Systemic Lupus Erythematosus (SLE)

Symptoms

• Fatigue: A common and persistent symptom, often debilitating.
• Butterfly Rash/ Malar rash: A characteristic red rash across the cheeks and nose, often worsened by sunlight.
• Joint Pain: Swelling, stiffness, and pain in the joints, especially in the hands, wrists, and knees.
• Photosensitivity: Sensitivity to sunlight, leading to rashes, fatigue, or flares.
• Kidney Problems: Inflammation of the kidneys (lupus nephritis), which can lead to swelling, high blood pressure, and kidney failure.
• Chest Pain: Caused by inflammation of the lining around the heart or lungs (pleuritis, pericarditis).
• Raynaud's Phenomenon: Reduced blood flow to the fingers and toes, causing them to turn white or blue in response to cold or stress.
• Hair Loss: Thinning or patchy hair loss is common in lupus.
• Mouth and Nasal Ulcers: Painful sores inside the mouth or nose.
• Neurological Symptoms: Headaches, confusion, memory issues, and seizures can occur due to brain inflammation.

How is the diagnosis done ?

• Medical History and Physical Examination: The doctor will assess symptoms, particularly those indicative of lupus, and look for characteristic signs like the butterfly rash.
• Blood Tests:
  • Antinuclear Antibody (ANA) Test: A positive ANA test is found in nearly all patients with lupus, though it is not specific to the disease.
  • Anti-dsDNA and Anti-Smith (Sm) Antibodies: These are more specific to lupus and can confirm the diagnosis.
  • Complement Levels (C3, C4): These proteins are often low in active lupus.
  • Erythrocyte Sedimentation Rate (ESR) and C-Reactive Protein (CRP): Elevated in inflammation but not specific to lupus.
  • Urinalysis: To detect kidney involvement (protein or blood in the urine).
• Biopsy: In cases of suspected lupus nephritis, a kidney biopsy may be necessary to assess the extent of kidney damage.
• Imaging: X-rays or ultrasounds may be used to evaluate damage to organs or joints.
• Lupus Criteria: The American College of Rheumatology (ACR) criteria and the Systemic Lupus International Collaborating Clinics (SLICC) criteria use a combination of clinical and laboratory findings to make the diagnosis.

Why is Early Diagnosis Important?

• Prevent Organ Damage: Early detection helps prevent or minimize damage to vital organs like the kidneys, heart, and lungs, where lupus can cause severe complications.
• Better Treatment Outcomes: With early diagnosis, treatment can be started promptly, which can help control symptoms and reduce flare-ups.
• Lower Risk of Complications: Delaying treatment increases the risk of permanent damage, including kidney failure, cardiovascular disease, and neurological impairments.
• Improved Quality of Life: Early management can reduce pain, fatigue, and other debilitating symptoms, improving a patient’s ability to function in daily life.
• Customized Treatment: Early diagnosis allows healthcare providers to tailor treatments based on the patient’s specific symptoms, avoiding unnecessary complications.

Treatment

• Hydroxychloroquine (Plaquenil) is a cornerstone of lupus treatment, especially for skin rashes and joint symptoms.
• Corticosteroids: Prednisone is commonly used to reduce inflammation during flare-ups, but long-term use is avoided due to side effects.
• Immunosuppressive Drugs:
  • Methotrexate and Azathioprine are used for moderate to severe lupus, particularly when organ systems like the kidneys or lungs are involved.
  • Mycophenolate mofetil: Often used for lupus nephritis.
• Biologic Drugs:
  • Belimumab (Benlysta) is a biologic drug that targets B-cells, which play a role in lupus pathogenesis.
  • Rituximab may also be used in certain cases of severe disease or when other treatments fail.
• Lifestyle Modifications:
  • Sun Protection: Since UV light can trigger lupus flares, patients should avoid direct sun exposure and use sunscreen.
  • Regular Exercise: Can help manage fatigue and improve mood.
  • Diet and Stress Management: Reducing stress and maintaining a healthy diet can improve overall well-being.
• Kidney-Specific Treatments: In cases of lupus nephritis, more aggressive therapies such as plasmapheresis, cyclophosphamide, or rituximab may be needed.

Prognosis

• Variable Course: Lupus is a highly variable disease, with some patients experiencing mild symptoms and others facing severe, life-threatening complications.
• Life Expectancy: With appropriate treatment, many people with lupus can live normal or near-normal lifespans. However, severe lupus can lead to complications such as kidney failure, cardiovascular disease, and infections that can reduce life expectancy.
• Flare-Ups and Remissions: Most people with lupus experience periods of disease activity (flare-ups) followed by periods of remission or low activity.
• Chronic Organ Damage: If not well controlled, lupus can cause long-term damage to organs, particularly the kidneys (lupus nephritis) and the heart.
• Early Treatment Improves Outcomes: With advances in treatment, including immunosuppressives and biologics, the prognosis has significantly improved, and patients can achieve better control of the disease and prevent irreversible organ damage.

Scoring

Several scoring systems are used to assess disease activity, severity, and organ involvement in lupus:

• SLEDAI (Systemic Lupus Erythematosus Disease Activity Index):
  The SLEDAI is a widely used score to assess lupus disease activity. It measures 24 variables, including joint, skin, renal, and neurological symptoms, and assigns points based on the severity of each.
  • 0: No activity (remission)
  • 1–5: Mild activity
  • 6–10: Moderate activity
  • >10: Severe activity
    The higher the score, the more active the disease.
• BILAG (British Isles Lupus Assessment Group) Index:
  This tool evaluates the severity of lupus symptoms in various organ systems (e.g., renal, mucocutaneous, musculoskeletal, and neurological). It provides a score for each organ based on clinical signs and symptoms.

These scoring systems help rheumatologists track disease activity, adjust treatment plans, and assess the effectiveness of therapies. Regular assessments help ensure optimal management and the prevention of organ damage in patients with lupus.