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

In women who are planning pregnancy, it is important to check for:

  • anti-Ro
  • anti-La
  • antiphospholipid antibodies

Treatment

So now you’re pretty convinced Mrs Lupus has Lupus. What are you going to do to help her?

There are a few categories of management that can be employed:

  • Education
  • Pharmacological intervention
  • Risk managment

Education involves ensuring your patient understands their condition and what it entails. Leaflets and websites can help with this too. You should also give lifestyle advice:

  • avoiding sun exposure
  • avoiding infection
  • using appropriate contraception

Pharmacological intervention includes:

  • 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

Clinical features:

  • Arthritis
    • non-erosive (Jaccoud’s arthropathy)
    • generalised arthralgia with morning stiffness but no¬†swelling is very common
  • Alopecia
  • Skin rash (discoid)
  • Photosensitivity
  • Malar rash
  • Oral ulcers
  • Fever
  • Neuropsychiatric
  • Renal
  • Cardiac
    • Pericarditis
    • myocarditis
    • endocarditis
    • pericardial tamponade
    • vasculitis
  • Haematological
    • leucopaenia early clue to SLE diagnosis
    • lymphopaenia most common manifestation of SLE other than positive ANA
    • mild neutropaenia relatively common in black people, but <1.5×10(9) is usually pathological
  • Pulmonary/pleural
    • pleurisy
    • lupus pneumonitis
    • pulmonary haemorrhage
    • pulmonary embolism
    • pulmonary hypertension
  • positive ANA (antinuclear antibodies)

Genetic factors:

  • HLA-DR2 and HLA-DR3
  • Complement C1q, C4 and C2
  • FcyRIIA, FcyRIIIA and FcyRIIB
  • CTLA-4 (a negative regulator of T cells)
  • PDCD-1 (CD28 immunoreceptor)
  • Cytokine genes IFN-a and TNF-a

Environmental triggers:

  • Drugs
    • minocycline, procainamide, hydralazine
  • Ultraviolet light
  • Viral infection
    • EBV, CMV, Retroviruses, parvovirus B19
  • Hormones
    • oestrogens
    • prolactin
  • Chemicals and heavy metals
    • silica, mercury
  • Diet
    • L-canavanine in alfalfa (maybe)

Anaemia in Lupus:

  • Normochromic normocytic anaemia of chronic disease
  • Antibody-mediated haemolytic anaemia
  • Iron-deficiency due to diet
  • Iron-deficiency due to blood loss
  • Pernicious anaemia (autoimmune)

(information from ABC of Rheumatology: Adebajo)

Leave a Reply

Fill in your details below or click an icon to log in:

WordPress.com Logo

You are commenting using your WordPress.com account. Log Out / Change )

Twitter picture

You are commenting using your Twitter account. Log Out / Change )

Facebook photo

You are commenting using your Facebook account. Log Out / Change )

Google+ photo

You are commenting using your Google+ account. Log Out / Change )

Connecting to %s