Systemic lupus erythematosus, SLE, or simply lupus, is an autoimmune disorder where the body’s immune system attacks its own tissue. Lupus is characterized by multisystem inflammation and production of auto-antibodies that bind to the body’s own antigens.
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Inflammation and autoantibodies can cause damage to any organ, but most often affect the skin, joints, blood cells, kidneys, and brain.
Lupus typically evolves in the form of recurrent episodes called flares, followed by periods of partial or complete remission.
Symptoms vary depending on the body system that is affected, and may include:
- fatigue, fever, or headache;
- joint pain, joint stiffness or muscle pain;
- skin rash, in particular a butterfly-shaped rash on the face known as “malar rash”; or rash that appears or worsens when exposed to sunlight;
- symptoms of anemia, shortness of breath, or chest pain;
- confusion or memory loss;
- nausea, or abdominal pain;
- and urinary symptoms.
Lupus is typically diagnosed between 15 and 45 years of age. The condition occurs more often in people of African, Hispanic, or Asian origins, and is much more common in women than in men.
Lupus has a significant genetic component. A large number of genes have been linked to the disease predisposition, with most of the genes having a role in the functioning or regulation of the innate or adaptive immune response.
Evidence points to dysregulated apoptosis, which leads to increased cell death in lupus patients. Fragments of dead cells are then presented to the immune system as part of regular pathogen patrols. In a healthy immune system, these self-antigens are usually ignored, but in lupus patients, genetic defects may result in formation of intolerant lymphocytes that react to the body’s own antigens. This causes aberrant activation of auto-reactive B- and T-cells, leading to production of autoantibodies and inflammatory factors. While genetic factors underlie the background mechanism, the disease itself is typically triggered by environmental factors such as infections, drugs, or exposure to sunlight.
Because of the wide range of possible symptoms, diagnosis of lupus is challenging. Diagnostic tests aim to detect autoantibodies and identify the affected systems, and may include:
- autoantibody tests,
- complete blood count and other blood tests,
- kidney function tests,
- liver function tests,
- imaging studies for joints, heart, lung, or brain,
- and biopsy sampling for skin, kidneys, joints or nervous system.
Treatment depends on the system that is affected, but most patients are treated with medications that reduce inflammation or modify the immune system. These include: hydroxychloroquine, corticosteroids, non-biologic and biologic immuno-suppressants, and nonsteroidal anti-inflammatory drugs.
22 май 2022
