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What is lupus? > Health and Wellness.

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Systemic lupus erythematosus is a chronic inflammatory autoimmune origin (although the exact cause is still unknown). This means that affected the immune system loses the ability to identify bacteria, viruses, and other external enemies that come into the Agency, and confuses the cells and healthy tissues as invaders, activating a production exaggerated antibodies which act on the patient's own cells (autoantibodies), by which any organ of the human body may be damaged.
Is about a disease that is estimated to suffer 40 of every 100,000 people - only in Spain-40,000 people suffer from it, and that it most often affects women, in a way that in 90% of cases it is women of reproductive age, although they may also suffer from it boys, old men and children. Women of color suffer it three times more than the white race.

Causes and risk factors of lupus

The causes of lupus and the mechanisms by which occurs are unknown, but know that there are a number of factors that, acting on someone genetically predisposed, may develop immune alteration and the symptoms of lupus. Among predisposing factors include elevated levels of estrogens (this explains the high frequency in women), ultraviolet radiation, medications, infectious agents, etc.
The name of the disease, 'lupus', is a doctor who in the 13th century popularized this name, since lesions of the face appearing on the affected reminded him to bites from Wolf ('lupus' in latin).

Symptoms of lupus

The lupus clinical manifestations are varied, and can appear all kinds of symptoms due to the alteration of various organs. The majority of patients have periods of exacerbation of the disease, which alternate with periods of remission, and it's rare that the disease completely.
Both at the beginning and during the clinical course, lupus can be accompanied by General manifestations in the form of fatigue, fever, weight loss, loss of appetite, and malaise. Let's see in more detail the most common symptoms of lupus :

Musculoskeletal manifestations

They are the most common lupus symptoms. Joint pains are practically in all patients, as well as the nonspecific muscle aches. The emergence of arthritis (inflammation of joints, which tends to be changing location) is also very common. We can see weakness, muscle inflammation, increase in the elasticity of tendons, as well as dislocations, deformities and joint.

Cutaneous manifestations

They are the next in frequency after the muscle-skeletal, in 80% of patients the disease sometime in. Currently the involvement of the skin is divided into three different forms of presentation:
  • Acute injuries: the most characteristic manifestation is a redness of the cheeks and bridge of the nose, giving rise to an appearance in wings of butterfly. These types of injuries occur in 50% of patients with lupus and usually occur after exposure to the Sun and coinciding with outbreaks of the disease. When injuries to forwards they rarely scarring.
  • Subacute injuries: are reddish lesions, high, annular and scales, which are distributed by areas exposed to the Sun such as neck, neckline, backs of the arms and shoulders. They appear in 10% of patients with lupus and heal without scarring afterwards.
  • Chronic lesions: also called discoid lupus; It appears in 20% of patients with lupus. They are circular lesions with reddish edge, and raised that are located on face, scalp, back of the hands and ears. When they heal they leave a permanent scar, and whether they affect the scalp develop alopecia in the area of the lesion.

Renal manifestations

The disturbance at kidney level causing lupus is nephritis. It appears in 50% of patients and is usually the most serious of all, manifestation since it is that determines the prognosis of these patients. It is usually asymptomatic, so it should be general urine in patients with lupus tests to diagnose it as soon as it appears.

Neurologic manifestations

Occur in 50-60% of cases, and they can be very varied. The most frequent are cognitive disorders, especially memory and reasoning problems. There may also be headache, convulsions, and even psychosis.

Cardiopulmonary manifestations

The most frequent pulmonary manifestation is the Pleurisy or inflammation of the pleura (lining around the lungs), and can produce pleural effusion. Similarly, the most common cardiac manifestation is pericarditis, or inflammation of the pericardium (the tissue that surrounds the heart), although there may also be abnormalities in the heart valves, heart failure, etc.

Hematologic manifestations

The most frequent manifestation of this group is anemia, which occurs in 70% of patients. There may also be a decrease in lymphocytes and platelets, but it tends to be mild and does not just impact.
At the level of clotting, due to certain antibodies, are frequent thrombotic events (formation of thrombi that can clog blood vessels, producing vascular alteration).

Other manifestations

Gastrointestinal manifestations unspecified such as nausea, vomiting, diarrhea, or even acute pancreatitis or ascites (presence of fluid in the abdominal cavity, which produces an increase in the diameter of the abdomen) can have. Also manifestations such as conjunctivitis and, in some cases, there may be increasing the size of the spleen, lymph nodes, increased in size in a generalized way, alterations in the function of the thyroid, etc.

