Systemic Rritematoso Lupus And Its Relationship With Rheumatic Conditions

Systemic Rritematoso Lupus and its relationship with rheumatic conditions

Introduction 

In the relationship to rheumatic conditions, colagenosis play an important role and, without suspicion, lupus is one of the most constant. The strains come (from the Latin wolf) systemic erythematosus (them) date back in the ancient times, the renown is related to the altered countenance of the imperturbable, which adopts a great similarity with the fault -spared wolf of a wolf, which exhibits in the pillory and the cheeks an erythema malar -shaped butterfly.

Strange is even the report that indicates that the word lupus does not come to Latin mouth, but from a Gallic simulation style (LOUP, literal carnival wolf) that the paronys used around the holes. This excuse was invested for the first succession in the Middle Ages by the chimera of its dermatological externalizations. The Lupus territory is attributed to the Galen Rogerius (12th century), which used it to nick.

The neoclassical signal was announced with the recognition made by Moric Kaposi, in 1872, of the systemic revelation of the dropper and the modern with the achievement, in 1948, of the cells of the erythematous lupus -le- (nevertheless the usufruct of these Cells such as exponents of opinion have been with a large part) and characterized by advances in the concept of pathological physiology and the clinical and laboratory characteristics of the ailment, as well as by the relief in the approach. The useful prescription for indisposition was avant -garde, for the first time, in 1894, when it was disclosed that quina was an effective therapy; Four years later the use of salicylates, in conjunction with the quina, proved equally more humanitarian.

This was the best habitable treatment for the imperturbable still unfinished from the twentieth century when Philip Showalter Hench discovered the competition of corticosteroids.

It is veneration that to the separate five million inhabitants throughout the universe have lupus and that more than 100,000 new incidents are diagnosed every year; Only in Spain around 20,000 any ones live with this apology. This condition is twice as usual than leukemia and ten more common opportunities than hemophilia. It was delayed to diagnose some symptoms of this disease because the glimpses are highly similar to those of various diseases.

The prevalence in the General Villa, at the branch of the Sector, is between four and 250 sets per 100,000 inhabitants: in North America, Asia and in northern Europe it affects 40 out of every 100,000 residents, with a dean incidence between The Hispanic and African -American village. About 90% of the events corresponds to the group of ladies of fruitful age (Affinity Women/Male: 9/1, other writers consider it 10/1). In the United States, the incidence and prevalence of systemic lupus erythematosus are longevos in subjects of black crib that of white quality, autonomously of love.

Other offices suggest that in Amerindian mestizos these selected occupy an intermediate side between the two sides. When analyzing the epidemiology of this excuse in the United States it is appreciated that it affects one in 4,000 people and that the parietas suffer from five to 15 successions more often than men.

Episer analysis evidenced in Spain a prevalence of nine facts per 100,000 residents. Another office made in Spain, for which a demonstration of 154 impassives was taken, showed that 54% had a pathological history ascending systemic lupus erythematosus or some collagen condition; By finding out the case and chewing the first nuance kinship guard with self-infammed apologies, the danger of suffering increased proportionally.

When studying in order to save a male family member with systemic lupus erythematosus did not increase the setback of autoimmune evils among the parents, despite if the persevering is courtesan the event for a family member is 95%, autonomously of love of love. Among monochigotic twins, a conformity of trances of systemic lupus erythematosus has been found from 24% to 58% of doubles, meanwhile that among diced twins the approval is 2.6%.

In Mexico, reports on its prevalence cite amounts of 88 percent. In Cuba, there have been pesques of morbidity and mortality that demonstrate that this apology is more persevering in effeminate love and that it is healed from the most benign stews even the most serious; Fords have been described by ins and outs even 10% of the imperturbable. In the province of Villa Clara, polyclinic and epidemiological in imperturbable with systemic lupus erythematosus that have taught the mood of this indisposition in the different searched population orders, which has approved a clearer and clearer chimera of the anecdotes of the transformation of constants.

Currently, there is a investigation with clinical, epidemiological, immune and hereditary feature of all the tenacious to gestate intervention activities in order to ascend their semblance law and recreate their survival. Systemic lupus erythematosus is an autoimmune ailment of chronic space, of ignorated sanity and has large clinical and multisystem externalizations due to the manufacture of autoantibodies against autoantigens and the squad of different immunocomplexes that mediate inflammatory responses when deposited in multiple organs and cloths, including Thyroid, sentimentality, bofes, heat, killing, unions and peel, etc.

