Perhimpunan Reumatologi Indonesia
Indonesian Rheumatology Association

LUPUS, Thousand Faces Disease

By: Natsir Akil

Lupus is also known as systemic lupus erytematosus (SLE) is an autoimmune disease. Autoimmune describes a condition in which the immune system in the body is unable to distinguish between germs and foreign objects from outside the body with the body’s own cells or tissues, so the immune system attacks the body’s own cells and tissues. Hence the appearance of the disease & nbsp; very diverse and the symptoms and signs resemble many other diseases, this disease is also known as a thousand faces disease. This term describes that in people with lupus, symptoms that are not typical & nbsp; and vague, which causes difficulty in recognizing this lupus disease. & nbsp;

The word lupus was first used in 1200 BC, to describe an abnormality of the face called ulceration. The word lupus itself comes from the Latin which means wolf. The term comes from a patch on the skin that looks like a bite from a wolf. The reddish patch that is characteristic of lupus is called malar butterfly rash , which is a reddish patch that crosses over the nose and spreads to both cheeks which resembles a butterfly. & Nbsp; Apart from affecting the skin and mucous membranes, lupus also attacks the joints, kidneys, heart, lungs, blood vessels, and brain.

Lupus is common in women, especially women of reproductive age & nbsp; compared to men, and the most age is at 15-45 years, however in children and the elderly can also be found. Although 90% of people with lupus have a positive ANA ( anti-nuclear antibody ) laboratory examination, no single laboratory test can confirm that a person has lupus. Many sufferers experience the symptoms of lupus for several years, before they are truly diagnosed with lupus.

Immune and lupus system

Every day our bodies are exposed to various foreign objects, either in the form of germs or chemical substances that can damage the body. The body still does not experience any disturbance because in it there is a system called the immune system (immune system) which kills germs or neutralizes incoming foreign objects. If the body is exposed to a foreign object, there are two immune responses that will occur, the first is a non-specific immune response, which is innate immunity ( innate immunity ) which responds to foreign objects even though it has never been previously exposed to it. these foreign objects. The second immune response is the specific immune response, which is an acquired immune response ( acquired ) that arises against a foreign object, to which the body has been previously exposed to the foreign object.

The immune system functions to protect the body against any foreign objects that enter, especially germs that can cause infection. The work of the immune system depends on the ability of the immune system to recognize molecules or antigens contained in foreign bodies or germs and then generate the appropriate reactions to remove the source & nbsp; the antigen in question. The process of recognizing antigens is carried out by the main elements of the immune system, namely lymphocyte cells, which is then followed by an effector phase involving various types of cells. The recognition of antigens is very important in normal immune system function, because lymphocytes must recognize all antigens contained in foreign bodies, which can potentially cause harm to the body, and at the same time they must ignore the body’s own tissue molecules.

This condition is called tolerance, where the immune system will only react to foreign objects that will damage the body, while with the body itself will not react. In this regard, the lymphocytes in an individual diversify during their development in such a way that the lymphocyte population as a whole is able to recognize all foreign objects / molecules and distinguish them from tissue molecules or the body’s own cells.

Under normal circumstances the immune system produces a protein called an antibody (immune protein). These antibodies function to defend our bodies against foreign objects attack by neutralizing foreign objects that enter our bodies. When the immune system is unable to recognize itself, a condition called autoimmune occurs, in which the immune system recognizes itself as a foreign object, so that the immune system will attack and damage the body’s own cells and tissues.

This condition occurs in people with lupus, where the immune system in lupus sufferers is unable to recognize themselves so that they attack and damage their own organs or tissues. In addition, the cells of the immune system also produce proteins called autoantibodies, which also damage the body’s own tissues and cells.

Causes of lupus and trigger factors

Lupus is a complex disease and it is not yet known the exact cause why a person has lupus. However, a combination of various factors including environmental, hormonal, immune system abnormalities, and genetic factors are thought to be the cause of lupus. Environmental factors include sun exposure, smoking, stress, certain drugs, and viral infections. Genetic factors play an important role in causing lupus. However, not everyone who has a genetic predisposition will suffer from lupus. Only about 10% of people with lupus have parents or twins who also have lupus. It is very important to pay attention to factors that can trigger or aggravate lupus symptoms. Some of the factors include: sun exposure, hard work and lack of rest, experiencing stress, suffering from infections, trauma, stopping lupus medications, and the use of certain drugs.

Signs and symptoms of lupus

Symptoms and signs of lupus differ from one patient to another. In fact, it is said that no two people have the same symptoms and signs of lupus. The appearance of lupus can also resemble many other diseases, so that lupus is also known as a thousand faces disease. Some sufferers have few symptoms, while others present with many. Symptoms can come and go. When the symptoms appear or get worse ( flare ) the patient feels sick, and when the symptoms disappear (remission) the patient feels healthy.

