### Date : 2024-06-24 16:27 ### Topic : Systemic Lupus Erythematosus (SLE) #medicine #rheumatology #internalmedicine ---- ### Systemic Lupus Erythematosus (SLE) Overview **Systemic Lupus Erythematosus (SLE)** is a chronic autoimmune disease characterized by inflammation and damage to various body tissues and organs. The immune system mistakenly attacks healthy tissue, causing widespread inflammation and tissue damage. SLE can affect the skin, joints, kidneys, brain, and other organs. ### Etiology **Multifactorial Causes:** - **Genetic Factors:** - Family history of autoimmune diseases increases the risk. - Specific genetic markers, such as certain HLA types, are associated with SLE. - **Environmental Factors:** - Ultraviolet (UV) light exposure. - Infections (e.g., Epstein-Barr virus). - Certain medications (drug-induced lupus). - Smoking. - **Hormonal Factors:** - SLE is more common in women, particularly during reproductive years, suggesting a role for hormones like estrogen. ### Pathophysiology 1. **Autoimmune Response:** - The immune system produces autoantibodies against self-antigens, forming immune complexes. 2. **Immune Complex Deposition:** - These immune complexes deposit in various tissues, triggering an inflammatory response. 3. **Inflammation and Tissue Damage:** - Chronic inflammation leads to damage in the affected organs, contributing to the diverse clinical manifestations of SLE. ### Clinical Features **1. Systemic Symptoms:** - **Fatigue:** Common and often severe. - **Fever:** Low-grade fever not due to infection. - **Weight Loss:** Unintentional weight loss. **2. Musculoskeletal:** - **Arthritis:** Non-erosive arthritis affecting multiple joints (polyarthritis), commonly involving the hands, wrists, and knees. - **Myalgia:** Muscle pain. **3. Dermatologic:** - **Malar Rash:** Butterfly-shaped rash across the cheeks and nose. - **Discoid Rash:** Raised, scaly patches, often leaving scars. - **Photosensitivity:** Skin rash resulting from exposure to sunlight. - **Alopecia:** Hair loss. **4. Renal:** - **Lupus Nephritis:** Inflammation of the kidneys, which can lead to proteinuria, hematuria, and renal dysfunction. **5. Cardiopulmonary:** - **Pleuritis:** Inflammation of the lining of the lungs, causing chest pain. - **Pericarditis:** Inflammation of the lining around the heart. - **Myocarditis:** Inflammation of the heart muscle. **6. Neurologic:** - **Seizures:** Occur in some patients. - **Psychosis:** Mental health issues, including mood swings and cognitive dysfunction. - **Headaches:** Including migraines. **7. Hematologic:** - **Anemia:** Low red blood cell count. - **Leukopenia:** Low white blood cell count. - **Thrombocytopenia:** Low platelet count. **8. Gastrointestinal:** - **Nausea and Vomiting:** Common gastrointestinal symptoms. - **Abdominal Pain:** Due to serositis or vasculitis. ### Diagnosis **1. Clinical Evaluation:** - Detailed patient history and physical examination focusing on the characteristic symptoms and signs of SLE. **2. Laboratory Tests:** - **Autoantibodies:** Antinuclear antibodies (ANA), anti-double-stranded DNA (anti-dsDNA), anti-Smith (anti-Sm), anti-phospholipid antibodies. - **Complete Blood Count (CBC):** To detect anemia, leukopenia, and thrombocytopenia. - **Urinalysis:** To detect proteinuria and hematuria indicating kidney involvement. - **Complement Levels:** Low levels of complement proteins (C3, C4) can indicate active disease. **3. Imaging Studies:** - **Chest X-Ray or CT Scan:** To evaluate lung involvement. - **Echocardiogram:** To assess heart involvement. **4. Biopsy:** - **Renal Biopsy:** May be necessary to diagnose and classify lupus nephritis. ### Example Case Study **Patient Profile:** - **Name:** Jee Hoon Ju - **Age:** 28 - **Occupation:** School Teacher **Medical History:** - Complaints of joint pain, fatigue, and a facial rash exacerbated by sunlight. **Clinical Evaluation:** - **Symptoms:** Butterfly rash on the face, joint swelling in hands and knees, and generalized fatigue. - **Laboratory Tests:** - Positive ANA, anti-dsDNA, and anti-Sm antibodies. - CBC shows anemia and leukopenia. - Urinalysis indicates proteinuria. **Diagnosis:** Based on clinical presentation and laboratory findings, Jee Hoon Ju is diagnosed with systemic lupus erythematosus (SLE). ### Management **1. Medication:** - **NSAIDs:** For pain and inflammation in mild cases. - **Antimalarials:** Hydroxychloroquine for skin and joint symptoms. - **Corticosteroids:** For acute flares and severe symptoms. - **Immunosuppressants:** Such as azathioprine, methotrexate, or mycophenolate mofetil for more severe disease. - **Biologics:** Belimumab, a monoclonal antibody, for refractory cases. **2. Lifestyle Modifications:** - **Sun Protection:** Use of sunscreen and protective clothing to avoid photosensitivity reactions. - **Diet and Exercise:** Balanced diet and regular exercise to maintain overall health. - **Smoking Cessation:** Smoking can exacerbate SLE symptoms and complications. **3. Monitoring and Follow-Up:** - Regular follow-up visits to monitor disease activity and adjust treatment. - Frequent laboratory tests to assess organ function and medication side effects. **4. Patient Education:** - Informing the patient about the nature of the disease, treatment options, and the importance of adherence to therapy. - Support groups and counseling for emotional and psychological support. ### Prognosis - **Variable Course:** SLE can range from mild to severe. With early diagnosis and appropriate treatment, many patients manage their symptoms effectively. - **Chronic Disease:** SLE is a lifelong condition that requires ongoing management. - **Complications:** Can include renal failure, cardiovascular disease, and increased risk of infections due to immunosuppressive therapy. ### Conclusion Systemic lupus erythematosus is a complex autoimmune disease with diverse clinical manifestations. It requires a comprehensive approach to diagnosis and management, including medications, lifestyle modifications, and regular monitoring. With appropriate treatment and follow-up, many patients with SLE can lead productive and fulfilling lives. Understanding the etiology, pathophysiology, and management strategies is crucial for effectively treating this chronic condition.