In the inquiries made in the province of Villa Clara, it has been scrutinized that, in the community of the tenacious, the excuse usually undertakes slowly, of facet that the conflicts appear gradually during an equanimous time of time; In circumstances spend months or years to honor the apology. In some constants the beginning is quite torpid, with ins and outs from the dawn, which can go to the death. A Diagnosed Succession The condition is with menstruits of references and exacerbations. The etiology of them is unknown; But, it is known that there is a variable elaboration of autoantibodies that, adhered to hereditary and environmental executors can be involved in their pathogenesis.

It is plausible that brand new pathogenic and etiological agents are involved, hence the polyclinic and laboratory diversity presented by the tenacious with them. The most invoked pathogenic authors are autoantibodies, B and T cells, hereditary, hormonal and environmental executors and apoptosis. Obtaining autoantibodies is a characteristic attribute of the constants with them.

These antibodies can be a key work in pathogenesis, a consequence of tissue trauma or acceptably the representation of a Casto etiological agent; However, not all antibodies are pathogenic, those that have adaptation to carry tissue laceration are from the quidam IgG and with reinstatement sympathy for deoxyribonucleic acid (DNA, for their monograms in British) bycatenary, which is a summary on the other hand drug dependent of The interaction between B cells and CD4 assistants+.

In the impassive ones with the influx of specific T cells for a autoantigen with operation to conceptualize the manufacture of autoantibodies. There is a veteran prevalence of the ailment in progenies of imperturbable that in the common village and a maximum incidence of common alleles that in a phalange care. An assembly of the indisposition with straight leukocyte antigens (HLA, for its monograms in English): HLA-B8, HLA-DR3 and HLA-DR2.

A low incidence of the appearance of the once of the menarche or then of menopause. In imperturbable with them a soluble way of the modified FAS protein has been described in which the transmembrane sphere is missing.

The participation of external agents as inducing agents could be important; The ultraviolet guide and the microorganism play a cardinal role, especially the rotavirus, as well as the habit of certain drugs such as hydralacin, procoinamide, isoniacid, methyldop. 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, disembarked or on high on the malar notability, tending to distribute over the nasolabial folds. two. Lupus Discoid: Erythematous patches on important with adherent and follicular keratosic scamming, atrophic snacks that can happen in ancient wounds.

Hierarchy criteria of systemic lupus erythematosus. 

Systemic Lupus International Collaboating Clinics (SLICC), 2012 Clinical criteria: 

• Acute epidermal lupus (lupus malar sarptull, bullous lupus, toxic epidermal, maculopapular rash, photosensitivity), in disappearance of dermatomyositis or subaguous epidermal lupus.
• Chronic epidermal lupus (discoid rash, overwhelmed hypertrophic lupus, lupus paniculitis or Lupus Deep, mocose lupus, lupus tumidus, loup perniosis, overlap lupus discoid/lichen flat).

A tenacious of them is classified if it presents, at least, four of the clinical or immunological criteria; At the except one clinical and another immune must be present. The imperturbable with renal biopsy compatible with lipple nephritis associated with ana or positive anti-dna. The beginning of the apology is variable, in some events it is very epidermal and only constitutional signs are presented; However, in others it can be largely torpid and serious and commit the story of the tenacious.

Lasity, anorexia and percentage dismissal are regular threats in the original clinical oil of the SLE; The tenth can appear both at the start and in the period of the dropper. The universality of the impassives have spectacular luxury luxury within the period of ailment, especially arthralgia, morning erection, non -erosive arthritis that affects large and few joints, myalgias and inflammatory myopathy.

Anemia is normal asaz in these patients, it is serious and it becomes hemolytic. They can devise thrombopenia, leukopenia and lymphopenia, which are generally related to the action of indisposition. It must also be possessed in telltors that some of the specific ones used in them can generate cytopenias. Cutaneous and viscous evils appear in 80% of the tenacious: photosensitivity, mouth pustulas, malar rash and discoid offenses. Subcutaneous abscesses and non -scar -cycatric alopecia are other common appearances; Likewise, leukocytoclastic vasculitis and periungual infarcts, etc.

Cardiovascular presumption occurs in the collective of the tenacious, especially pericarditis, but myocarditis can still be born, which is a manic externalization and occurs in circumstance of driving disorders, cardiomegaly and heart failure. Transesophageal echocardiography is the most exciteable evidence to delve endocardial saint, which has its most academic way in the so-called liberty-sacks endocarditis, which produces fibroinflamatory warts.

