SLE / Discoid LE

Question 1

SLE- what is it?

Answer 1

systemic lupus erythematousus

inflammatory autoimmune connective tissue disease

systemic= affects lots of organs
erythematous= red malaria rash
varying and non specific symptoms
relapsing and remitting
chronic inflammation so shortened life expectancy, CVS disease and infection

Question 2

what is the pathophysiology of SLE

Answer 2

anti nuclear antibodies to proteins within the persons own cell nucleus. creates an inflammatory response

Question 3

symptoms of SLE

Answer 3

Fatigue
Weight loss
Arthralgia (joint pain) and non-erosive arthritis
Myalgia (muscle pain)
Fever
Photosensitive malar rash. This is a “butterfly” shaped rash across the nose and cheek bones that gets worse with sunlight.
Lymphadenopathy and splenomegaly
Shortness of breath
Pleuritic chest pain
Mouth ulcers
Hair loss
Raynaud’s phenomenon

Question 4

investigations of SLE

Answer 4

Autoantibodies (see below)

Full blood count (normocytic anaemia of chronic disease)

C3 and C4 levels (decreased in active disease)

CRP and ESR (raised with active inflammation)
Immunoglobulins (raised due to activation of B cells with inflammation)

Urinalysis and urine protein:creatinine ratio for proteinuria in lupus nephritis

Renal biopsy can be used to investigate for lupus nephritis

Question 5

what are the auto antibodies involved in SLE?

Answer 5

ANA: auto nuclear antibodies
gains the normal protein int eh cell nucleus
blood test for ANA

anti-dsDNA (anti double stranded DNA).

*antibodies to extractable nuclear antigens (anti ENA antibodies) will check for antibodies to specific protein in the cell nucleus:

Anti-Smith (highly specific to SLE but not very sensitive)
Anti-centromere antibodies (most associated with limited cutaneous systemic sclerosis)
Anti-Ro and Anti-La (most associated with Sjogren’s syndrome)
Anti-Scl-70 (most associated with systemic sclerosis)
Anti-Jo-1 (most associated with dermatomyositis)

antiphsopholipid antibodies

Question 6

how is SLE diagnosed?

Answer 6

SLICC criteria
ACR crtieria
confirm the presence of antinuclear antibodies and establishing a certain number of clinical features

Question 7

what are the complications of SLE?

Answer 7

1. CVS (blood vessels - HTN, coronary artery disease)
2. infection (2’ to immunosuppressants)
3. anaemia of chronic disease. affects the bone marrow - normocytic anaemia. leucoplacia, neutropenia, thrombocytoepnia
4. pericarditis / pleurisy
5. interstitial lung disease (pulmonary fibrosis)
6. lupus nephritis
7. neuropsychiatric SLE (inflammation of the CNS) (optic neuritis, transverse myelitis, psychosis)
8. recurrent miscarriage (intrauterine growth restriction, pre eclampsia, pre-term labour)
9. venous thromboembolism
   (antiphospholipid syndrome)

Question 8

treatment for SLE

Answer 8

anti inflammatory meds
immunisupression

conservative:
suncream
sun avoiance

medical:
NSAID, steroids, hydroxychloroquine
methotrexate
mycophenolate mofetil
azathioprine
tarcolimus

biological therapies
rituimab
belimumab

Question 9

discoid lupus erythematous

Answer 9

non cancerous chronic skin condition more common in young women, darker skinner patients and smokers

associated with an increased risk of developing SLE and rarely progresses to SCC

Question 10

presentation of discoid lupus erythematous

Answer 10

face, ears, scalp 
photosensitive
scarring and alopecia
hyper-pigmented
hypo-pigmented scars

Inflamed
Dry
Erythematous
Patchy
Crusty and scaling

Question 11

management of discoid lupus erythematous

Answer 11

Sun protection
Topical steroids
Intralesional steroid injections
Hydroxychloroquine

skin biopsy to confirm diagnosis