Description
Systemic Lupus Erythematosus (SLE) is a chronic autoimmune disease that can affect various parts of the body, including the skin, joints, kidneys, heart, lungs, brain, and blood vessels. In SLE, the body's immune system mistakenly attacks its own tissues, leading to inflammation and damage. The exact cause of SLE is not fully understood, but it is believed to involve a combination of genetic, environmental, and hormonal factors.One of the hallmark features of SLE is its wide range of symptoms, which can vary greatly from person to person and can also fluctuate over time. Common symptoms include fatigue, joint pain and stiffness, skin rashes (such as the classic "butterfly" rash across the cheeks and nose), fever, photosensitivity (sensitivity to sunlight), hair loss, mouth ulcers, and chest pain with deep breathing.SLE can also cause complications in various organs and systems of the body. For example, inflammation of the kidneys (known as lupus nephritis) can lead to kidney damage and impaired function. Inflammation of the heart and blood vessels can increase the risk of cardiovascular disease. Inflammation of the brain and nervous system can cause headaches, confusion, seizures, and other neurological symptoms.
Résumé
- Lupus, once a highly fatal autoimmune disease, is now a chronic, manageable condition with normal life expectancy through medication. It's an autoantibody-mediated disease, presenting a diverse range of symptoms that often require multiple specialists for diagnosis and treatment.
- The disease primarily affects young women of childbearing age (15-45) and can be devastating due to its potential to impact major organs. While genetic factors contribute, it's not a simple Mendelian inheritance but rather a complex interplay of multiple gene polymorphisms. Environmental triggers, such as drugs, infections, sunlight, and viral infections like EBV, play a significant role.
- Estrogens also play a role, altering immune profiles and influencing phenotypic expression. This contributes to a higher prevalence in females, with a ratio of 7:1 compared to males. The underlying mechanism involves both B-cell and T-cell deficits, leading to the production of antibodies against nuclear, cytoplasmic, and cell wall antigens.
- Various types of lupus exist, including discoid lupus (DLE), neonatal lupus, drug-induced lupus (DIL), and systemic lupus erythematosus (SLE), which manifests systemically with acute or chronic features.
- Clinical presentation can vary widely, often including a characteristic malar rash exacerbated by sun exposure, along with different forms of skin lesions. Arthritis is another common symptom, often presenting as seronegative polyarthritis. Renal involvement is a serious complication, potentially leading to nephrotic syndrome, nephritis, or chronic kidney disease.
- Neurological manifestations (NPSLE) can include seizures, psychosis, focal deficits, headaches, and depression. Cardiovascular complications can affect the endocardium, myocardium, and pericardium. Pulmonary involvement can result in pleural effusion, vanishing lung syndrome, pneumonia, or diffuse alveolar hemorrhage.
- Hematological findings are diverse, including anemia, lymphopenia, thrombocytopenia, and Coombs-positive hemolytic anemia. Liver manifestations, such as ascites and abdominal pain, can also occur.
- Management includes symptomatic therapy, immune therapy, and biological therapy. Antimalarials, corticosteroids, and immunosuppressants like mycophenolate, azathioprine, and cyclophosphamide are commonly used. Biological medications such as IVIG, rituximab, and belimumab are also employed. Sunscreen is crucial, especially in sunny regions, with an SPF of 30-40 recommended. Comprehensive management by a rheumatologist is essential.
Exemple de certificat
À propos des intervenants
Dr Shanmuganandan Krishnan
Professeur SBMCH, consultant en rhumatologie, hôpitaux Apollo, Chennai
Divulgation financière
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