5. Lupus Flashcards

1
Q

What does SLE stand for?

A

Systemic Lupus Erythematosus

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2
Q

What is Lupus?

A

Illness where the body’s attacked by its own immune system

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3
Q

Pathogenesis of Lupus? 3 points

A
  • Bodys immune system malfunctions and produces autoantibodies
  • Autoantibodies attack the bodies own tissue and cells
  • Leads to autoantibody-mediated inflammation and cell damage and destruction can affect: blood cells, skin, joints, kidney, lungs, nervous system and other organs
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4
Q

What do we mean my multisystem? how is lupus a multi system disorder?

A
  • Affects many different systems in the body

- photosensitive skin eruptions, pneumonitis, myocarditis, nephritis, CNS

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5
Q

What is the 10 year survival rate for Lupus?

A
  • 90% - very severe
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6
Q

Pathophysiology - What happens to the B-cells?

A

Hyperctivated B-cells

  • Number of activated B cells producing Ig increase in peripheral blood
  • Lupus B-cell are more prone to polyclonal activation by specific antigens
  • Raised IL-6 and IL-10 conc may promote B-Cell hyperactivity
  • B-Cell responses to activating signals are abnormal

B-Cell responses to activating signals are abnormal

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7
Q

Pathophysiology - What happens to the T-cells?

A
  • Number of activated T-Cells increase in peripheral blood
  • Abnormal early events of T-Cell activation
  • T-Cell function skewed towards B-cells help with Ig production
  • Lupus T-Cells produce little IL-2 on stimulation
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8
Q

Pathophysiology - What happens to abnormal phagocytic functions

A
  • Phagocytic cells cannot bind or process immune complexes efficiently
  • Phagocytosis of apoptotic cells impaired
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9
Q

Pathophysiology - What happens to abnormal immunoregulation

A
  • Defective clearance of immune cells and apoptotic materials because of defects
  • Suppressive activity of suppressor T-cells and NK cells on activated of TandB-cell network is inadequate
  • Idiotypic control of antibody production is dysregulated
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10
Q

How can lupus link to sex hormones?

A

Relative risk increases for women on HRT or OC

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11
Q

What is the main effects oestrogen has on cells?

A

B-cells - increase b-cell differentiation and in vitro Ig production
T-Cells- decreased proliferative response to antigens and mitogens - dec IL-2R expression and IL-2 production in activated peripheral blood T-cells
Monocytes - inc IL-10 production and cNOS release

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12
Q

What signs and symptoms would you see in a patient with lupus?

A

Fatigue
Aches and pains - no joint swelling
Dermatological signs

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13
Q

What are the dermatological effects of Lupus?

A
  • Maylar Rash (butterfly)
  • Discoid Lupus
  • Thick, red scaly patches on the skin
  • Alopecia
  • Ulcers - mouth, nasal and vaginal
  • Skin lesions
  • UV sensitivity
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14
Q

How do we treat discoid lupus?

A

Steroid cream

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15
Q

What is the main difference between joint pain SLE and RA?

A
  • SLE is all lover rather than just some joints

- SLE is less disabling

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16
Q

How do we investigate Lupus?

A
  • CRP is normal, ESR is raised
  • ANA occur in nearly all cases
  • double stranded DNA - specific for SLE
  • Lupus band test - shows IgG and IgM deposited at demo-epidermal junction.
17
Q

Which other system are affected? and how?

A
  • Cardiac/pilmonary - Pericarditis, Myocarditis and Endocarditis, Pulmonary inflammation or pleurisy
  • Renal - dec GFR (leads to renal failure), nephritis, haematuria, proteinuria
    Neurological - Headaches, depression, seizures, cognitive dysfunction, anxiety
    Non specific - Lethargy, weight loss, fever, extreme malaise
18
Q

Name 4 diagnostic criteria for lupus

A
  • Maylar rash
  • Discoid rash
  • Photosensitivity
  • Oral Ulcers
  • Arthritis
19
Q

ANA testing for lupus

A

Titer >1.40

  • Complete blood count
  • Serum creatine level
20
Q

Name 5 types of drugs that can induce lupus

What are the two forms of antibody caused by these?

A
  • Antihypertensives
  • Antimicrobials
  • Anticonvulsants
  • immunosuppressants
  • antithyroid drugs
  • Positive anti-histone antibody - procainamide, hydralazine, minocycline
  • Positive anti Ro antibody - Hydrochlorothiazide, CCB
21
Q

Name 5 types of Drugs used for treatment in SLE and what they’re used for

A
  • NSAIDs - musculoskeletal manifestations
  • Steroids - prednisolone - Maylar rash
  • Anti-malarial - Chloroquine - 150mg max daily dose - fatigue and rashes
  • Cytotoxic drugs - Azothiaprine - Reduces rate renal failure
  • Immunosuppresant - Mycophenolate
22
Q

Name a biological drug for lupus

A

Belimumab - Blocks BLyS(B-lymphocyte stimulator) - responsible for B cell apoptosis

23
Q

What are the main causes of death in SLE patients?

A
  • cardiovascular
  • CNS lupus
  • Infection
  • Renal failure