Autoimmune disease Systemic lupus erythematosus
Introduction
In the bandage of rheumatic apologies collagenosis have a central role and, without suspicion, systemic lupus erythematosus is one of the most common. The signs of lupus (from Latin wolf) systemic erythematosus (them) begin to the ancient era, prestige is related to the swollen nose of the imperturbable, which adopts a great resemblance to the scratched coat of a wolf, which shows in the Neapias andThe cheeks a malar rash with a butterfly wings. More anomalous is even the report that indicates that the Lupus period does not come to annimal of Latin, but in a frank mask (LOUP, equal carnival wolf) that the courtesans used to the moles. This ailment was considered for the first time in the Middle Ages by the history of its dermatological premises.
The Lupus territory is assigned to the doctor Rogerius (12th century), which used it to blame the university malar exasperation. The Neoclassical Decade declared with the back that Moric Kaposi made, in 1872, of the systemic explanation of the indisposition and the modern with the touch, in 1948, of the cells of the lupus erythematosus – leceings le- (but the system of these cellsAs indicative of opinion, it has been largely separated) and was characterized by progress in the knowledge of pathological physiology and the clinical and laboratory characteristics of the Gotera, as well as by the improvements in the approach. The useful prescription for the dropper was advanced, for the first time, in 1894, when it was disclosed that quina was an effective therapy;Four years later the salicilate avio, in conjunction with the quina, proved even more productive. This was the best vacant treatment for the imperturbable still incomplete of the twentieth century when Philip Showalter Hench discovered the effectiveness of corticosteroids.
It is loyal to that apart from five million heads throughout the cosmos have lupus and that more than 100,000 new hazards are diagnosed every year;Only in Spain around 20,000 any ones live with this drop. This condition is twice as usual than leukemia and ten opportunities more persevering than hemophilia. You can last a long time varied years to diagnose a Lupus inhabitant because the signs are extremely similar to those of several common apologies. The prevalence in the General Villa, at the branch of the area, is between four and 250 events per 100,000 residents: in North America, Asia and in the ideal of Europe it affects 40 out of 100 000 natives, with a maximum incidence betweenThe Hispanic and African American town. About 90% of the risks corresponds to the team of ladies in permanence opimo (women’s/male similarity: 9/1, other creators consider it 10/1). In the United States the incidence and prevalence of systemic lupus erythematosus are maximum in subjects of the black stew than of the white caste, freely of the genital. Other doctorates suggest that in Ibero -American mestizos these chosen occupy an intermediate place between Battalion entertainment. When analyzing the epidemiology of this gotera in the United States it is appreciated that it affects one in 4,000 people and that the companions suffer from five to 15 more often more often than men than men.
Episer’s booklet evidenced in Spain a prevalence of nine episodes per 100,000 inhabitants another buffet carried out in Spain, for which a sign of 154 impassibles was taken, showed that 54% had kinship pathological history of lupus erythematosus systemic or an excuse of the excuse of thecollagen;When reviewing the danger and deliberating the guarism of first -degree kinships with self-immune leaks, the setback of affecting increased them proportionally. By spelling by nature, sheltering a male family member with systemic lupus erythematosus did not increase the danger of self-infammed apologies among the progenies, although if the imperturbable one is relative the danger to a relative is 95%, autonomously of sex. Among monochigotic twins, a unanimity of risks of Systemic Lupus erythematosus from 24% to 58% of the peers have been located, in the meantime that among diced twins, harmony is 2.6%.
In Mexico, reports on their prevalence cite multipliers of 88 percent. In Cuba, morbidity and mortality investigations have been carried out that show that this condition is more natural in effeminate intercourse and that it is in the most benign stews even the most serious;They have described deceased for ins and outs even in 10% of the impassive. In the province of Villa Clara, hospitals and epidemiological explorations have been verified in imperturbable with systemic lupus erythematosTransformation of impassives. A research with clinical, epidemiological, immune and hereditary feature of all imperturbable to project intervention works is currently performed in order to improve their sowing hierarchy and reinforce their survival.
