Systemic Erythomatous Lupus is a chronic inflammatory disease of autoimmune origin, the symptoms of which may appear in various organs slowly and progressively (in months) or more rapidly (in weeks) and vary with phases of activity and remission.
Two main types of lupus are recognized: the cutaneous, which manifests only with patches of skin (usually reddish or erythematous and hence the name lupus erythematosus), especially in areas that are exposed to sunlight (face, ears, neck (“V Neckline) and arms) and the systemic, in which one or more internal organs are affected.
Because it is a disease of the immune system, which is responsible for antibody production and organization of inflammation mechanisms in all organs when a person has SLE they may have different types of symptoms and various body locations. Some symptoms are generally such as fever, weight loss, loss of appetite, weakness, and discouragement. Other, organ-specific such as joint pain, skin blemishes, pleural inflammation, hypertension, and / or kidney problems.
Who has lupus?
Lupus can occur in people of any age, race, and gender, but women are much more affected. It occurs mainly between 20 and 45 years, being a little more frequent in people of mixed race and Afro-descendants. In India, we do not have exact figures, but estimates indicate that there are about 65,000 people with lupus, most of them women. It is thus believed that one in 1,700 women in Brazil has the disease.
What causes lupus?
Although the cause of SEL is not known, it is known that genetic, hormonal and environmental factors play a part in its development. Therefore, people who are born with genetic susceptibility to develop the disease, at some point, after an interaction with environmental factors (solar radiation, viral infections or other microorganisms), have immunological changes.
The main one is the imbalance in the production of antibodies that react with proteins in the body itself and cause inflammation in various organs such as the skin, mucous membranes, pleura and lungs, joints, kidneys, etc.). Thus, we understand that the type of symptom that a person develops depends on the type of autoantibody that the person has and that how the development of each antibody relates to each person’s genetic characteristics,
What are the symptoms of the disease?
The symptoms of SEL are diverse and typically vary in intensity according to the phase of activity or remission of the disease. It is very common for the person to have general manifestations such as tiredness, discouragement, low fever (but rarely, maybe high), weight loss and loss of appetite.
Manifestations may occur due to inflammation in the skin, joints (joints), kidneys, nerves, brain and membranes covering the lung (pleura) and heart (pericardium). Other manifestations may occur due to decreased blood cells (red and white blood cells) due to antibodies against these cells. These symptoms may arise in isolation or together and may occur simultaneously or sequentially. Children, adolescents or even adults may have swollen ganglia,
The most frequent clinical manifestations are:
Skin lesions: occur in about 80% of cases, throughout the course of the disease. The most characteristic lesions are reddish patches on the cheekbones and back of the nose, called butterfly wing lesions (the distribution on the face resembles a butterfly) and leave no scar.
Discoid lesions, which also occur more frequently in areas exposed to light, are well delimited and can leave scars with atrophy and changes in skin color. Vasculitis (inflammation of small vessels) can also occur on the skin, causing painful red or wine spots on the fingertips or toes.
Another very characteristic manifestation in SEL is what is called photosensitivity, which is nothing more than the development of a disproportionate sensitivity to sunlight. In this case, With only a little exposure to light or sun, it may result in skin blemishes as well as general symptoms (tiredness) or fever. Hair loss is very common but typically occurs in the activity stages of the disease and in most people hair usually regrow with treatment.
Articular: Joint pain with or without joint swelling occurs at some point in over 90% of people with SEL and mainly involves the joints of the hands, wrists, knees, and feet, which tend to be quite painful and occur in a way that occurs. intermittent, with periods of improvement and worsening. Sometimes they also arise as tendonitis.
Inflammation of the membranes covering the lung and heart is relatively common and may be mild and asymptomatic, or manifest as chest pain. Characteristically in the case of lung, pain occurs when breathing and may also cause a dry cough and shortness of breath. In pericarditis, in addition to chest pain, there may be palpitations and shortness of breath.
Kidney inflammation (nephritis): is one of the most worrying and occurs in about 50% of people with SEL. At first, there may not be any symptoms, only changes in blood and/or urine tests. In the most severe forms, high blood pressure, swelling in the legs, urine becomes foamy and there may be a decrease in the amount of urine. When not treated promptly and properly the kidney fails (kidney failure) and the patient may need dialysis or kidney transplantation.
Neuropsychiatric disorders: These manifestations are less frequent, but may cause seizures, mood or behavior changes (depression), depression, and peripheral nerve and spinal cord changes.
Blood: Changes in blood cells are due to antibodies against these cells, causing their destruction. Thus, if antibodies against red blood cells (RBCs) will cause anemia, against white blood cells will cause decreased white cells (leukopenia or lymphopenia) and if against platelets will cause decreased platelets (thrombocytopenia).
Symptoms caused by changes in blood cells vary widely. Anemia can cause pale skin and mucous membranes and tiredness, and thrombocytopenia can cause increased menstrual bleeding, bruising and gum bleeding. Usually, the decrease in white blood cells is asymptomatic.
How is the diagnosis made?
The diagnosis is made by the doctor recognizing one or more of the above symptoms. At the same time, as some changes in blood and urine tests are very characteristic, they are also commonly used for the final definition of the diagnosis. Common blood and urine tests are useful not only for the diagnosis of the disease but are also very important in defining whether there is SLE activity.
Although there is no test that is exclusively for SEL (100% specific), the presence of the test called ANF (antinuclear factor or antibody), especially with high titers, in a person with signs and symptoms characteristic of SEL, allows the diagnosis with much assurance.
Other laboratory tests such as anti-Sm and anti-DNA antibodies are very specific but occur in only 40% to 50% of people with SEL. At the same time, some blood and/or urine tests may be ordered to assist not in diagnosing SEL but in identifying whether or not there are signs of disease activity.