The most pedantic spits are mitral and aortic and predominates, from the clinical point of view, valvular insufficiency on stenosis. Cardioembolic phenomena can be rented that predispose valvular eninfection. Some imperturbable can introduce coronary excuse and acute myocardial infarction. Other altercations that can be appeared are pulmonary revelations, which can attend from suffocation, the intersposition of interstitial pulmonary gacetilla, acute lupus pneumonitis and pleural spills including pulmonary hemorrhage.

In it, intestinal requisitors can occur that are prestigious with vasculitis, pancreatitis or spontaneous peritonitis. Other unusual samples are primary biliary cirrhosis, autoimmune cholangitis and thrombotic occlusive vasculopathy with Budd-Chiari syndrome. Neuropsychiatric ostentations can also occur, which are highly variable, including disturbances and paranoia, cognitive inconveniences and confusional countries, migraine, strokes, transverse myelitis, Guillain Barré syndrome, Sydenham Korea, polyineuritis Motor sensitive, multineuritis and devotery of cranial doubles, etc.

Renal bruise is characterized by proteinuria, hematuria, leukocyturia, kidney rollers, renal class and arterial hypertension. It is usually accompanied by positivization of anti-DNA and sunset antibodies on the steps of complement fractions. Many imperturbable are with increases in the degrees of cholesterol and triglycerides. Life nephritis can be silent and all the successions correspond to mesangial glomerulonephritis. Other Guisas of renal redundancy are secondary, abnormal amyloidosis in LES, and renal mine thrombosis.

In the province of Villa Clara, thickening has been spied on the incidence of lupus nephritis as a première of a les. These persevering have a more torpid transformation than those who debut with other clinical samples, so it could be a symptom of bad auspices. The renal biopsy allows detailing the type of laceration and the most appropriate point of view and must be performed behind the renal bruising scale.

Laboratory poll. The most used are: hemoglobin, sedimentation acceleration, platelets, leukogram with differential, lupus anticoagulant, C3, C4, immunoglobulins, serology, ana, anti-dna, anti-SM, daring of antiphospholipid antibodies, Addis count, proteinuria of 24 24 hours, echocardiogram, bark bort by immunofluorescence and renal biopsy, among others. Special situations – Lupus in pediatric age: from the town of children with rheumatic excuses 2% suffers systemic lupus erythematosus.

There are supporting epidemiological documents. The incidence ranges between 0.4 and 9.0 per 100,000 children. It presents more in the black, oriental and Hindu class than in Caucasic. In four-year-old Benjamines, female-masculine litany is 2 to 1, increases in adolescence even 3 to 1 or 5.5 to 1. The condition can begin with acute or insidious fashion; Even the signs precede the opinion of the apology in years. The most constant signs in children are tenth, coincidental discomfort, arthralgia or arthritis and malar rash; However, any of the clinical sagacities clinical hatchings may be flagrant. In some mishaps the beginning is artificial, with debt for force. In Villa Clara, mishaps have been announced that have inaugurated with cerebral infarction, lupus nephritis, Korea, etc. The truth is that the impassives in which the beginning is effective have a more torpid and gloomy transformation.

Survival multipliers reach, in various grandullones states, more than 90% at five years and even 88% at 10 years; In Cuba, the exams reveal, according to the curvatures of Kaplan and Meier, a 95% survival at five years and 88% at 10 and 15 years of entourage. Despite these animating figures, the case of maximum morbidity due to the treated treatments persists.

The heterogeneity of Lupus in pediatric forguration is not only rigged to the difficulties associated with the bolderies prescriptions but also to the characteristics hospitals of the excuse since there is no characteristic oil in the advent either during the course of its transformation; The multisystemic nature of the affection is expressed as a spot.

The persevering with Lupus must commit strict corners with care of acute appearance, since an active them should be affected with immediacy to minimize the living blow. The recognition of these tenacies must be multidisciplinary, with the rectory of specialists in rheumatology and pediatrics and those of nephrology, cardiology, neurology, psychology and hematology, among others.

Therapy with glucocorticoids and immunosuppressive drugs is necessary in the treatment of all children and adolescents with them. The final feeling of the erythematous lupus that begins in child.  

Lupus and pregnancy

The effect of pregnancy has been inquired and controversial, especially with ligation to the incidence of the acuteness of the ailment during procreation. The explorations have stated that the dedication of lupus can perfectly increase, devalue or subtract unchanged during pregnancy. One of the greatest dangers of soras with them is the intelligence of activation of ailment during pregnancy; However, not all floors agree in this seasoning.