Developing
Systemic lupus erythematosus is an autoimmune excuse of chronic bed, impenetrable complaint and has innumerable externalizations hospitals and multisystemic due to the elaboration of autoantibodies against autoantigens and the appearance of different immunocomplexes that mediate inflammatory responses to depositing in heterogeneous organs and clothsovaries, the soul, the bofes, the ingenuity, the race, the junctures and the cortex, etc.
In the inquiries made in the province of Villa Clara, it has been found that, in all the persevering, the excuse usually undertakes gradually, of facet that the signs appear little by little during a menstrual of vague period;In Bagatelas, months or years spend the performance diagnose the apology. In some imperturbable the beginning is Asaz Torpido, with complications from births, which can date death. A diagnosed opportunity The ailment is left with refrud and exacerbiar jalons. The etiology of the virginal;Although, it is known that there is a variable extraction of autoantibodies that, close to hereditary and environmental delegates may be involved in their pathogenesis. It is truthful that unequal pathogenic and etiological agents are involved, hence the hospital and laboratory wealth presented by the constants with them. The most invoked pathogenic elements are autoantibodies, B and T cells, hereditary, hormonal and environmental aspects and apoptosis. Obtaining autoantibodies is a characteristic tilde of the tenacious with them.
These antibodies can be a key parody in pathogenesis, a consequence of tissue deterioration or proportionally the scar of an unknown etiological agent;Although, not all antibodies are pathogenic, those that have effectiveness to cause tissue laceration are IgG fur and with entry connection by deoxyribonucleic acid (DNA, for their abbreviations in Saxon) bycatenary, which is also an assistant litigation of the interaction betweenB cells and associated T cells CD4+. In the constants with them, the specific T cells has been described for an intelligence autoantigen to conceptualize the elaboration of autoantibodies. There is a maximum prevalence of the apology as constant as in the general city and a dean incidence of common alleles than in an exercise group. A connection of the ailment with indulgent leukocyte (HLA) has been described: HLA-B8, HLA-DR3 and HLA-DR2. A low incidence of coming from the former ailment of menarche or subsequently of menopause.
In imperturbable with them a soluble appearance of the passionate fas protein has been described in which the transmembrane estate is missing. The participation of external agents as inducing agents could be important;Ultraviolet electricity and microorganism play an important role, especially rotavirus, as well as the habit of certain drugs such as hydralacin, procoinamide, isoniacid, methyldopa and chlorpromacin, other others. For the Opinion of the American Society of Rheumatology (American College of Rheumatology) in 1982 proposed 11 category criteria (which were reviewed in 1997) of which four or more are required for the opinion: 1. Facial erythema: Fixed erythema, simple or on high malar magnificence, tending to distribute over the nasolabial folds. two. Lupus Discoid: Erythematous patches on outstanding with adherent and follicular keratosis, atrophic signs that may exist in ancient offenses. 3. PHOTOSENIZIBILITY: RASH OF CORTZE AS REFLUED OF A SINGULAR ALERGY TO THE PAVEMENTED LUMINAR. 4. Oral ulcers: vocal or nasopharyngeal wound, usually non -hurtful, observed by a surgeon. 5. Arthritis: Non -erosive arthritis that compromises two or more peripheral hinges. 6. Serositis: Pericarditis or pleural flow with clinical and analytical tests. 7. Renal disease: Persistent proteinuria> 0.5g/day, micro or macroscopic hematuria, cell laminators composed of red blood cells or hemoglobin or granular, tubular or mixed subject. 8. Neurological alterations: disturbances or disposable. 9. Hematological alterations: Hemolytic anemia with reticulocytosis or leukopenia 2 opportunities towards the high indication value if determined by ELISA). • Anti-SM: Paint of the antibody in front of the SM antigen. • Positivity of antiphospholipid antibodies (AAF): Positive lupus anticoagulant or winged plasma reacin (RPR) False positive or anti-β2 Glucoprotein I positive or positive anti-gardeolipin antibodies. • Low complement (C3, C4 or CH50). • Positive direct coombs in lack of hemolytic anemia. The criteria are cumulative and do not need to be present at the same period.
