Systemic lupus erythematosus

Question 1

What is SLE?

Answer 1

Autoimmune connective tissue disease
Complex multi-system disease with various presentations
Characterised by remission and flares

Question 2

Who is it common in?

Answer 2

Females 9x more likely
Age >30 yrs

Question 3

What puts you at high risk of SLE

Answer 3

Can be familial
Oestrogens- thought to be permissive of autoimmunity

Question 4

Which ethnicity have more severe disease and prognosis

Answer 4

People of colour
Asians, Hispanic, Afro-Caribbeans

Question 5

Caucasians suffer from more ….

Answer 5

more skin and joint involvement

Question 6

COCP could cause …. in SLE

Answer 6

flare-ups in pt with lupus

Question 7

What is the pathophysiology?

Answer 7

• inadequate T cell suppressor activity with increased B cell activity
• most patients have antibodies to certain cell
  nucleus components
  Extra:
  Altered clearance of apoptotic bodies/ increase apoptosis in SLE → if not removed, apoptotic bodies degrade → release of potential auto-antigens → immune complexes formed → immune complexes can stimulate cells to produce IFN which cause inflammation
  patients often have low C1, C2, C3, C4 counts - these help clear apoptotic proteins

Question 8

What are the main symptoms and signs of SLE : mneumonic

Answer 8

SOAP BRAIN- mneumonic
Fever to fight all inflammation below

S- serositis- plerisy, pericarditis- chest pain, trouble breathing, meningitis

O- oral ulcers- usually painless, palate most specific

A- arthritis- small joints nonerosive

P-photosensitivity - malar or discoid rash (naso-labial sparing)

B- Blood disorders - Low WCC, Lymphopenia, thrombocytopenia, haemolytic anaemia

R- renal involvement- glomerulonephritis

A- autoantibodies- ANA positive >90%

I- immunological tests - low complements

N- neurological disorder- seizures or psychosis

Question 9

What are the visible symptoms ?

Answer 9

Alopecia- Inflammation on scalp- Hairloss
Malar rash/discoid rash with naso-labial sparing
Mouth ulcers
Joint inflammation- synovitis
Raynauds phenomenon
Nail fold vasculopathy

Question 10

What would you ask the patient when questioning flare-up ?

Answer 10

• Increased fatigue
• Fever
• Arthralgia
• weight changes

Question 11

What investigations show high disease activity?

Answer 11

Anti-dsDNA titre rises with disease activity
Complement proteins (C3/C4)- fall with increased disease activity

Question 12

What investigations to do for SLE?

Answer 12

• Autoantibodies
• Full blood count (normocytic anaemia of chronic disease)
• C3 and C4 levels (decreased in active disease)
• CRP - normal
• ESR and plasma viscosity- elevated due to increased Ig
• Immunoglobulins (raised due to activation of B cells with inflammation)- Coombs- detect autoantibodies
• U&Es, LFTs

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

Skin or renal biopsy can be diagnostic (renal can help prognosticate)

Question 13

What will the FBC show in SLE?

Answer 13

eukopenia→ lymphopenia
thrombocytopenia
anaemia- low Hb (of chronic disease- normocytic normochromic)

Question 14

What do you want to rule out in SLE symptoms?

Answer 14

Infection

Question 15

What autoantibodies do we test for? Which is most common?

Answer 15

anti-nuclear antibodies- ANA +ve (95% of cases)
Anti-Ro
Anti-La
Anti-dsDNA
Anti- Sm
Antiphospholipid antibodies

Question 16

Which ANA antibodies are clinically relevant in SLE?

Answer 16

IgG

Question 17

Which antibodies are specific to SLE? What are they useful for?

Answer 17

Anti-dsDNA and Anti-Smith
Anti- ds DNA- Useful in monitoring disease activity and response to treatment.

Question 18

What criteria do we use for diagnosis of SLE?

Answer 18

SLICC criteria
At least 4 criteria, with at least one clinical criterion AND one immunologic criterion
OR
Lupus nephritis as the sole clinical criterion in the presence of ANA or anti-dsDNA antibodies.

Question 19

What conservative management would you advise?

Answer 19

Sun protection (UV light triggers SLE as alters the structure of DNA makes it more immunogenic)

Healthy lifestyle advice due to CVD risk
- Smoking cessation

Question 20

What drugs trigger SLE - like symptoms

Answer 20

Isoniazid
Minocycline
TNF inhibitors

Question 21

Treatment of SLE: First line?

Answer 21

Hydroxychloroquine (HCQ) - improves rash and arthralgia
Corticosteroids- short course prednisolone for flare-ups
NSAIDs - pain management

Question 22

What other drugs are used other than first line ?

Answer 22

DMARDS - mycophenolate mofetil, methotrexate , azathioprine (preg safe)
Biologics- rituximab and Belimumab
Low dose ramipril (ACEi) - good for proteinuria, managing BP

Question 23

What other drugs are used other than first line ?

Answer 23

DMARDS - mycophenolate mofetil, methotrexate , azathioprine (preg safe)
Biologics- rituximab and Belimumab
Low dose ramipril (ACEi) - good for proteinuria, managing BP

Question 24

How does Rituximab work?

Answer 24

monoclonal antibody that targets the CD20 protein on the surface of B cell

Question 25

Some complications ?

Answer 25

Recurrent miscarriage is common in systemic lupus erythematosus

High CVD Risk- Coronary artery disease and HTN

Interstitial lung disease

Lupus nephritis →end-stage renal failure

Neuropsychiatric SLE