Although lupus can affect all organs in the body, in most cases, lupus affects only a few parts of the body. For example, a person may only experience swelling of the knees and fever. In other sufferers only feel tired all day long or only disturbances in the kidneys. & Nbsp; In other cases, there may only be red patches on the skin. & Nbsp; Over time, more symptoms may appear. & Nbsp; The course of lupus is slow, with intermittent symptoms. In women with lupus, the symptoms and diagnosis are generally made between the ages of 15-45 years. However, lupus can also be found in children and the elderly.

In some people, the manifestations of lupus are mild, but in others, lupus can present with severe and life-threatening symptoms. & nbsp; Symptoms commonly found in lupus sufferers are pain and stiffness in the joints without swelling, pain and weakness in muscles, fever of unknown cause, feeling very tired, reddish spots on the face that resemble a butterfly or reddish patches on the skin in place. other, weight loss, low red blood cells, trouble thinking / remembering or confusion, kidney disorders, chest pain when taking a deep breath, reddish spots on the skin when exposed to sunlight, hair loss, fingertips or toes pale or purplish when exposed to cold. Meanwhile, rare symptoms are blood clotting disorders, convulsions, painless mouth or nose ulcers, headaches, paralysis of the limbs (stroke), dry eyes and mental disorders in the form of feeling sad.

Lupus Precautions

The Indonesian Rheumatology Association ( Indonesian Rheumatism Association – IRA) makes a guideline in being aware of the possibility of someone suffering from lupus. There are 11 criteria, if 2 or more of the criteria listed below are found, then we need to be aware that someone has lupus. Criteria for lupus precautions include:

1. Young women with abnormalities in 2 or more organs

2. There are common symptoms such as fatigue, fever without evidence of infection, and unexplained weight loss.

3. There are abnormalities in muscle and bone organs such as arthritis (arthritis), joint pain (atralgia), inflammation of the muscles (myositis)

4. Abnormalities in the skin and mucous membranes in the form of reddish spots on the face that resemble a butterfly, the skin turns red when exposed to the sun (photosensitivity), lesions on the mucous membrane of the mouth (thrush), hair loss (baldness), the tips of the fingers and toes becomes pale when exposed to the cold.

5. Kidney disorders include red urine, protein in the urine (proteinuria), swelling throughout the body due to kidney problems (nephrotic syndrome)

6. Disturbance of the digestive system with symptoms of nausea, vomiting and abdominal pain

7. Pulmonary disorders in the form of lesions in the lung tissue, increased pulmonary blood vessel pressure (pulmonary hypertension)

8. Inflammation of the heart muscle (myocarditis) and the lining of the heart (pericarditis / endocarditis)

9. Enlargement of the spleen (splenomegaly), liver (hepatomegaly), and lymph tissue (lymphadenopathy)

10. Lack of red blood cells (anemia), white blood cells (leukopenia), and blood clotting cells (thrombocytopenia)

11. Psychiatric disorders (psychosis) and disorders of the nerves such as convulsions

Lupus management

Until now, there is no medicine that can cure lupus. The goals of lupus management are to prevent flares , to overcome the symptoms that appear, and most importantly to prevent organ damage. Lupus management includes education and counseling, rehabilitation programs, and administration of drugs.

Medicines that can be used in people with lupus include:

1. Non-steroidal anti-inflammatory drugs (NSAIDs), these drugs can be used to treat pain and swelling in joints and muscles. Usually only used in mild lupus and vital organs are not affected. It needs to be careful in its use because it can cause stomach disorders, headaches, fluid buildup in the body, disorders of the liver, blood and kidneys. This drug is also avoided for use in pregnant women after the first three months of pregnancy. Likewise, breastfeeding women need to be careful.

2. Corticosteroids or steroids, these drugs are used to treat swelling and pain in various organs of the body. In large doses, this drug can suppress the work of the immune system. Symptoms of lupus respond quickly to the improvement of this drug. Once the symptoms improve, the dose of this drug needs to be reduced slowly to the smallest dose that can still control disease activity. In addition to its strong effect in overcoming lupus symptoms, this drug also has many side effects that must be taken into consideration in its use. Short-term side effects include facial swelling ( moon face ), acne breakouts, heartburn, increased appetite, weight gain, and mood swings. These side effects usually disappear after the drug is stopped. Long-term side effects include easy bruising, thinning skin and hair, bone loss, increased blood pressure, increased blood sugar, weakness in muscles, infections, and cataracts. Some sufferers may suffer from injury, depression, or heart failure. Corticosteroids can be used during pregnancy.

3. Anti-malarial drugs, these drugs are used for the prevention and treatment of malaria, but also have a good effect in overcoming the symptoms of lupus. The effectiveness of this drug is seen both in lupus with skin and musculoskeletal involvement, as well as good for treating symptoms of fatigue and inflammation in the lungs. There are two drugs that are often used, namely chloroquine and hydroxychloroquine. The main side effect of using this drug is visual disturbances. Before using anti-malarial drugs, patients are advised to have their eyes checked by an ophthalmologist.

