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Musculoskeletal > Systemic Lupus Erythematosus > Flashcards

Systemic Lupus Erythematosus Flashcards

1
Q

SLE clinical features

A 
General features
-Malaise
-Fatigue
-Fever
-Weight loss
-Lymphadenopathy
Specific features
-Butterfly rash
-Alopecia
-Arthralgia
-Raynaud's phenomenon
Other features
-Inflammation of the kidney, CNS, heart or lungs 
-Accelerated atherosclerosis
-Vasculitis
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2
Q

SLE treatment (mild disease)

A

Mild disease (joint +/- skin involvement)

  • Paracetamol +/- NSAIDs
  • Hydroxychloroquine
  • Topical corticosteroids
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3
Q

SLE treatment (moderate disease)

A

Moderate disease (inflammation of other organs eg: pleuritis, pericarditis, mild nephritis)

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4
Q

SLE treatment (severe disease)

A

Severe disease (severe inflammation in vital organs eg: severe nephritis, CNS disease, pulmonary disease, cardiac involvement, AIHA, thrombocytopaenia, TTP)

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5
Q

SLE epidemiology

A

/

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6
Q

ACR criteria for SLE

A

4 or more of:

  • malar rash
  • discoid rash
  • photosensitivity
  • oral ulcers
  • arthritis
  • serositis (pleuritis or pericarditis)
  • renal disorder (eg: proteinuria >0.5g/24h)
  • neurological disorder (eg: seizures/psychosis)
  • haematological disorder
  • immunologic disorder (eg: anti-dsDNA Abs)
  • antinuclear antibody in raised titre
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7
Q

SLE pathogenesis: autoantibody formation

A
  • abnormal clearance of apoptotic cell material
  • dendritic cell uptake of autoantigens and B cell activation
  • B cell Ig class switch and affinity mutation
  • IgG autoantibodies
  • immune complexes
  • complement activation and cytokine generation
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8
Q

SLE pathophysiology: autoantibody formation

A

/

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9
Q

Assessing SLE disease severity

A
  • identify pattern of organ involvement
  • monitor function of affected organs
  • identify pattern of autoantibodies expressed
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10
Q

SLE laboratory tests

A
  • Antinuclear antibodies
  • Anti-dsDNA and Sm
  • Anti-Ro and/or La
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11
Q

SLE prognosis and survival

A

15 year survival:

  • no nephritis=85%
  • nephritis=60%

-prognosis worse if black, male and of low socio-economic status

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12
Q

SLE genetic associations

A
  • multiple genes implicated=Fc receptors, IRF5, CTLA4, MHC class II HLA genes
  • complement deficiency=C1q and C3
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13
Q

SLE lab tests

A

ANTINUCLEAR ANTIBODIES IN SERUM

-ANAs relatively non-specific, pattern important

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14
Q

Antinuclear antibodies

A

Homogenous=Abs to DNA

  • Speckled=Abs to Ro, La, Sm, RNP
  • Nucleolar= topoisomerase-scleroderma
  • Centromere=limited cutaneous scleroderma
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15
Q

Lab test of anti-dsDNA and Sm

A

-more specific but less sensitive

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16
Q

Lab test of anti-Ro and/or La

A
  • common in subacute cutaneous LE

- neonatal lupus syndrome and Sjogren’s syndrome

17
Q

SLE lab tests

A
  • increased complement consumption
  • anti-cardiolipin antibodies
  • lupus anticoagulant
  • beta1 glycoprotein
18
Q

Haematology of SLE

A
  • lymphopenia
  • normochromic anaemia
  • leukopenia
  • AIHA (acquired autoimmune haemolytic anaemia)
  • thrombocytopenia
19
Q

Renal tests of SLE

A
  • proteinuria, haematuria

- active urinary sediment

20
Q

Assessing SLE disease severity

A

1) identify pattern of organ involvement
2) Monitor function of affected organs
- renal=BP, U and E, urine sediment and prot:crea ratio
- lungs/CVS=lung function, echocardiography
- skin, haematology, eyes
3) Identify pattern of autoantibodies expressed
- anti-dsDNA, anti-Sm=renal disease
- anti-cardiolipin antibodies

21
Q

Try and pre-empt severe attacks

A 
CLINICAL FEATURES
-weight loss, fatigue, malaise, hair loss
-alopecia
-rash
LABORATORY MARKERS
-ESR
-increased complement consumption
-increased anti-dsDNA
-other Abs eg: ANA and CRP poor indicators
22
Q

Azathioprine

A
  • effective steroid-sparing agent
  • moderate to severe disease give 2.5mg/kg/day
  • 20% of patients get neutropenia (3/1000 severe bone marrow suppression)
  • regular FBC and biochemistry monitoring=negative effect on liver
  • favoured as safe during pregnancy=SLE affects young women during window of fertilisation
23
Q

Cyclophosphamide

A
  • used in severe organ involvement (iv pulsed or oral prescription)
  • side effects=BM suppression, infertility, cystitis (acrolein), risk of infection
24
Q

Mycophenolate mofetil

A
  • reversible inhibitor of inosine monophosphate dehydrogenase
  • rate-limiting enzyme in de novo purine synthesis
  • lymphocytes=dependent upon de novo purine synthesis
25
Q

Rituximab

A
  • anti-CD20 mAb therapy
  • leads to depletion of B cells
  • effective in lupus nephritis

Musculoskeletal (8 decks)

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