Systemic Lupus Erythematous/SLE (1)

Question 1

What is it?

Who’s it more common in?

What is its pathophysiology?

What are the main causes of death in these pts?

Answer 1

➊ Multi-system, autoimmune, inflammatory disease

➋ Afro-Caribbeans women of child-bearing age (peak age of onset 20-40 yrs)

➌ Presence of Anti-Nuclear Ab’s (ANA) and anti-dsDNA leads to chronic inflammation

➍ CVD and Lupus Nephritis

Question 2

How does it present?
→ What are the triggers?

What are its features?

Answer 2

➊ Follows a relapsing-remitting course, with flares being triggered
→ • Overexposure to sunlight
• Infections
• Stress
• Oestrogen-containing contraception (e.g. COCP)

➋ • Non-specific symptoms - Fatigue, Weight loss, Fever, Malaise
• Arthralgia
• Photosensitive Malar rash - butterfly-shaped rash worsened by sunlight
• Mouth/nose/genital ulcers
• Lymphadenopathy, Splenomegaly
• CVS - Raynaud’s, Pericarditis, Myocarditis
• Lupus nephritis - usually asymptomatic before presenting as nephritic/otic syndrome

Question 3

Investigations:
Which bloods should be done?

What can be used to monitor disease activity and rx response?

What is important to assess for in these pts?
→ How is this done?

Answer 3

➊ • ANA - +ve in most cases but not diagnostic
• Anti-dsDNA and Anti-Smith - Much more SLE specific than ANA, so can be diagnostic
• C3, C4 - Low
• Anti-phospholipid (e.g. Anticardiolipin)
• ESR
• FBC and Clotting - A raised PT suggests presence of lupus anticoagulant and should prompt checking for anti-phospholipid syndrome. These pts often have anaemia of chronic disease.
• U+Es and urinalysis - screen for renal involvement

➋ Anti-dsDNA

➌ Lupus Nephritis
→ • Urinalysis and ACR - for haematuria and proteinuria
• Renal biopsy

Question 4

Diagnosis:
What is needed for a diagnosis?

What are the clinical findings?

What are the laboratory findings?

Answer 4

➊ 4+ findings (at least 1 clinical and 1 laboratory) OR Biopsy-proven Lupus nephritis with +ve ANA/Anti-dsDNA

➋ SOAP BRAIN MD:
• SOAP - Serositis (pericarditis, pleuritis), Oral ulcers, Arthritis, Photosensitivity rash
• BRAIN - Blood, Renal protein, ANA +ve, Immunologic (dsDNA +ve), Neurolopsyhiatric (seizures, psychosis)
• MD - Malar rash, Discoid rash

➌ • ANA +ve
• Anti-dsDNA +ve
• Anti-Smith +ve
• Ant-cardiolipin +ve
• Low complement (C3 or C4)
• +ve Direct Coombs test

Question 5

Management:
What is the mainstay of treatment?

What is given if there’s prominent organ involvement?
→ What’s usually given in combination with this? Why?

What is given for very severe flares with renal, neurological or haematological effects?

What can renal involvement lead to?
→ How is this managed?

Answer 5

➊ • NSAIDs and Hydroxychloroquine for mild disease +/- short-term corticosteroids for flares
• Sunscreen for photosensitive malar rash

➋ Long-term corticosteroids
→ DMARDs as a ‘steroid sparing agent’ to reduce the steroid dose

➌ High dose corticosteroids in combination with immunosuppressants - Cyclophosphamide usually most effective

➍ HTN
→ ACEi

Question 6

Antiphospholipid Syndrome (APS):
What is it?

How does it present?

How is it diagnosed?

How is it managed?

Answer 6

➊ Hypercoagulable state due to anti-cardiolipin, leading to clots as well as pregnancy-related complications, like miscarriage, stillbirth and pre-eclampsia

➋ CLOT:
* Coagulation defect - usually VTE, but can be arterial
* Livedo reticularis - mottled, cyanotic skin discolouration
* Obstetric - recurrent miscarriage, pre-eclampsia
* Thrombocytopenia

➌ 1+ of the following +ve on 2 occasions, 12-weeks apart:
* Anti-cardiolipin
* Anti-beta2-GPI
* Positive lupus anticoagulant assay

➍ * 75 mg Aspirin and LMWH - warfarin avoided as it’s teratogenic
* Avoid COCP/HRT to reduce risk of VTE

N.B. It’s important to note that, outside of pregnancy, APS is treated with Warfarin.