Systemic lupus erythematosus (SLE)

Question 1

Describe SLE?

Answer 1

• Multisystem autoimmune disease
• Autoantibodies against autoantigens (ANA) which form immune complexes
• Incomplete removal of the complexes results in immune responses
  
  • Causes tissue inflammation and damage
• Environmental triggers play a part (Epstein barr virus)

Question 2

Who is SLE more prevalent in?

Answer 2

• Afro-Caribbeans, Asians
• HLA B8, DR2 or DR3 positive

Question 3

Clinical features of lupus?

Answer 3

• Relapsing, remitting with variable presentation
• Often non-specific (malaise, fatigue, myalgia, fever)
• Lymphadenopathy, weight loss, alopecia, nail-fold infarcts, Raynaud’s

Question 4

What is Raynaud’s?

Answer 4

• Peripheral digit ischaemia
  
  • Due to paroxysmal vasospasm
• Triggered by cold or emotion

Question 5

Describe the immunology of SLE?

Answer 5

• ANA (+)
• High dsDNA antibody titre
• ENA, anti-RO, anti-La, anti-Sm, anti-RNP
• Rheumatoid factor
• Anti-cardiolipin, lupus anticoagulant

Question 6

What other AI conditions can SLE be associated with?

Answer 6

• Sjogren’s
• Autoimmune thyroid disease

Question 7

Describe the diagnosis of SLE?

Answer 7

1. Anti-dsDNA antibody titres
2. Complement (Reduced C3, C4)
3. ESR
   
   • FBC, U&Es, LFTs, CRP

Question 8

When should SLE be considered?

Answer 8

• Multisystem disorder
• Raised ESR
• Normal CRP

Question 9

Describe drug induced lupus?

Answer 9

• >80 drugs
• Isoniazid, hydralazine, phenytoin
• Procainamide, quinidine, chlorpromazine
• Anti-TNF agents

Question 10

Describe the management of SLE?

Answer 10

• Sunblock
• Hydroxychloroquine
  
  • Reduces disease activity and improves survival
• Topical steroids for skin flares

Question 11

Describe the maintenance of SLE?

Answer 11

• NSAIDs
• Hydroxychloroquine for joint an skin symptoms
• Steroid sparing: Azathioprine, methotrexate, mycophenolate
• Belimumab (monoclonal antibody) as add on therapy

Question 12

How can mild flares of SLE be managed?

Answer 12

• Hydroxychloroquine or
• Low-dose steroids

Question 13

How can moderate flares of SLE be managed?

Answer 13

• DMARDs or
• Mycophenolate

Question 14

How can severe flares of SLE be managed?

Answer 14

• Urgent high-dose steroids
• Mycophenolate
• Rituximab
• Cyclophosphamide

Question 15

Describe Antiphospholipid syndrome?

Answer 15

• Antiphospholipid antibodies
  
  • Cardiolipin
  • Lupus anticoagulant
  • Anti- beta2 glycoprotein 1
• Signs: CLOTS

Question 16

What are the signs of Antiphospholipid syndrome?

Answer 16

• CLOTS
  
  • Coagulation defect
  • Livedo reticularis
  • Obstetric (recurrent miscarriage)
  • Thrombocytopenia

Question 17

Describe the diagnosis and treatment of antiphospholipid syndrome?

Answer 17

• Diagnosis
  
  • Persistent antiphospholipid antibodies with clinical features
• Treatment
  
  • Anticoagulation
  • Seek advice in pregnancy

Question 18

Describe Systemic lupus international collaborating clinics classification

Answer 18

Question 19

Marker of Drug induced lupus?

Answer 19

Anti-histone antibodies