What is SLE a disease?
Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that can affect all organs of the body, especially the skin, joints, blood and kidneys.SLE is a chronic disease, meaning that it can last for a long time. Autoimmune disease means a disorder of the immune system. Instead of protecting the body from bacteria and viruses, the immune system attacks the patient’s own tissues instead.
The name systemic lupus erythematosus was established in the early 20th century. Systemic means affecting many organs of the body, lupus (lupus) is derived from the Latin word for wolf, referring to the typical butterfly spots on the face that remind doctors of the white marks on a wolf’s face, and erythematosus (erythema) is a Greek word meaning red, referring to a red rash.
Is this a common disease?
SLE is a rare disease that affects 0.5/100,000 children per year. It is very rare for SLE to develop before the age of 5 and very rare for it to develop before puberty.
In prepubertal children, the prevalence is higher in boys than in postpubertal children.
SLE is seen worldwide and is more common in black Americans, Latinos, Asians, and American Indians.
What are the causes of this disease?
The exact cause of SLE is not known. It is only known that SLE is an autoimmune disease and that when one has an autoimmune disease, the immune system loses the ability to distinguish between foreign invaders and one’s own tissues and cells. The immune system makes a mistake and produces autoantibodies that remove its own normal cells as foreign antigens, with the result that the autoimmune reaction causes inflammation, which in the case of SLE affects some specific organs (joints, kidneys, skin, etc.). Inflammation means that it affects parts of the body, causing localized heat, congestion, swelling and sometimes tenderness. When the inflammation lasts longer, as in the case of SLE, damage to the tissues and impairment of the normal function of the organs may occur. This is why the goal of treating SLE is to reduce inflammation.
It is now believed that multiple genetic risk factors combine with environmental factors to cause an abnormal immune response. SLE is known to be triggered by a number of factors, including hormonal imbalances during puberty and a number of environmental factors, such as sun exposure, some viral infections and the application of certain medications.
Is it hereditary?
SLE is not a genetic disorder because it is not passed directly from parent to child. However, children do inherit from their parents some as yet unknown genetic factors that may cause them to develop SLE. the presence of these factors does not necessarily mean that they develop SLE, but that they are more likely to develop the disease. It is uncommon for a child with SLE to have a relative with an autoimmune disease in the family, and it is very rare to have two children with SLE in the same family.
Why does my child have this disease? Is it preventable?
The cause of SLE is not known, but a combination of genetic susceptibility and certain environmental factors may be necessary to induce the disease. It is well established that genetic and environmental factors play separate roles in the induction of SLE.
SLE cannot be prevented, but children with the disease should avoid exposure to certain environments that can trigger and relapse the disease (such as exposure to sunlight without sunscreen, some viral infections, stress, sex hormones, and certain medications).
Is it contagious?
SLE is not contagious, it is not passed from one person to another like an infectious disease.
What are its main symptoms?
The onset of SLE is often slow, with some clinical manifestations appearing over weeks, months, or even years. The clinical manifestations are nonspecific. Lethargy and depression are the most common clinical manifestations in the early stages of SLE, and many pediatric SLE patients have intermittent or persistent fever, weight loss, and loss of appetite.
As time progresses, many children develop specific manifestations, which are caused by the involvement of organs in the body. Damage to the skin and mucous membranes is very common and can have different rashes, sun allergy (rash caused by sun exposure), ulcers of the nose and oral mucosa. The typical butterfly erythema presents as a cheek rash across the bridge of the nose and appears in 1/3 to 1/2 of affected children. Sometimes there is alopecia, and sometimes Raynaud’s phenomenon, which manifests as hands that can turn red, white, or purple when exposed to cold environments. Other symptoms include swollen and stiff joints, muscle pain, anemia, easy bleeding, headaches, seizures and chest pain. Renal involvement is present to varying degrees in most pediatric SLE patients and is a major determinant of the long-term prognosis of the disease. The main manifestations of renal involvement are increased blood pressure, hematuria and swelling, mainly in the feet, lower extremities and upper eyelids.
Does every child present the same way?