Diagnosis of lupus

The diagnosis of lupus is based on the presence of clinical manifestations listed above. Some laboratory data can help in the diagnosis, such as anemia or the presence of autoantibodies. There are many types, and they may also be present in healthy people, so the presence of these autoantibodies have to accompanied by the clinic to reach the diagnosis.
There is a list with 11 specific clinical manifestations that help the diagnosis of lupus. The presence of four of these 11 demonstrations is needed in order to establish the diagnosis.
Systemic lupus erythematosus diagnostic criteria are as follows:
1. malar Erythema: is the redness of the cheeks and the bridge of the nose in "butterfly wings".
2. eruption discoid: are circular lesions with reddish edge, and raised that are located on face, scalp, back of the hands and ears.
3. photosensitivity: skin rash caused by an overreaction to the sunlight.
4. oral ulcers: that are usually painless.
5. nonerosive arthritis, i.e., inflammation of the joints but that does not erode the bone. The erosion of the bones of the joints occurs in other diseases such as rheumatoid arthritis.
6. Serositis: is an inflammation of the membranes lining the lung (Pleurisy) or the heart (pericarditis).
7. renal disorders: with excretion of protein in the urine or substances that indicate kidney damage.
8. neurologic disorder: such as seizures or psychosis.
9. hematological disorder: with anemia, leukopenia (low white blood cells) or thrombocytopenia (decrease in platelets).
10. immune disorder: with the production of certain autoantibodies for example called anti-DNA, anti SM, or antiphospholipid antibodies.
11. antinuclear antibodies: which is the antibody that appears most frequently in systemic lupus erythematosus, in such a way that if they do not appear it is very rare that the symptoms are for a lupus. However, they are antibodies specific for lupus, so it can also appear in other diseases and even in healthy people.

Treatment of lupus

There are currently no drugs with ability to cure lupus, so that the treatment will consist of reducing acute crises and their symptoms.
Treatment must be individualized for each patient, depending on the type and severity of the clinical manifestations; Therefore, before beginning treatment, should make an assessment of the affected organs and disease activity.
Generally it should be recommended avoiding exposure to ultraviolet radiation, as well as drugs and situations that may precipitate a new outbreak, such as oral contraceptives, infections, surgery, etc.

Mild manifestations

In the treatment of the minor such as arthritis, headaches, joint and muscle, fever or fatigue, nonsteroidal anti-inflammatory non-drugs (NSAIDs) are useful. If these are not effective they used corticosteroids in low doses. Hydroxychloroquine, which is also useful for fatigue and arthritis is indicated for Cutaneous manifestations. The topical corticosteroids (applied in the form of ointment) are useful in skin lesions.

Serious manifestations

In the case of neurological, renal disorders, anemia and other serious manifestations, is necessary the use of corticoids at high doses. On many occasions, it is necessary to add to the corticosteroid immunosuppressants such as cyclophosphamide and Cyclosporine.

What's new in the treatment of lupus

In recent years new therapies designed to treat autoimmune diseases have been developed successfully. They are treatment aimed specifically against any part of the immune system and manage to inhibit its function, which leads to a relief of symptoms. They are called biological therapies or targeted therapies.
In the case of lupus, various medications that inhibit some functions of B lymphocytes, which are largely responsible for the symptoms of the disease white blood cells are being used successfully. Recently approved the use of belimumab in patients with a high degree of activity of lupus. Belimumab is an antibody which manages to suppress B cells that cause damage to the body. Encouraging results using rituximab, an antibody designed specifically to a protein on the surface of B cells, have also been observed and which manages to reduce the activity of the disease.
There are other treatments in research such as alentuzimab, epratuzimab and others. Biological therapies in general reserve to patients who have had poor response with the usual therapies or who have obtained only a partial response. They are generally well tolerated but, to suppress the immune system, we must monitor the possible occurrence of infections or tumors.

Prognosis of lupus

Lupus is a chronic disease of variable course where there are from forms that just change survival (less aggressive forms), to forms that develop quickly and endanger the patient's life. The most frequent is presented with an intermittent course, with exacerbations and remissions. It is sometimes difficult to determine whether the patient has a reactivation of the disease, sobreañadida disease, or a complication of treatment.
During the past 20 years the survival of lupus patients has been increased in a linear fashion, thanks to early diagnosis and treatments used, so survival is currently exceeding 75% to the ten years of the onset of symptoms.
The factors influencing the prognosis of the disease are kidney function, the presence of anemia and the involvement of the central nervous system. In such a way that causes frequent death are infections, kidney failure and neurological injury.

Special cases of lupus

Drug induced Lupus (seudolupus)

It's a very similar to lupus clinical picture but that is induced by drugs. The clinical manifestations are mostly of predominance of General disorders, joint pains, arthritis, but rarely produce renal or Neurologic involvement. Immune level are also very similar, since autoantibodies are also only found types vary slightly in relation to the of systemic lupus erythematosus.
There are many drugs capable of producing this type of lupus, being the most frequent isoniazid, chlorpromazine, D-penicillamine, quinidine, phenytoin, etc.
This picture is equally frequent in men than in women and as soon as the drug is removed.

Lupus and pregnancy

Fertility is normal in patients with lupus, but they are increased spontaneous abortions, prematurity and fetal deaths, especially in patients who carry a particular type of antibody (anticoagulant).
You should pregnancy occur when the disease is controlled and, if they require corticosteroids, dexamethasone and betamethasone, avoid long life and could pass to the fetus. However, if there is no severe renal involvement and the disease is controlled, the majority of the patients complete the pregnancy and give birth to normal children.

Neonatal Lupus

It affects a small percentage of the children born to mothers who have certain antibodies, which passed to the fetus and cause symptoms. Manifestations consist of the appearance of skin, lesions similar to subacute lesions, in areas exposed to the Sun from two months of life, heart (permanent cardiac conduction blockage) and Hematological disturbances.
These antibodies are kept for six months and then disappear, in such a way that the skin lesions also cease to exist.
Published for educational purposes
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