, in which the arterial wall gets thickened and stiffer. Ovarian hyperstimulation (OH) just isn't a contraindication in chosen ladies with antiphospholipid syndrome and/or systemic lupus erythematosus (SLE). A younger girl who was once persistently positive antiphospholipid antibodies, low protein free S and homozygous for the paraoxonase G192A mutation underwent three guides of OH; after the fourth direction she developed severe thromboembolism and medical points of SLE despite the usage of a gonadotropin-releasing hormone agonist alongside aspirin that must have minimised the risk of OH syndrome [23].
SLE related to Lymphocytic hypophysitis (LYH) was once suspected. And the medical symptoms and laboratory checks were ameliorated after glucocorticoid treatment (involve twice methylprednisolone (MPIV) pulse therapy and oral forty mg/d of MPIV) combined with cyclophosphamide healing. Even though, LYH related to SLE were mentioned, a 15-year teen, as visible in this case, is a rare first presentation of SLE [24]. CVID is characterized by an accelerated incidence of autoimmune diseases too. Here, we describe a child supplying an abnormal pattern of infections and autoimmune phenomena, pleasurable diagnostic criteria for systemic lupus erythematosus (SLE) [25]. SLE-derived neutrophils spontaneously produce sort I interferons (IFNα), strongly associated with disease development, release chromatin-containing neutrophil extracellular traps (NETs), potentially functioning as a supply of nuclear auto-antigen, and may prompt B cells in a T mobile unbiased trend. In contrast, stages and services of regulatory neutrophils (Nregs) involved in T celldependent B mobile differentiation and germinal middle reactions, are dysregulated in female lupus-susceptible mice for the duration of sickness development [26]. On this city lupus populace, a few causes may have an effect on remedy compliance. Explanations associated with non-compliance are not what had been found in different populations. Further reviews watching into particular reasons for special areas of non-compliance as good as addressing these issues will likely be main in both remedy and results in lupus sufferers in imposing appropriate interventions [27]. Bone diminution in SLE appears to be attributable by means of homocysteine that impact bone formation and bone resorption approach [28]. Brucellosis and systemic lupus erythematosus are two illnesses with exceptional origin and treatment targets characterised by using multi-organ involvement chiefly affecting important and peripheral fearful techniques. It's recognized, that scientific signs of brucellosis are non-detailed and might mimic many different illnesses [29]. The article reports correlating know-how bought from human in vitro characterization and murine in vivo stories and highlights the essential and multifaceted contribution of % to the pathogenesis of systemic autoimmune manifestation [30].
To determine the clinical and serological traits in pediatric systemic lupus erythematosus (SLE). This retrospective be trained integrated 37 sufferers with SLE. All patients fulfilled the ACR revised standards for SLE and diagnosed between 1994 and 2009. Anti-nuclear antibodies had been detected by using indirect immunofluorescence (IIF) on liver rat sections. Anti-dsDNA, anti-Sm, anti-nucleosome, anti-SSA, anti-SSB and anti- RNP antibodies had been detected by ELISA. Anti-dsDNA antibodies were detected also through IIF on Chrithidia luciliae. Probably the most normal signs were anemia (86.5%), proteinuria (seventy three%) and malar rash (sixty seven.6%). The frequency of arthritis and photosensitivity were forty five.9% and forty three.2% respectively. Leucopenia, thrombocytopenia and oral ulcer were reward in 37.Eight%, 32.4% and 18.9% of cases respectively. The frequency of discoid rash used to be 13.5%. Anti-dsDNA antibodies had been detected in 81.1%, anti-Sm and anti-RNP in 56.Eight%, anti-SSA in 43.2% and anti-SSB in 35.1%. The easiest frequency of childhood SLE is headquartered at the age of puberty. Renal disorder is very time-honored in paediatric SLE [31].
The coexistence of systemic lupus erythematosus (SLE) and inflammatory bowel sickness (IBD) is rare. This file stories the English and eastern literature protecting the reported cases of concomitant SLE and IBD. We recognized 17 circumstances of concomitant SLE and Crohn’s ailment (CD) and 13 instances of concomitant SLE and ulcerative colitis (UC). We located that many patients (19/28) developed SLE before IBD. Furthermore, SLE used to be close to under no circumstances lively at presentation of IBD, and flares of SLE have been uncommon after IBD development [32].
The diagnostic standards of SLE are the same in each children and adults. Prognosis of SLE is relatively exclusive from its classification. The prognosis won't fulfill the American institution of Rheumatology (ACR) classification criteria which were outlined and validated for the purposes of medical trials and were not specially developed as diagnostic standards. This differentiation is enormously foremost to preclude inappropriately delayed treatment ready for the classification standards to be fulfilled [33]. The most regularly occurring hematological abnormality used to be anemia which was because of a couple of mechanisms. There was once an inverse association of arthritis with hematological manifestations. A massive quantity of sufferers did not fulfill the ACR criteria at the time of diagnosis however did so on follow up. ACR criteria are vulnerable to diagnose such sufferers and for that reason wants revision. We additionally propose an alternative to ACR standards as “The Kozhikode criteria for SLE [34].
T cells play a vital position in Systemic Lupus Erythematosus (SLE) pathogenesis. The discovery of key steps that result in SLE T cellphone dysfunction allowed a few investigators to advise specific treatments for SLE. Herein, we discuss the capabilities of novel medicines concentrating on SLE T cells, similar to fostamatinib and anti-IL-17 antibodies. Moreover, we discuss using already authorized drugs corresponding to rapamycin, dipyridamole and N acetylcysteine as distinct healing procedures for SLE [35]. Heart valve abnormalities can also be determined in 1 of every three sufferers with systemic lupus erythematosus (SLE), even as valvular vegetations comparable to Libman Sacks endocarditis, are present in 1 on every 10 SLE sufferers. The prognosis of Libman Sacks endocarditis turns into difficult, particularly in differentiating it from infective endocarditis as each ailments may reward in a similar way [36]. The organization of NS and autoimmune problems, comparable to thyroiditis, vasculitis, vitiligo, celiac disorder or anterior uveitis, and SLE has been pronounced in isolated instances or case series previously many years [37]. The effective use of intravenous immunoglobulins in autoimmune problems has been cited by way of a few authors. IVIG has been used to deal with two instances with lupus nephritis, decreasing the proteinuria and decreasing the pathogenic anti-dsDNA antibodies [38].
It is a infrequent case that a younger lupus patient had cardiac involvement no longer only myocardial infarction but additionally pulmonary hypertension. After the prednisone and immunosuppressive drug therapy, mixed with anticoagulation, antiplatelet, and vasodilator remedy the sufferer won dramatic medical growth. Additional medical gain knowledge of could support us to understand the pathology mechanism and effectivity of therapy [39]. Lupus pathogenesis stays incompletely understood and the heterogeneous nature of disease has made scientific investigation extra elaborate in comparison with different rheumatologic illnesses. Moreover, the shortage of consistency in sickness undertaking indices employed in clinical trials has made direct assessment of distinctive treatments in SLE more difficult. There may be additionally a necessity for more distinctive biomarkers that can better reflect lupus disease exercise. However, most important advances in our figuring out of SLE previously ten years have led to a renaissance of research endeavor to establish novel designated sellers to extra without difficulty and safely deal with patients with lupus [40].
From these, we can reveal that SLE pathogenesis has a great influence over the other internal and external organs in a human body. There are various strategies that are needed to be developed for preventing SLE to occur [17].