The activation case seems to undergo the level of the diligence of the dropper in the ravine in the six to twelve months of the generation. Glomerulonephritis is the significant setback of LES during pregnancy. This is much maximum if lupus nephritis is active at the moment of reproduction or if pregnancy occurs very close, in period, of the reference resource. It must be taken into account that pre-eclampsia also leads to the stagnant of proteinuria and becomes more difficult aesthetics between this and renal sharpening.

The activations of them can occur in any stretch of the transformation of tiredness, they can also occur varied months later; The universality of the exasperations of the gotera during pregnancy are not usually severe. The trace of anti-Rro/SS-A and anti-the/SSB antibodies is associated with fetal defect and is responsible for the neonatal lupus and a fattening in the acuity of monsters, as well as the antiphospholipid antibody antibody audience Fetal degenerations, preterm illuminations and delay in intrauterine development. There are useful laboratory parameters to permit.

In the active the latter they usually get off, although it does not always happen in this way. Serum complement values ​​may be frequent or increased during pregnancy in section of other latent growth. In pregnancy the treatment must be monitored to minimize the blows to failure and the stepmother; The habit of potentially teratogens must be failed. Glucocorticoids are favorable to focus on the active systemic lupus erythematosus. Fluorated agents (dexamethasone and betamethasone) reach fetal circulation and achieve unions similar to those of maternal circulation; By this jurisprudence, they are used in the treatment of engendro (unfinished cardiac jam or fetal myocarditis).

As the placenta has the efficiency of holding prednisolone in inactive narcotic is used in the point of view of the trough; It is recommended to use the lowest effective dose to dodge unfavorable impacts such as premature delivery due to premature films, delay in fetal process, hypertension or diabetes of the preparation.

The entire sisters with glucocorticoids during pregnancy can fall in love with stress doses, usually during a prolonged birth suffering or in a caesarean event. The use of antimalaric during pregnancy is disputed; Eccentric occasion have been described acts of pleasant defects such as cleft, drunkenness, ataxia, vestibular atrophy and deafness associated with the usufruct of chloroquine and spring. However, rumatology specialists use, mainly, hydroxycloroquine to reflect that chance of fetal detriment is much last with the specific one that with the exasperation of ailment. Evolution and sponsorship of the imperturbable with the systemic lupus erythematosus is an indisposition news that currencies with exasperations and emissions.

There are danger executors that increase the frequency of reactivation of the excuse such as the thesis, infections, surgical interventions, stress locations, trouble and the taking of verbal contraceptives, etc; Opportunities is not practicable to warn if it is a renewal of courage, a mycosis, a confusion of the point of view or an intercurrent ailment.

The most common scythe complaints are infections, cerebrovascular trances, nephropathy and neurological wounds; However, in recent years, the survival of these imperturbable has greatly managed due, in large part, to the exploitation of diagnostic roads, the use of therapies information and the row of specialists in rheumatology with a high scientific degree.

More worn medications in the point of view of the habit of glucocorticoids, antipalúdico and conventional immunosuppressants (azathioprine, methotrexate, cyclosporine, cyclophosphamide and leflunomide) constitute the base of the systemic erythematous lupus approach. 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 diplomas indicate the capacity of tacrolimus and abatacept at the point of view of lupus nephritis. There are currently different anatomical agents in clinical development aimed at the growth of interaction between T and B cells; However, there are many flowering molecules, currently in practice medical center, only two can be calculated: 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 admonition for which it has been allowed is that of the active (moderate or rigorous extreme) that is not controlled with habitual treatment, the clinical decorations in which the constant active constants with joint and mucocutaneous exhibitions are most effective.

At present, nothing can be cracked about your convenience in nephritis and serious vehemence of the primary anxious procedure. The rituximab offers the rebel expression of Belimumab.

The offices exposed to reaching their authorization in the treatment of them were failed, among other things, by methodological overwhelms; However, there are abundant practices of impassive successions with various difficult appearances contrary to standard treatment (nephritis, neurological appearances, cytopenias) that responded to rituximab. Other goals are nowadays in advance: the points of the complement, the immunoglobulin receptors or certain peptide scenes derived from the exploited autoantibodies for the specific vaccine sketches.

Systemic lupus erythematosus is a multifactorial ailment in which genetics, environment and sensual hormones play a preponderant role; That is why his phrase differs from an imperturbable to another and the appearances hospitals of the condition are highly variable. His incidence has reinforced in recent years worldwide; However, the most capable diagnostic norms information trade and the achievement of new more accurate treatments has tolerated to enlarge patient survival.

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