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MSK 2 > Lupus and related autoimmune connective tissue disease > Flashcards

Lupus and related autoimmune connective tissue disease Flashcards

1
Q

What are the autoimmune connective tissue disorders?

A

-Systemic lupus erythematosus
-Sjögren’s syndrome
-Systemic sclerosis (scleroderma)
-Autoimmune inflammatory muscle disease

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2
Q

Where is the site of ankylosing spondylitis?

A

Enthesis

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3
Q

What is systemic lupus erythematosus?

A

-Autoimmune disease involving disturbance in both innate and adaptive immune systems
-Multi site inflammation

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4
Q

What antibodies do you get in SLE?

A

Anti nuclear antibodies

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5
Q

What is arthralgia?

A

Aching joints

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6
Q

What is Raynaud’s phenomenon?

A

-Intermittent vasospasm of digits

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7
Q

What is the progression of Raynaud’s phenomenon?

A

-White= vasospasm leads to blanching of digits (blood vessels contracted)
-Blue= cyanosis as static venous blood deoxygenates
-Red= reactive hyperaemia

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8
Q

What is hyperaemia?

A

Body adjusts blood flow to meet metabolic needs of different tissues

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9
Q

What are characteristics of autoimmune connective tissue disorders?

A
  1. Arthralgia and arthritis= typically non erosive
  2. Serum autoantibodies
  3. Raynaud’s phenomenon
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10
Q

What can severe Raynaud’s cause?

A

-Tissue ischaemia
-Ulcers
-Necrosis

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11
Q

What is the main risk factor for SLE?

A

Female

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12
Q

What is the specific clinical feature seen in SLE?

A

Malar rash= butterfly shaped rash (erythema that spares the nasolabial fold + photosensitive rash)

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13
Q

What is the significance of anti nuclear antibodies?

A

-Negative ANA rules out SLE
-Positive ANA does not mean SLE

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14
Q

What other autoantibodies can SLE patients get?

A

Antiphospholipid antibodies

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15
Q

What are antiphospholipid antibodies associated with an increased risk of?

A

-Thrombosis
-Pregnancy loss (miscarriage)

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16
Q

When is anti phospholipid antibody syndrome diagnosed?

A

-Antiphospholipid antibodies
-Clinical event

17
Q

What is primary anti phospholipid antibody syndrome?

A

Anti phospholipid antibody syndrome in the absence of SLE

18
Q

What are the main antibodies in SLE?

A

-Antinuclear antibodies (ANA)
-Anti double stranded DNA antibodies (anti dsDNA)
-Anti phospholipid antibodies
-Anti Sm antibodies

19
Q

Which antibodies are specific to SLE?

A

-Anti double stranded DNA antibodies
-Anti Sm antibodies

20
Q

What autoantibodies are present in rheumatoid arthritis?

A

-Rheumatoid factor
-Anti cyclic citrullinated peptide antibody

21
Q

What is the immunopathogenesis of SLE?

A

-Overactivity of type 1 interferon pathway
-Complement pathway abnormalities
-Autoreactive B and T cells

22
Q

What is the proposed mechanism of SLE?

A

Impaired clearance of cell debris port apoptosis so nuclear antigens are presented on cell surface

23
Q

What are key inflammatory markers for SLE?

A

-High ESR
-Normal CRP (unless infection)

24
Q

What are key renal investigations for SLE?

A

-Urine protein
-Creatinine
-Kidney biopsy

25
Q

What is the typical presentation of an unwell patient with active lupus?

A

-Low complement C3 and C4 levels
-High anti dsDNA antibodies

26
Q

What are the aims of management of SLE?

A

-Remission/low disease activity and prevention of flares
-Balancing controlling disease vs iatrogenic harm

27
Q

What are the main side effects of NB steroids?

A

-Infection
-Osteoporosis
-Avascular necrosis

28
Q

Where is the most common site of avascular necrosis?

A

Hips

29
Q

What does specific choice of treater depend on?

A

-Disease severity
-Organ manifestations

30
Q

What is the treatment for mild SLE?

A

Hydroxychloroquine

31
Q

What is the treatment for serious SLE?

A

Immunomodulatory agents

32
Q

What is the treatment for persistently active SLE?

A

B cell targeted therapies

33
Q

What is the treatment for severe/life threatening SLE?

A

IV steroids AND IV cyclophosphamide

34
Q

What should patients with SLE + antiphospholipid antibody syndrome be treated with?

A

Anticoagulation medication (eg- warfarin)

MSK 2 (5 decks)

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