Systemic Lupus Erythematosus: An Autoimmune Disease Affecting Multiple Organs

Systemic Lupus Erythematosus: An Autoimmune Disease Affecting Multiple Organs

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that causes inflammation in various organs throughout the body. The immune system of people with SLE attacks healthy tissues and organs, leading to damage and a wide range of symptoms.

# Symptoms of Systemic Lupus Erythematosus

Systemic Symptoms:

• Fever: The most common symptom, usually mild and persistent.

• Fatigue: Prolonged fatigue, often accompanied by weight loss.

Muscle Symptoms:

• Myositis: Causes pain, muscle weakness, increased muscle enzymes, and muscle atrophy. Muscle biopsy reveals interstitial infiltration.

Bone Symptoms:

• Avascular necrosis of bone: Occurs in 10% of patients.

Joint Symptoms:

• Arthritis: Common in small, medium, and symmetrical joints. It can be acute, subacute, or chronic.

• Non-destructive arthritis: However, prone to infectious arthritis.

Skin and Mucosal Lesions:

• Butterfly rash: Acute, flat rash, pink in color, sensitive to sunlight.

• Discoid rash: Chronic.

• Mouth and nose ulcers: A common symptom.

• Raynaud’s phenomenon: Causes numbness, tingling, whitening, or bluing of the fingertips and toes when exposed to cold.

Kidney Damage:

• Glomerulonephritis: Occurs in 70-80% of patients.

Neurological and Psychiatric:

• Generalized seizures: Occurs in 15%, often responsive to corticosteroids and antiepileptic drugs.

• Headaches, migraines, syncope.

• Cranial nerve damage, peripheral neuropathy, transverse myelitis, chorea.

• Psychiatric: 40-50% of patients exhibit psychosis, acute or chronic psychiatric disorders.

Cardiovascular:

• Pericarditis.

• Libman-Sacks endocarditis.

• Myocarditis.

• Arterial and venous thrombosis.

• Hypertension.

• Coronary artery disease.

Lung and Pleura:

• Pleural effusion: Unilateral or bilateral.

• Atypical lupus pneumonia.

Blood Symptoms:

• Leukopenia: Mainly lymphocytes, commonly seen during disease progression.

• Anemia: Common, inflammatory anemia (normocytic, normochromic), Coombs-positive hemolytic anemia.

• Thrombocytopenia: Peripheral platelet depletion.

Gastrointestinal:

• Liver dysfunction.

• Ascites.

• Acute pancreatitis.

• Gastrointestinal disorders.

Eye:

• Retinitis, retinal exudate.

• Conjunctivitis.

• Sjogren’s syndrome: Dryness of the lacrimal and salivary glands.

# Diagnosis of Systemic Lupus Erythematosus

Diagnostic Laboratory Tests:

• Hematology: Increased erythrocyte sedimentation rate during acute episodes, mild increase in CRP during disease progression. If elevated, it suggests infection.

• Immunological Tests:

• Antinuclear antibodies (ANA): Almost always positive in SLE.

• Anti-double-stranded DNA antibodies (dsDNA): Highly specific for SLE.

• Antiphospholipid antibodies: Include anticardiolipin antibodies.

• Latex test, Waaler-Rose: Used for diagnosing syphilis, but can also be positive in SLE.

ACR 1997 Diagnostic Criteria for SLE:

To diagnose SLE, patients must meet at least 4 out of the following 11 criteria:

1. Malar rash.

2. Discoid rash.

3. Photosensitivity.

4. Oral ulcers.

5. Nonerosive arthritis.

6. Pleuritis or pericarditis.

7. Renal disorder: Proteinuria > 500mg/24h or cellular casts.

8. Neurological disorder: Not attributable to another cause.

9. Hematologic disorder.

10. Immunological disorder: Anti-DNA antibodies or antiphospholipid antibodies.

11. Antinuclear antibodies (ANA).

# Causes of Systemic Lupus Erythematosus

• Exact cause remains unknown: Could be due to genetic factors, hormones, environment, infections, etc.

• Drug-induced lupus: Certain drugs can cause symptoms similar to SLE, known as drug-induced lupus.

• Antihypertensive medications: Hydralazine, methyldopa.

• Antiarrhythmic medications: Procainamide, quinidine.

• Sulfadiazine, interferon, antithyroid drugs…

# Treatment of Systemic Lupus Erythematosus

Treatment Goals:

• Symptom control: Reduce inflammation, pain, and fatigue.

• Prevent organ damage: Protect kidneys, heart, and brain.

• Improve quality of life: Help patients maintain normal activities.

Treatment Approaches:

• Treatment for mild cases: NSAIDS + antimalarials. During disease progression, corticosteroids in low doses (10-20mg/day) can be used for a short period.

• Treatment for severe cases: Corticosteroids 1-2mg/kg/day + antimalarials. In severe cases, azathioprine 1.5-2.5mg/kg/day may be used. If life-threatening, cyclophosphamide may be administered.

• Treatment for thrombocytopenia, hemolytic anemia: Corticosteroids, if resistant to corticosteroids, use dapsone or vinblastine infusion. Splenectomy is often performed followed by anti-pneumococcal vaccination.

• Treatment for neurological and psychiatric disorders: Corticosteroids + antimalarials.

• Treatment for cutaneous lesions: Topical corticosteroids + sunscreen.

• Treatment for antiphospholipid syndrome: Anticoagulants.

Note:

• SLE treatment should be performed under the supervision of a qualified specialist.

• Adhere to the treatment regimen and monitor regularly to detect complications early.

• Lifestyle changes: Avoid sun exposure, get enough rest, eat a balanced diet, and engage in light exercise.

# Systemic Lupus Erythematosus and Pregnancy

• Risks for the mother: Pregnancy can worsen SLE, particularly kidney damage.

• Risks for the fetus: Miscarriage, preterm birth, stillbirth.

• Conditions for pregnant women with SLE:

• No clinical disease progression or laboratory abnormalities in the 6 months to 1 year prior.

• No contraindications: Cardiovascular disease, severe hypertension, uncompensated valvular heart disease, history of thrombosis, dependence on corticosteroids > 0.5mg/kg/day.

• SLE management during pregnancy:

• Mild cases: Corticosteroids 0.2-0.3mg/kg/day. If not already using, use corticosteroids 0.5mg/kg/day in the last trimester. Hydroxychloroquine can be continued (especially with a history of kidney disease).

• Progressive cases: Azathioprine or cyclosporine A, after delivery, corticosteroids should not be reduced.

Contraception issues:

• Intrauterine devices (IUDs) are not recommended: Increased risk of infection.

• Use very low-dose oral contraceptives: Luteran 5…

• Contraindications for oral contraceptives: History of thrombosis, circulating antithrombin antibodies, or false positive serological tests.

# Conclusion

Systemic lupus erythematosus is a chronic autoimmune disease affecting multiple organs. The disease is treatable but not curable. Regular monitoring, adherence to the treatment regimen, and appropriate lifestyle modifications are crucial for managing the disease, improving quality of life, and preventing complications.