SLE

Question 1

What is SLE

Common associations

Answer 1

A multisystem, autoimmune disease characterised by B cell secretion of autoantibodies to a variety of autoantigens which form immune complexes that deposit in a range of sites eg Kidneys
Typically affects females of child bearing age and ethnic peoples

associations:

• Sjogren’s syndrome
• AI thyroid disease
• Antiphospholipid syndrome

Question 2

Diagnostic factors?

Answer 2

DRAMA SIPHON

D - discoid rash 
R - renal disorders 
A - arthritis 
M - malar rash 
A - ANA +ve 

S - serositis (pleuritis + pericarditis)
I - immunological (AB studies) 
P - photosensitivity 
H - haematological (haem anaemia, leukpoenia, throm)
O - oral ulcers 
N - neurological (seizures or psychosis)

Question 3

Symptoms and Signs of SLE

Answer 3

Symptoms:
most present predominantly with rash (of which there are several), arthritis and hameatological/immunological abnormalities

other Sx: systems inquiry essentially

• general = malaise, weight loss, fever
• MSK = arthralgia/arthritis
• Derm = rash, alopecia, oral and nasal ulcers
• Neuro = delirium, dementia, convulsions, LOV, stroke, headache, depression, anxiety
• renal = haematuria, oedema, high BP
• Resp = dyspnea and CP
• Cardio = pericarditis, myocarditis, murmurs, angina
• Haem = lymphadenopathy, anaemia, thrombophlebitis
• GI = nausea, vomiting, pseudoobstruction
• Antiphospholipid = recurrent miscarriage, DVT/PE
• Sjogrens symptoms = dry eyes, mouth, enlarged parotid

Question 4

Examination for SLE

Answer 4

General: cushingoid, weight loss, mental state
Skin: discoid and malar rash, photosensitivity
Hands: vasculitis, rash, raynauds, arthropathy
arms: livedo retiularis, purpura, proximal myopathy
head: alopecia, eyes for scleritis, mouth for ulcers, CN lesions
Chest: murmur, rub, P effusion, Pulmonary fibrosis, collapse
abdo: heptosplenomegaly
legs: rash, prox myopathy, small j synovitis, cerebellar ataxia
Other: BP, temp, urinanalysis

Question 5

Investigations for SLE

Answer 5

Haematological:
FBC: normocytic/chromic anaemia of chronic disease
: haemolytic anaema = high retics and bili
ESR elevated with normal CRP
Leukopenia and thrombocytopenia
Prolonged APTT that is not corrected with addition of normal plasma

Immunological:
ANA = sensitive not specific
Anti-dsDNA, Anti-SM - specific (70%)
Low C3 and C4 as being consumed in active disease

Other: 
renal biopsy (minimal change, membraneous, diffuses, FSGN)

Question 6

Management of SLE:

Answer 6

Acute Flares:

• Prednisolone
• IV cyclophosphamide - for life threatening flare

Maintenance:

• NSAIDs + hydroxychloroquine for joint and skin
• Steroids for CNS, Lungs, heat, haem
• Methotrexate, mycophenolate/azathioprine for steroid sparing
• warfarin for hypercoagulability - if prev DVT/PE

Lupus Nephritis:

• Steroids + cyclophosphamide
• Mycophenolate and rituximab for GN
• ACE/ARB for BP

Cutaneous Sx:

• Topical steroids
• sunscreen and avoid sun!!

Question 7

Anti-phospholipid syndrome

Answer 7

often associated with SLE
= autoimmune hypercoaulable state cause by anti-phospholipid ab causing both venous and arterial clots

CLOTS= 
Coagulation defect 
Levedo reticularis (skin mottling) 
Obstetric (recurrent miscarriages) 
Thrombocytopenia 

= TREAT with ASPIRIN or WARFARIN