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Year 2 - Muscoloskeletal > Rheum - SLE > Flashcards

Rheum - SLE Flashcards

1
Q

SLE?

A

Systemic Lupus Erythematosus

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2
Q

What is SLE?

A

It is a rare disease

• a part of a family of overlapping chronic AI diseases including - RA, SLE, systemic sclerosis, polymyositis

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3
Q

Background info surrounding SLE?

A

o M: F = 1: 9 and presents at 15-40 years of age.
o More prevalent in Afro-Caribbean’s, Asians and Chinese people – prevalence = 4-280/100,000

o Principally affects the joints and skin – also affects lungs, kidneys and has unique haematology

Genetic associations:
 Multiple genes:
• Fc receptors, IRF5, CTLA4, MHC class 2 HLA genes.
 Complement deficiency – C1q and C3.

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4
Q

How does SLE present?

A 
Malaise
Fatigue
Fever
Weight loss
Lymphadenopathy (lymphomas excluded)
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5
Q

What is special features associated with SLE?

A

Butterfly rash! (on face!!)

Alopecia
Arthralgia
Raynaud’s phenomenon

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6
Q

Other features associated with SLE?

A

Inflammation of the kidney, CNS, heart, lungs
Accelerated atherosclerosis
Vasculitis

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7
Q

What is the ACR criteria for diagnosis of SLE?

A

4+ of the 11 criteria

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8
Q

Pathogenesis of SLE?

A

Pathogenesis NOT fully understood

• but tend to have OVERACTIVE IS - particularly their humoral immunity

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9
Q

What are the 2 things associated with the pathogenesis of SLE?

A
  1. Apoptotic defects

2. B-Cell hyperactivity

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10
Q

Explain the effect of apoptotic defects on SLE

A

There is impaired clearance associated with inflammation of apoptotic bodies in SLE
• the apoptotic bodies linger in the body
and
• expose nuclear antigens on their surface which generate auto-antibodies

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11
Q

Explain the effect of B-Cell Hyperactivity on SLE

A

Overactive b-cells are exposed to the auto-antigens and the plasma cells begin to produce auto-ABs which form immune complexes
• this deposits in tissues (mainly kidneys/skin) which then activates complement in the tissues

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12
Q

Describe the process of auto-Ab formation in SLE

A
  1. Abnormal clearance of apoptotic bodies
  2. Dendrites take up autoantigens –> b-cell activation
  3. B-cell Ig-class switch and affinity maturation
  4. IgG-autoantibodies
  5. Immune complexes
  6. Complement activation via classical pathway
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13
Q

How can SLE be diagnosed in the lab?

A

First, send a serum to check for anti-nuclear antibodies.
• This is NOT diagnostic of SLE

Serum is combined with cells and if ANAs are present, they will bind to the cells’ nuclear antigens
• a fluorescently labelled AB is added that binds to ANAs and you observe the pattern of attachment

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14
Q

What tests can be ordered in the lab to assess the activity of the disease?

A
  • ANA
  • Anti-dsDNA and Sm
  • Anti-Ro and/or La
  • Other tests
  • Haematology
  • Renal

(onenote!!)

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15
Q

Why will INCREASED complement consumption be diagnostic of SLE?

A

Patients with ACTIVE lupus will have
• more complement bound to immune complexes

and so the blood complement will be LOW in active lupus.

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16
Q

What else do SLE patients present with?

A

SLE patients will present with AI haemolytic anaemia

• ABs bind to RBC surfaces and they are extravascularly removed in the spleen

17
Q

What is lupus correlated with?

A

LOW counts of everything!

• i.e. -paenia

18
Q

How do you assess the severity of SLE?

A
  1. Identify pattern of organ involvement
  2. Monitor function of affected organs:
    a. Renal – BP, U&E, urine sediment GFR
    b. Lungs/CVS – lung function tests, echocardiography
  3. Identify the patterns of auto-Abs expressed
    a. Anti-dsDNA, anti-Sm – high levels of these ABs can predict renal disease
    b. Anti-cardiolipin antibodies.
19
Q

How can you try and pre-empty severe attacks associated with SLE?

A

Picking up on symptoms early can result in less damage to organ system
• Wt loss, fatigue, malaise, hair loss
• Alopecia
• Rash

ESR will RISE is the disease is becoming more severe!
Other lab markers include
 • increased complement consumption
 • increased anti-dsDNA
 • other Abs e.g. ANA and CRP
20
Q

How is SLE treated iniitally?

A

The disease is split into 3 groups based on severity:

• MILD - no sign of organ involvement

• MODERATE - inflammation of other organs
e.g. pleuritis, pericarditis, mild nephritis

• SEVERE - severe inflammation in vital organs
e.g. severe nephritis, CNS/pulmonary disease etc

21
Q

How do you treat MILD SLE?

A

A. Paracetamol +/- NSAID
- monitor renal function

B. Hydroxychloroquine

  • arthropathy
  • cutaneous manifestations
  • mild disease activity

C. Topical corticosteroids

22
Q

What is an indicator that the SLE has now become MODERATE?

A

Hydroxychloroquine/NSAID STOP working

23
Q

How do you treat MODERATE SLE?

A

Switch from topical –> ORAL glucocorticoids
• high dose initially to suppress disease activity AND THEN titrate down
• BUT very toxic so can give with steroid-sparing agents to lower dose required

24
Q

How do you treat SEVERE SLE?

A

Azathioprine - a steroid-sparing drug
• comes with a risk of neutropenia & bone marrow suppression
• SO requires blood monitoring

Cyclophosphamide
• only used in SEVERE organ involvement
• side effect is INFERTILITY

25
Q

What are some novel treatment of SEVERE SLE?

A

Mycophenolate mofetil
&
Rituximab

These drugs are as effective in inducing remission as cyclophosphamide
• they are teratogenic (so shouldn’t try to conceive) BUT has no effect on fertility
AND
• inhibit lymphocyte proliferation!!

26
Q

Prognosis and survival of patients with SLE?

A

15-year survival:
o No nephritis = 85%
o Nephritis = 60%

Prognosis is worse if – black, male, low socio-economic background/status

There is an early peak in mortality in SLE and then a late peak
o Early death – renal failure, CNS disease, infection.
o Late death – MI, stroke.

27
Q

What will be seen in a blood film to indicate SLE?

A
  • Shistocytes
  • Spherocytes
  • Tear drop cells
  • FEW - leukocytes & platelets

(onenote!!)

28
Q

What will be seen in a renal biopsy to indicate SLE?

A

• Hyper-cellular
- MORE cells than normal

• Mesangial proliferation

• Crescent development
- inflammatory cells that have migrated into the glomerulus

• Rapidly proliferating glomeulonephritis

(onenote!!)

Year 2 - Muscoloskeletal (10 decks)

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