The presentation of SLE is very variable from one individual to another, so the signs and symptoms are different for each child. All of the symptoms described above can occur, either early in the course of the disease or at any time during the course of the disease.
Is there a difference between the disease in children and in adults?
In general, SLE in children and adolescents is similar to that of adults. However, in children, the lesions change more rapidly and in some ways are more severe.
How is the disease diagnosed?
SLE is diagnosed by combining clinical symptoms (e.g., pain), signs (e.g., fever), and laboratory tests, and by excluding other diseases. In order to differentiate it from other diseases, doctors from the American Rheumatism Association have established 11 criteria for diagnosing SLE.
These criteria are some of the more common presentations of SLE patients. To establish a diagnosis of SLE, a patient must have at least 4 of these 11 criteria from the start of the disease to the time of diagnosis. However, sometimes there are not enough of the above criteria for a diagnosis to be made by an experienced physician.
These criteria are
1. “Butterfly” spots. It is a red rash on the face that appears on the cheeks and the bridge of the nose.
2. Sun sensitivity. This is an excessive skin reaction to sunlight. Usually, only the exposed skin is involved, while the clothing-covered skin has no rash.
3. Discoid lupus. This is a coin-like, scaly rash that rises above the skin and appears on the face, scalp, ears, chest, or arms. After the lesions heal, they may leave a scar. Discoid lesions are more common in blacks than in other races.
4. Mucosal ulcers. Small breaks in the nose or mouth, often painless, but nasal ulcers may cause nosebleeds.
5. Arthritis. Most pediatric SLE patients have arthritis. It can cause pain and swelling in the hands, wrists, elbows, knees, or other joints of the arms and legs. The pain may be wandering, meaning that it goes from one joint to another. Arthritis in SLE is usually not a permanent change (not disabling).
6. Pleurisy. Pleurisy is an inflammation of the pleura, the outer membrane that envelops the surface of the lungs. Pericarditis is an inflammation of the pericardium, the membrane that lines the surface of the heart. Inflammation of these soft tissues can cause pericardial effusion. Pleurisy can cause specific chest pain that manifests itself as aggravation on inspiration.
7. Renal involvement. Renal involvement may occur in almost all pediatric SLE patients and can be mild or severe. It is often asymptomatic at first and may be detected only during urine tests and blood tests regarding renal function. Children with significant renal impairment may have hematuria and swelling, especially in the feet and legs.
8. Central nervous system involvement. This includes headaches, seizures and psychoneurological changes such as: inability to concentrate, memory loss, changes in temperament, depression and psychosis (a severe mental abnormality that manifests itself as a disorder of thinking and behavior).
9. Blood cell abnormalities. It is caused by autoantibodies attacking blood cells. The process of destruction of red blood cells (which are capable of carrying oxygen from the lungs to the rest of the body) is called hemolysis and can cause hemolytic anemia. This destruction may be slow and relatively mild, or it may be very rapid and require emergency treatment. Leukopenia is called hypocytosis and is usually not dangerous in SLE. Thrombocytopenia is called thrombocytopenia, and children with thrombocytopenia are prone to skin bruising and bleeding from various parts of the body, such as the gastrointestinal tract, urinary tract, uterus, and brain.
10. Immunological abnormalities. It refers to the presence of autoantibodies in the blood, which are associated with SLE.
(1) Anti-double-stranded DNA antibodies This antibody is directed against genetic material in cells and is found mainly in SLE. This test should be repeated often because anti-double-stranded DNA antibodies seem to increase when SLE is active, so this test can help doctors monitor the level of disease activity.
(2) Anti-Sm antibody refers to the patient’s name. this is the first patient in whom this antibody was found in the blood and her name was Smith. this antibody is almost unique to lupus and often helps to establish the diagnosis.
(3) Positive antiphospholipid antibody.
11. Antinuclear antibodies (ANA). are autoantibodies that directly target the nucleus and can be found in almost all patients with SLE. However, a positive ANA is not in itself proof of SLE, as it can be found in some other diseases that are not SLE, and can be weakly positive in about 5% of healthy children.