SLE Flashcards

1
Q

What is the general pathophysiology behind SLE

A

Autoabs made against a variety of auto antigens which form immune complexes
Poor clearance of immune complexes results in a host of immune responses -> tissue inflammation and damage

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2
Q

What sex is more commonly affected?

A

Female 9:1

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3
Q

What group of people is it more common in?

A

Afro-caribbean and Asians

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4
Q

What is the usual age of onset ?

A

20-40

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5
Q

What genes is it associated w?

A

HLA B8, DR2, DR3

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6
Q

How is a diagnosis made?

A

≥ 4 criteria (1 clinical and 1 lab)
OR
biopsy proven lupus nephritis w + ANA or antiDNA

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7
Q

What are the 11 clinical criteria?

A
  1. Acute cutaneous lupus
  2. Chronic cutaneous lupus
  3. Non-scarring alopecia
  4. Oral/nasal ulcers
  5. Synovitis
  6. Serositis
  7. Urinalysis - proteinuria or red cell casts
  8. Neuro features
  9. Haemolytic anaemia
  10. Leucopenia
  11. Thrombocytopenia
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8
Q

What are the lab criteria?

A
  1. +ve ANA
  2. Anti-dsDNA
  3. Anti-smiths abs
  4. Anti-phospholipid abs
  5. Low complement (3,4,50)
    • direct Coombs test
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9
Q

What are the features of acute cutaneous lupus ?

A
  • malar rash
  • fixed erythema, flat or raised over malar eminence, sparing nasolabial folds
  • Bullous lupus - toxic epidermal necrolysis variant of SLE
  • Photosensitive rash
  • maculopapular lupus rash
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10
Q

What are chronic cutaneous features of lupus?

A
  1. discoid rash - erythematous scaly well demarcated rash in sun exposed areas
    lesions may progress to become pigmented + hyperkeratotic before atrophic
    3 stage rash - inc ears, cheeks, scalp, forehead and chest: erythema ->pigmented hyperkeratotic oedematous papules -> atrophic depressed lesions
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11
Q

How many joints need to be affected by synovitis to meet the clinical criteria?

A

≥2 joints or ≥2 tender joints w >30mins morning stiffness

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12
Q

What are the features of serositis as part of the clinical criteria?

A

a. lungs - pleurisy, pleural rub, effusion

b. Pericardial - pain >1day, effusion, rub or pericarditis on ECG

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13
Q

What are the possible neurological features of lupus?

A
seizures
psychosis
mononeuritis multiplex
myelitis
peripheral/cranial neuropathy
Cerebritis/acute confusional state
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14
Q

What levels must be met to fit the criteria for leucopenia?

A

WCC<4 at least once
OR
Lymphopenia <1 at least once

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15
Q

What levels must be met to fit the criteria for thrombocytopenia?

A

platelets <100

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16
Q

What are non-specific features of SLE?

A
Malaise
myalgia
fever
fatigue
lymphadenopathy
weight loss
nailfold infarcts
non-infective endocarditis
Raynauds
17
Q

How would you monitor activity of SLE?

A
  1. Anti-dsDNA ab titres
  2. Complement: low C3/C4
  3. ESR
18
Q

What marker is usually normal in SLE that if raised would point ti another diagnosis and what would they be?

A

CRP

If raised consider infection, serositis or arthritis

19
Q

What are the general measures in managing SLE?

A
  • High factor sun cream
  • Hydroxychloroquine
  • Screen for comorbidities + med toxicity
20
Q

How would u manage skin flares?

A

trial topical steroids

21
Q

What would u give for maintenance therapy?

A
  • NSAIDs (unless renal disease)
  • Hydroxychloroquine
  • Steroid sparing agents: azathioprine, methotrexate, mycophenolate
  • Belimumab - add on for auto ab disease w high activity
22
Q

What is the rx of mild flare?S

A

hydroxychloroquine
or
high dose steroids

23
Q

What is the rx of mod flares?

A

DMARDs
or
mycophenolate

24
Q

When does SLE become severe and what is the rx of severe flares?

A
If organ or life threatening e.g. haemolytic anaemia, nephritis, severe pericarditis or CNS disease 
urgent high dose steroids 
mycophenolate 
rituximab 
cyclophosphamide
25
Q

What drugs cause drug induced lupus

A
isoniazid
hydralazine
procainamide
quinidine
chlorpromazine
mincocycline 
phenytoin
anti-TNFs
26
Q

What are the main signs in drug induced lupus?

A

skin and lung