4. Immunosuppressive drugs, these drugs aim to suppress the immune system in people with lupus, especially when used in severe lupus. The drugs include azathioprine , cyclophosphamide , mycofenolate mofetil , and methotrexate . Side effects that can occur with the use of this drug include nausea, vomiting, hair loss, bladder disorders, decreased fertility, cancer, and infections.

& nbsp; lupus and pregnancy

Since most people with lupus are women and the diagnosis is generally made at reproductive age, the issue of pregnancy and lupus becomes important. Some things that need to be known regarding lupus and pregnancy are the fertility rate of women with lupus (fertility), & nbsp; the right time to get pregnant, the risk of flare during pregnancy, the likelihood of a baby being born safely, the safety of using drugs used to control lupus during pregnancy and breastfeeding, and the use of contraception. Lupus does not usually reduce fertility in women with lupus, however it does increase the risk of pregnancy and complications.

Some of the factors that may affect the fertility of a person with lupus are irregular menstrual cycles that can occur during the active phase of lupus and the use of high doses of steroid drugs. Kidney disorders that occur due to lupus can cause amenorrhea (no menstruation). Cyclophosphamide drug administration can also cause interference with the ovaries (ovaries). One third of people with lupus experience improvement in symptoms during pregnancy, another third get worse, and the remaining third have no change in symptoms. There is no test that can predict who will experience worsening of symptoms during pregnancy, but people with lupus who have been in remission for more than six months have a low risk of recurrence ( flare ) and the baby can be born with normal.

A number of studies have found that being pregnant while lupus is active increases the risk of flares during pregnancy. & nbsp; Likewise, the risk of preeclampsia increases in people with lupus. The risk of kidney failure also increases in pregnant lupus patients with kidney disorders (lupus nephritis) which can lead to death. & Nbsp; Pregnant women with lupus, especially those receiving corticosteroids are at risk for increased blood pressure, diabetes (diabetes), and kidney complications. For people with lupus who don’t want to get pregnant, it is advisable to use contraception. The use of contraception in people with lupus is based on the patient’s condition and is given individually.

Oral contraceptives are an option for patients with stable disease conditions, without an antiphospholipid syndrome (APS) disorder. There are concerns about the use of oral contraceptives, because in the contraceptive pill there is the hormone estrogen which can trigger a recurrence of lupus, but several studies have shown that this opinion is weak. Meanwhile, the use of a spiral (IUD) is not recommended for lupus sufferers who are receiving steroids or drugs that suppress the immune system, because of the risk of infection, so the choice is condoms. Consultation with experts is very important to determine contraception that suits the patient’s condition. & Nbsp; & nbsp; & nbsp;

Diet management in lupus sufferers

Currently there are no definite standards regarding the diet that people with lupus must follow. Diet in lupus sufferers may need adjustments depending on symptoms, treatment given, and other things. Lupus sufferers with high blood fats, need to consume low-fat foods. A low-fat diet is also useful for avoiding the risk of suffering from heart disease. Several studies have also shown that a low-fat diet will suppress an overactive immune system. In conditions of high body temperature it is necessary to consume foods with high calories.

Lupus sufferers who have kidney problems should avoid foods with high protein content, because it will aggravate the impaired kidney function of people with lupus. If you are taking steroids, which can cause weight gain, you will need to cut calories. In lupus patients who are currently using steroids, it is also advisable to consume lots of foods that contain calcium, which is useful for avoiding bone loss (osteoporosis), & nbsp; both due to the use of steroids, and due to lupus disease & nbsp; alone. Because people with lupus are not allowed to be exposed to sunlight, it is advisable to consume lots of foods that contain lots of vitamin D.

Sports and lupus

Exercise or exercise in people with lupus is still very important. However, exercise must be adapted to the patient’s condition and the degree of activity of the disease, and you should consult with the treating doctor before starting the exercise. With practice, it is hoped that lupus sufferers can maintain muscle strength, prevent joint stiffness, overcome fatigue symptoms, and prevent weight gain.

The organization American College of Rheumatology , suggests 4 forms of exercise that can be done in people with lupus, namely:

1. flexibility exercise which includes stretching exercises and joint scope exercises, aimed at preventing stiffness and increasing flexibility of muscles and joints,

2. Muscle strengthening exercises, aiming so that strong muscles can better support the joints,

3. Aerobic exercise, including walking, cycling and swimming. This exercise aims to improve heart and lung function

4. body awareness exercises, which include yoga, tai chi, and pilates. This exercise aims to improve posture, balance, and coordination.

* The author is an Internal Medicine Specialist-Consultant in Rheumatology at Restu Ibu Hospital and RSKD Balikpapan, East Kalimantan

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