Systemic Lupus Erythema (SLE) Flashcards

1
Q

what is it?

A

-Chronic AI disease

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2
Q

pathogenesis (proposed)

A

-Defect in apoptosis causes increased cell death

-Defective clearance of apoptotic cell debris causes persistence of antigen and immune complex production

-Circulating immune complexes with antigens in various tissues

  • in small blood vessels - complement activation and inflammation - basement membranes of skin and kidneys
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3
Q

who gets it?

A

black»white though low prevalence in Africa
90% cases in women normally of child bearing age
Onset after puberty - 20s-30s

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4
Q

what is the prognosis?

A

currently 10yr survival exceeds 90%
Previously mortality was due to the disease itself now mortality is a result of side effects of medication - infections or CV events

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5
Q

constitutional presentation

A
  • Fever
  • Fatigue
  • Weight loss
  • Pulmonary embolism
  • Pulmonary hypertension
  • Interstitial lung disease
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6
Q

musculoskeletal presentation

A
  • Arthralgia
    -Myalgia
    -Inflammatory arthritis
    -Arthopathy rarely erosive/ derforming
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7
Q

mucocutaneous presentation

A

-Malar rash
-Photosensitivity
-Discoid lupus/ Subacute cutaneous lupus: oral/nasal ulceration + Raynaud’s

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8
Q

renal presentation

A

lupus nephritis

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9
Q

respiratory presentation

A
  • pleurisy
  • pleural effusion
  • pneumonitis
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10
Q

haematological presentation

A
  • leukopenia
  • lymphopenia
  • anaemia
  • thrombocytopenia
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11
Q

neuropsychiatric presentation

A

seizures
- psychosis
- headache
- aseptic meningitis

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12
Q

cardiac presentation

A
  • pericarditis
  • pericardial effusion
  • pulmonary hypertension
  • sterile endocarditis
  • accelerated ischaemic heart disease
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13
Q

Gastrointestinal presentation

A
  • autoimmune hepatitis
  • pancreatitis
  • mesenteric vasculitis
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14
Q

what are the investigations?

A

no one diagnostic test

  • FBC may show - anaemia - leucopenia - thromobocytopenia
    -Urinalysis - look for glomerulonephritis

-Imaging - organ involvement (CT, MRI, echo)

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15
Q

antibodies to test

A
  • ANA - high sensitivity (>95% of patients)
  • Anti-dsDNA
  • Anti-SM - low sensitivity -
  • specific c3/4 - low sensitivity when disease is active
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16
Q

how is it diagnosed?

A

Clinical findings

SLICC classification criteria for systemic lupus erythematous - if >4 criteria (1 clinical and 1 lab) or a biopsy-proven lupus nephritis with positive ANA or anti-DNA if you diagnose, check glomerulonephritis

17
Q

how is it managed?

A
  • Skin disease & arthralgia: hydroxychloroquine, topical steroids, NSAIDs
  • Inflammatory arthritis / evidence of organ involvement (pericardial disease, ILD) –

o immunosuppression with azathioprine / mycophenolate mofetil
o corticosteroids at moderate doses for short periods

severe organ disease (lupus nephritis / CNS lupus) – IV steroids, cyclophosphamideunresponsive cases – IV immunoglobulin, rituximab

18
Q

how is it monitored?

A

investigated according to symptoms

Check regularly (vary with disease activity and warns of a flare) - anti-dsDNA - c3/4

Levels urinalysis - blood or protein to check for glomerulonephritis evaluate and manage CV risk factors - major cause of mortality - BP - cholesterol