Lupus

Systemic lupus erythematosus (SLE or lupus) is an autoimmune disease of connective tissue that can affect any part of the body. As in other autoimmune diseases, the immune system attacks its own cells and tissues, resulting in inflammation and tissue damage.

SLE most often harms the heart, joints, skin, lungs, blood vessels, liver, kidneys and nervous system. The evolution of the disease is unpredictable, leading to attacks and remissions. The disease occurs nine times more often in women than in men, especially between the ages of 15 and 50 years, being more common in people who have European ancestry.

The LES is treatable mainly symptomatically with steroids and immunosuppressants. Currently there is still no cure. SLE can have fatal complications, however, currently the fatalities have become increasingly rare. The survival rate at five years for people with SLE in the United States, Canada and Europe is approximately 95%, 90% in 10 years and 78% in 20 years.


History

There are several versions about the origin of the name of the disease. (Lupus means wolf in Latin, and erythema (ερυθρός) means red in Greek) One version is that cases of porphyria (a disease with symptoms similar to lupus) generated folklore about vampires and werewolves due to the photosensitivity, scarring, growth hair and stains on teeth. Another theory relates the term loup, a French style of mask that women used to hide the rashes on their faces.

In 1895, the Canadian physician Sir William Osler best characterized the involvement of various tissues of the body and added the word "systemic" to the name.

Pathophysiology

There are periods of inactivity that can last for weeks, months or years. Some patients may not develop serious complications. Is not transferable and its manifestations vary greatly from one patient to another. There are simple cases (which require minimal medical interventions) and severe (damage to the vital organs such as lung, heart, kidney and brain).

Causes

It is believed that the pathogenesis involves a basic defect in maintaining self-tolerance with activation of B cells (B lymphocytes). This process can occur secondarily to some combination of hereditary defects in the regulation of T helper cells (Th-T helper). It is also believed that the primary defect occurs in CD4 T cells that drive B cell autoantigen-specific antibodies.

Genetics

* Disease Clinic: individuals whose relatives have been diagnosed are more likely to present the SLE or another autoimmune disease. The concordance rate in monozygotic twins ranges from 12% to 15%.
* Immunological Changes: Family members have a higher frequency of positive ANA, hypocomplementemia, hypergammaglobulinemia, serologic test for syphilis and false positive antilymphocyte antibodies.
* HLA: Lupus is associated with HLA-DR2 and HLA-DR3.
* Mutations are housed on the short arm of chromosome 6.

Types

There are three types of Lupus: Discoid lupus, systemic lupus and drug-induced lupus.

Discoid lupus

It is always limited to the skin. It is identified by rashes that appear on the face, neck and scalp. Approximately 10% of discoid lupus can evolve into systemic lupus, which can affect almost any organ or body systems.

Systemic lupus

Usually more severe than discoid lupus and can affect almost all organs and systems. In some people only lesions predominate in the skin and joints, in others there may be involvement of the kidneys, heart, lungs or blood.

Drug-induced lupus

The drug-induced lupus erythematosus occurs as a result of the use of certain drugs or medicines. The symptoms are very similar to systemic lupus. The very drugs for lupus can also lead to a state of induced lupus. So you must be absolutely sure of the diagnosis before starting treatment with corticosteroids, antimalarials and anti-inflammatory reuquinol as a whole.

Signs and symptoms

Fatigue is often the first sign. Most people present continuous or intermittent fever, weight loss and malaise. Regardless of the clinical manifestation, the vast majority of patients are critical periods interspersed with periods of relative improvement or even downtime.

Diagnosis
Laboratory Tests
Autoantibodies


It is perhaps the most consistent laboratory finding of the disease. Some autoantibodies such as anti-Sm and anti-DNA double helix (dsDNA) have diagnostic value because they are highly specific for SLE. The other antibodies are not specific, but its presence helps the diagnosis.
Autoantibodies in systemic lupus erythematosus Autoantibody Incidence (%) Specificity
80-90 HIGH ANTI-dsDNA
ANTI-ssDNA 80-90 -
ANTI-Sm 30 HIGH
ANTI-RNP 30-40 -
Anti-Ro/SS-A 30-40 -
Anti-La/SS-B 25 -
ANTI-P 10-15 HIGH

Complement

Fluctuations in levels of C3, C4 and CH50 may be useful in monitoring the disease activity.

Tests VHS and mucoproteins are nonspecific, but used to evaluate the inflammatory activity. The complete blood count can be found normocytic anemia, leukopenia (leukocytes rate below 4,000 / mm ³), lymphopenia (lymphocyte count less than 20% of total leukocytes) and thrombocytopenia (platelet rate below 100,000 / mm ³)

Protein electrophoresis

Important to evaluate elevation of gamma globulin, which most often is associated with disease activity. Also useful for assessing level of albumin and inflammatory activity.

Evaluation of different organs

The assessment of renal function (urine I, Urea, and Creatinine Clearance) must be made regardless of the presence of clinical manifestations. Other tests should be conducted in accordance with the involvement of each organ specifically.

Diagnostic Criteria

The diagnosis of SLE is made when four or more of the criteria listed below are present:

* Malar erythema (redness characteristic in the nose and face), usually in the form of "butterfly"
* Discoid skin lesions (discoid rash)
* Photosensitivity
* Oral ulcers and / or nasopharyngeal, observed by physician
* Non-erosive arthritis of two or more peripheral joints, with pain, swelling or effusion
* Changes hematologic (hemolytic anemia or leukopenia, lymphocytopenia or thrombocytopenia) in the absence of a drug that can produce similar findings
* Abnormalities immune (antibody anti-DNA double helix, antiSm, antiphospholipid and / or false-positive serologic test for syphilis)
* Antinuclear factor (ANF) was positive
* Serositis (pleuritis or pericarditis)
* Changes Neurological: seizures or psychosis without other apparent cause
* Abnormalities in tests of renal function: proteinuria (removal of proteins in the urine) greater than 0.5 g per day or the presence of cellular cylinders on microscopic examination of urine

This diagnostic method has a specificity of 95% and a sensitivity of 75%, so that when at least four criteria are met, on average 95 of every 100 patients will, in fact, systemic lupus erythematosus. However, only 75 out of 100 patients with SLE were positive for four or more of 11 criteria.

Treatment


Prevent infection. General symptoms most often respond to treatment of other clinical manifestations. The isolated fever can be treated with aspirin or nonsteroidal antiinflammatory drugs. Steroids and immunosuppressive agents (especially cyclophosphamide) are indicated in severe cases.

Since there is a very accurate diagnosis for diseases called autoimmune diseases, should be careful when administering medications, since their effects can be very aggressive in the body.

Pregnant women with lupus require strict medical supervision throughout the pregnancy, since the disease can also reach the fetus.

Epidemiology

* Sex: There is a clear prevalence in females (8 females in 10 patients), usually appearing during the reproductive years (menarche to menopause)
* Age group: The first symptoms usually occur between the second and fourth decade of life.
* City: It is more common in the U.S., fewer incidents in England
* Ethnic Distribution: It is more common in blacks, with an incidence of 11.7 per 100,000 while the incidence in whites is 2.7 / 100,000.
* Overall: 27.5 per million for white women and 75.4 per million for black women in America.