A tenacious of them is classified if it presents, at least, four of the clinical or immunological criteria;to the separate one must be flagrant one clinical and another immune. It is also classified from the tenacious with renal biopsy compatible with lupus nephritis associated with ana or positive anti-dna.14 The premiere of the excuse is variable, in some risks it is enormously superficial and only constitutional indications are presented;Then, in others it can be very torpid and serious and entangle the biography of the imperturbable. The weakness, anorexia and bankruptcy of yugo are usual signs in the preliminary clinical bank of the them;Titch can be passed at the beginning as in the spatches of apology. The collective of the constants have an affectation of the locomotor baroo in the time of apology, especially arthralgia, morning severity, non -erosive arthritis that affects large and scarce together, myalgia and inflammatory myopathy. Anemia is quite common in these patients, it is serious and it becomes hemolytic. They can gestate thrombopenia, leukopenia and lymphopenia, which generally relate to the indisposition. It should also be endured on scale that some of the worn syrups can produce cytopenias. Cutaneous and mockery conditions appear in 80% of the imperturbable: photosensitivity, mouth ulcers, Malar eczema and discoid evils. Subcutaneous cysts and non -scar -cycatric alopecia are other usual samples;You can still expose leukocytoclastic vasculitis and periungueal infarcts, etc.
Cardiovascular Ñoñería is presented in the generality of the imperturbable, especially pericarditis, but can also emerge myocarditis, which is an atypical expression and occurs in the guise of changes in driving, cardiomegaly and heart failure. Transesophageal echocardiography is the most impressionable experience to spell endocardial devices, which has its most collegial fate in the so-called libman-sacks endocarditis, which produces fibroinflamatory warts. The most spectacular canillas are mitral and aortic and predominates, from the clinical approach, valvular insufficiency on stenosis. Cardioembolic phenomena can be created that predispose valvular overinfection. Some constants can comment on coronary excuse and acute myocardial infarction. Other variations that can be exposed are pulmonary ecclosions, which can harmonize from the blush, the interstitial gatete -pulmonary news, acute lupus pneumonitis and pleural flow even pulmonary bleeding. In them, stomach dislikes that are predominant with vasculitis, pancreatitis or spontaneous peritonitis can appear. Other unusual signs are primary biliary cirrhosis, autoimmune cholangitis and thrombotic oclusive vasculopathy with Budd-Chiari syndrome. Neuropsychiatric externalizations can also occur, which are very variable, including commmocions and mania, cognitive inconveniences and confusional countries, cephalalgia, strokes, transverse myelitis, Guillain Barré syndrome, Sydenham Korea, polyineuritisMotor sensitive, multineuritis and sucks of cranial pairs, etc.
Renal evil is characterized by proteinuria, hematuria, leukocyturia, kidney rollers, agitations in renal recital and arterial hypertension. It is usually accompanied by the positivization of anti-DNA and abajadero antibodies at the levels of complement fractions. Many persevering are with increases in cholesterol and triglycerides levels. Life nephritis can be silent and the generality of the successions corresponds to mesangial glomerulonephritis. Other facets of renal spectacularity are secondary, admirable amyloidosis, and renal quarry thrombosis.32,33 In the province of Villa Clara, a bulge has been proven in the incidence of lupus nephritis as the beginning of a les. These imperturbable have a more torpid transformation than those who debut with other clinical samples, so it could be an indication of bad prediction. Renal biopsy allows clarifying the type of offense and the most appropriate treatment and must be performed by what is optional to minimize the scale of renal damage. Laboratory contests are useful for both the opinion of the condition and to assess the transformation of the persevering, as well as the recipe addition. The most used are: hemoglobin, sedimentation speed, platelets, leukogram with differential, lupus anticoagulant, C3, C4, immunoglobulins, serology, ana, anti-DNA, anti-SM, concretion of antiphospholipid antibodies, Addis count, proteinuria of 24 24hours, echocardiogram, Badana biopsy by immunofluorescence and renal biopsy, among others. Special situations – Lupus in pediatric age: from the town of children with rheumatic evils 2% suffers systemic lupus erythematosus. There are grains devout epidemiological factors. The incidence ranges between 0.4 and 9.0 per 100,000 children. It presents more in the black, oriental and Hindu quality than in Caucasic. In the four-year-old Benjamines, the female-masculine invoice is 2 to 1, it increases in very much 3 to 1 or 5.5 to 1.
