SLE

The Story

Here’s a story to help remember the key features of SLE:

Mrs Lupus is an African woman who has felt tired for as long as she can remember. She has also recently noticed a rash across her cheeks. She’s also had some joint pain and has noticed her skin reacts when she is exposed to bright sunlight. She also finds she gets mouth ulcers and her hair has fallen out in some places, leaving patches of baldness that are upsetting her.

• Ethnicity risk 
• Female gender risk
• fatigue
• malar rash
• arthritis
• photosensitivity
• mouth ulcers
• alopecia

She’s recently had a nasty bout of glandular fever, which she recons is because of her low white blood cells. It’s also made her feel very low in mood, and she’s had a pounding headache ever since it started.

• trigger – viral infection
• lymphopaenia/leucopaenia are early clues to SLE
• neuropsychiatric manifestations

You ask her about her chest, and whether she has had any difficulty breathing or chest pain, to which she says she feels short of breath and has chest pain a lot of the time, which is made better by sitting forward. She also doesn’t like to go out in the cold, as it makes her fingers go blue

• Pleurisy
• Pericarditis
• Raynaud’s phenomenon

Mrs Lupus is planning to have a baby, but wanted to ask you about the best course of action with regard to her treatment beforehand.

• Pre-pregnancy counselling is vital to ensure optimal disease control

Investigations

You decide she most likely has SLE, so what investigations are you going to do?

To diagnose and monitor her condition:

• Full blood count
  • White cell count
• Urinalysis
• Serum Creatinine
• ANA
• Anti-extractable nuclear antigen
• Anti-ribonucleoprotein = mixed connective tissue disease
• Anti-dsDNA = useful for predicting those at risk of renal disease
• Complement
  • C3 and C4 fall with disease activity

Treatment

• NSAIDS
• Corticosteroids
• Hydroxychloroquine
• Azathioprine
• Methotrexate
• Cyclosporin
• Leflunomide
• Cyclophosphamide
  • often given as ‘pulse’ intravenous therapy
    • usually for systemic vasculitis and proliferative glomerulonephritis
• Mycophenolate mofetil

The basics:

• autoimmune, multi-system, chronic disease associated with genetic and environmental risk factors
• more common in women and non-white ethnicities
• Lupus nephritis occurs in around half of patients
• patients need to be counselled before pregnancy to optimise control of the disease
• cardiovascular disease is a growing cause of death