Systemic Lupus Erythematosus (SLE) Quiz

Systemic Lupus Erythematosus (SLE)

• SLE is the most common connective tissue disease.
• 90% of affected individuals are women.
• The peak age at onset is between 20 and 30 years.
• Common symptoms include fever, skin rash, arthralgia, oral ulcers, weight loss, hair loss, and Raynaud's phenomenon.

Diagnostic Criteria for SLE

• Malar rash: Fixed erythema, flat or raised, sparing the nasolabial folds.
• Discoid rash: Erythematous raised patches with adherent keratotic scarring.
• Photosensitivity: Rash due to unusual reaction to sunlight.
• Oral ulcers: Oral or nasopharyngeal ulceration, which may be painless.
• Arthritis: Non-erosive, involving two or more peripheral joints.
• Serositis: Pleuritis (pleuritic pain or rub, or pleural effusion) or pericarditis (rub, effusion).
• Renal disorder: Persistent proteinuria > 0.5 g/day or cellular casts.
• Neurological disorder: Seizures or psychosis.
• Haematological disorder: Haemolytic anaemia or leucopenia, or lymphopenia, or thrombocytopenia.
• Immunological disorder: Anti-DNA or Anti-Smith or antiphospholipid antibodies.
• Antinuclear antibody (ANA) disorder: Abnormal titre of ANA by immunofluorescence.

Management of SLE

Mild to Moderate

• Avoid exposure to sunlight and use sun block.
• Use NSAIDs.
• Use hydroxychloroquine.
• Use steroid.
• Use azathioprine or methotrexate.
• Control of CV risk factors.

Life-threatening Lupus

• Use methylprednisolone IV.
• Use cyclophosphamide IV.
• Use mycophenolate mofetil (MMF).
• Use rituximab.
• Use biologics; belimumab.

Antiphospholipid Syndrome (APS)

• Clinical syndrome presented mainly by recurrent miscarriages and recurrent arterial and venous thrombosis.
• Characterized by presence of anticardiolipin antibodies and lupus anticoagulants which target protein C or β2-glycoprotein and lead to thrombosis.
• May occur alone (primary APS) or in association with other autoimmune disease like SLE, SSc, RA.

Clinical Features of APS

• Thromboembolic events.
• Adverse pregnancy outcome.
• Venous thromboembolism.
• Arterial thromboembolism.
• Livedo reticularis.
• Skin necrosis.
• Catastrophic APS: acute vascular occlusion in 3 different organs within short time less than one week.

Diagnostic Criteria of APS

• Arterial or Venous thromboembolic events.
• Adverse pregnancy outcome (repeated abortions, premature labor, intrauterine fetal death).
• Livedo reticularis.
• Positive antiphospholipid antibodies (lupus anticoagulants, anticardiolipin antibodies, anti-beta2 glycoprotein) at least 3 months apart.

Treatment of APS

Primary Prophylaxis against Thrombotic Events

• Use aspirin.
• Use statins.
• Use hydroxychloroquine.
• Use vitamin K antagonists (Warfarin).

Secondary Prophylaxis against Thrombotic Events

• Use vitamin K antagonists (Warfarin).
• The desired INR target: ≥ 2.
• Use direct oral anticoagulants (DOAC): ±.

During Pregnancy

• Treatment aims to improve the pregnancy outcome.
• Use aspirin + heparin.
• Use aspirin + low molecular weight heparin (LMWH).