The indisposition can originate from acute or insidious guise;In addition the indications precede in years the opinion of the excuse. The most persevering indications in children are tenth, usual discomfort, arthralgia or arthritis and Eccema Malar;However, any of the appearances look at adults may be flagrant.34 In some Azares the Premère is sensationalist, with danger to energy. In Villa Clara, risks have been transmitted that have opened with cerebral infarction, lupus nephritis, Korea, etc. The truth is that the imperturbable in which the première is pomposo have a more torpid and gloomy transformation. Survival monograms reach, in multiple flowery countries, more than 90% at five years and even 88% at 10 years;In Cuba the offices reveal, according to the revolts of Kaplan and Meier, a 95% survival at five years and 88% at 10 and 15 years of fence. Despite these comforting figures, the Albur persists of maximum morbidity due to the treatments used.34 The variety of lupus in the pediatric era is not only affiliated with the difficulties associated with the recipe resolutions but also to the clinical characteristics of the indisposition since there is no characteristic canvas in the start either during the time of its transformation;The multisystemic symbol of the gotera is expressed as a disparate guise in each trance predominantly in some the arrogance of an organ and a method and in others the mixture of several.
Those impassively with lupus must maintain strict corners with an examination of reactants acutely, since an active should congenize with immediacy to minimize living evil. The accompaniment of these imperturbable must be multidisciplinary, with the rectory of the specialists in rheumatology and pediatrics and those of nephrology, cardiology, neurology, psychology and hematology, among others. Therapy with glucocorticoids and immunosuppressive mejunjes is necessary in the approach to all children and adolescents with them. The final sponsorship of erythematous lupus that begins in childhood is not even defined, the future can donate new liniments except warriors, more effective and allow a better caste of history to patients.34 – lupus and tiredness: the clash of pregnancy in the have been inquired and is controversial, especially with memory to the incidence of efforts of the condition during conception. The explorations have reflected that the courage of lupus can correctly increase, frighten or prolong unaltered during pregnancy.35 One of the greatest inconveniences of the troughs with them is the mockery of activation of the dropper during the trouble;But, not all exams agree in this location. The activation event seems to obey the degree of dedication of the dropper in the sister in the six to twelve months ago.35 Glomerulonephritis is an important danger of relapses of Les during pregnancy. This is a lot of maximum if lupus nephritis is active at the moment of pregnancy or if the tiredness occurs highly close, in period, of expedition progress. It is necessary to force flagrant that pre-eclampsia still fosters the manifestation of proteinuria and becomes more difficult to be worth between this and renal sharpening.35,36 Activations can appear in any menstruation of the transformation of pregnancy, multiple months can still occur later;The totality of the exasperations of the condition during the hurry are not usually severe. The view of anti-Rro/SS-A and anti-the/SSB antibodies is associated with fetal damage and is responsible for the neonatal lupus and progress in the grace of engendros, as well as the facade of antiphospholipid antibodies that are equally associatedto fetal depreciation, preterm births and delay in intrauterine progress.
There are useful laboratory parameters to calculate the lupus task that usually vary during boredom: erythrosedimentation increases, hemoglobin decreases and serum levels of C3 and C4 increase. In the active the latter they usually get off, however it does not always happen in this way. SERICAL COMPLEMENT WIRDS may be frequent or increased during pregnancy in hierarchy of other underlying judgments.35 In pregnancy, the point of view must be monitored to minimize defects to the engendro and the cane;The habit of potentially teratogens should be evaded. Glucocorticoids are appropriate to speak systemic lupus erythematoso. Fluorated agents (dexamethasone and betamethasone) reach fetal circulation and achieve unions similar to those of maternal circulation;For this reason, they are used in the abortion approach (unfinished cardiac jam or fetal myocarditis). As the placenta has the aptitude of sticking prednisolone in inactive ponzoña it is used in the creek approach;It is recommended to use the lowest effective dose to overcome harmful impacts such as premature birth due to premature binzas, delay in fetal impulse, hypertension or gravity diabetes. The superior efforts with glucocorticoids during conception can protest stress doses, usually during a prolonged birth duty or in episode of a caesarean section.35-37 The antimalaric enjoyment during the cut is litigated;Curious opportunity have described innate defects such as cleft, obfuscation, ataxia, vestibular insensitivity and deafness associated with the chloroquine and spring service.
However, profuse rheumatology specialists use, mainly, hydroxychloroquine for thinking that fetal blow spill is much last with the specific one that with the exacerbation of the disease.35,37 Evolution and feeling of the imperturbable with the systemic lupus erythematosus is an apology gazette that heals with irritations and condonations. There are danger numbers that increase the frequency of reactivation of the excuse such as paving thesis, infections, surgical interventions, stress locations, pregnancy and the taking of lingual contraceptions, etc;It is not understandable to successions if it is a renewal of slaughter, a mycosis, a complication of the approach or an intercurrent condition. The most persevering death soras are infections, the cerebrovascular ephemeris, nephropathy and neurological cardinals; 32,38 but, in recent years it has been captured by prodigally the survival of these constants due, in large part, to the convenience ofnews diagnostic roads, the habit of therapies news and the germination of rheumatology specialists with a high scientific rage. MOST AJADOS IN THE TREATMENT OF LES The use of glucocorticoids, antipalúdico and conventional immunosuppressants (azathioprine, methotrexate, cyclosporine, cyclophosphamide and leflunomide) constitute the base of the treatment of systemic lupus erythematosus. Mopheyl mycophenolate inhibits, specifically, inosine dehydrogenase-5-monophosphate, and blocks the proliferation of lymphocytes and the response of antibody antibodies assistants of T cells;Two or three grams are used daily. Other exams indicate the aptitude of the tacrolimus and the abatacept in the loup nephritis approach. There are currently different physiological agents in clinical development focused on the resource of interaction between T and B cells;However, there are many molecules in season, currently in practice health center, only two can be crumbled: the Belimumab and the Rituximab. The Belimumab is a Cabg2 Cabg2 monoclonal antibody directed in front of the BAFF/Blas, it has been the first agent specifically approved for the treatment of the LES in the last 50 years. The mention for which it has been robbed is that of the active (moderate or austere step) that is not controlled with standard treatment, the clinical circles in which it seems more effective are the imperturbable assets with joint and mucocutaneous hatching. At present, nothing can be observed about its advantage in nephritis and severe beatings of the elementary upset system. The rituximab offers the opposite jeta of Belimumab. The treaties aimed at achieving their certification in the treatment of them were failed, among other things, by methodological paper;Although, there are nourishing veteran of impassive chain with various prudent signals opposed to standard treatment (nephritis, neurological hatchings, cytopenias) that responded to the point of view with rituximab. Other targets prescriptions are now in granazón: complement multipliers, immunoglobulin receptors or certain peptide scenes derived from autoantibodies used for the specific vaccine project.
Conclusions
Systemic lupus erythematosus is a multifactorial condition in which genetics, environment and carnal hormones play a preponderant role;That is why its phrase differs from one tenacious to another and the clinical communications of the excuse are asaz variables. Its incidence has increased in recent years throughout the planet;Although, the communications ranks more competent diagnostic rules and the acquisition of new more accurate treatments have authorized to extend the survival of the impassive impassives
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