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MSK > Lupus, Systemic Sclerosis & Sjogren's > Flashcards

Lupus, Systemic Sclerosis & Sjogren's Flashcards

1
Q

Define connective tissue diseases

A

Conditions associated with spontaneous overactivity of the immune system

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2
Q

Describe the epidemiology of SLE

A

9:1 female to male ratio commonly presents in childbearing years, involves genetic, hormonal, immunological and environmental factors

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3
Q

How are auto-antibodies produced in SLE?

A
  1. Loss of immune regulation
  2. Increased and defective apoptosis
  3. Necrotic cells release nuclear material which act as auto-antigens
  4. Immune system cannot distinguish between ‘normal’ and ‘unwanted’ cells
  5. B and T cells are stimulated
  6. Auto-antibodies are produced
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4
Q

After SLE auto-antibodies are produced what happens?

A
  1. Complexes of antigens and auto-antibodies form and circulate and get deposited in the wall of blood vessels
  2. This activates complement which attracts leucocytes and releases cytokines
  3. Cytokine release causes inflammation
  4. Necrosis and scarring occurs
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5
Q

Is complement high or low during active SLE?

A

Low due to complex formation

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6
Q

Where in the body are clinical features of SLE found?

A 
Cutaneous 
Joints 
Neurological 
Serositis 
Haematological 
Renal
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7
Q

What are the cutaneous clinical features of SLE?

A

Malar rash (butterfly), oral ulcers, discoid rash

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8
Q

What joint symptoms are present in SLE?

A

Synovitis, tenderness, >2 joints, >30mins morning stiffness

- Jaccouds arthropathy

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9
Q

What neurological symptoms can present in SLE?

A

Delirium, psychosis, seizures

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10
Q

Define serositis

A

Inflammation of serous tissues (heart/lungs)

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11
Q

What haematological symptoms can SLE present with?

A

Leukopenia, thrombocytopenia, haemolytic anaemia

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12
Q

What renal symptoms can be present in SLE?

A

Proteinuria, nephritis, red cell casts

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13
Q

What is the first test done in SLE?

A

Urinalysis

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14
Q

What antibodies can be present in SLE?

A 
Anti-nuclear antibody 
Anti-double stranded DNA antibody 
Anti-phospholipid antibodies 
Anti-Ro 
Anti-Sm
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15
Q

Which antibody is most specific for SLE?

A

Anti-double stranded DNA

Anti-Sm (rarely present)

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16
Q

Name three anti-phospholipid antibodies

A

Lupus anticoagulant
Anti-cardiolipin antibodies
Anti -beta2glycoprotein antibodies

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17
Q

What is the major risk of anti-phospholipid antibodies in women?

A

Associated with venous and arterial thrombosis and recurrent miscarriage

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18
Q

What are the risks of Anti-Ro?

A

Associated with neonatal heart block and lupus

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19
Q

What should all patients with lupus be given?

A

Sun protection

Hydroxychloroquine

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20
Q

Name the scoring system used to monitor SLE activity

A

SLEDAI

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21
Q

Define mild SLE

A

Mild arthritis/rash SLEDAI = 6

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22
Q

Define moderate SLE

A

RA-like arthritis, cutaneous vasculitis, SLEDAI 7-12

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23
Q

Define severe SLE

A

major organ threatening disease SLEDAI >12

24
Q

What is meant by refractory disease?

A

Failure to attain clinical remission

25
Q

What is remission in terms of SLE?

A

SLEDAI = 0

HCQ but no steroids

26
Q

What should all patients with anti-phosolipid antibodies be prescribed?

A

Antiplatelets/anticoagulants

27
Q

What is second line for all severities of SLE after HCQ?

A

Glucocorticoids - route of administration/dose will vary depending on severity

28
Q

In mild refractory or 1st line moderate disease after HCQ and GC what is the next possible treatment option?

A

Methotrexate/Azathioprine

29
Q

What is given in moderate refractory SLE after HCQ and GC?

A

Belimumab

30
Q

What is given in moderate disease fourth line?

A

Calcineurin Inhibitors

31
Q

If calcineurin inhibitors don’t work what is next line in moderate disease?

A

Mycophenolate Mofetil

32
Q

What are the treatment options in severe disease after HCQ and GC?

A

Mycophenolate mofetil (1st)
Cyclophosphamide (1st and refractory)
Rituximab (refractory)

33
Q

What is Sjogren’s syndrome?

A

Chronic inflammatory autoimmune disorder with lymphocytic infiltration and fibrosis of exocrine glands

34
Q

What are the symptoms of sjogren’s?

A 
Dry; eyes, mouth, throat, vagina
Parotid gland enlargement 
Joint pain
Fatigue 
Unexplained dental caries
35
Q

What causes secondary sjogren’s?

A

Rheumatoid arthritis

SLE

36
Q

How is sjogren’s diagnosed?

A
  • Schimers test for conjunctival dryness
  • IgG and ESR
  • Anti-Ro/Anti-La antibodies
37
Q

What are patients with sjogren’s at increased risk of?

A

Lymphoma

38
Q

How is sjogren’s treated?

A

Symptomatic treatment
NSAIDs
Hydroxychloroquine for arthralgia
Immunosuppressants in severe disease

39
Q

What is systemic sclerosis?

A

multisystem autoimmune disease

  • vasculopathy
  • autoimmunity
  • fibrosis
40
Q

What are the two types of systemic sclerosis?

A

Diffuse

Limited

41
Q

Describe diffuse systemic sclerosis

A 
Whole body affected
Interstitial lung disease 
Pulmonary hypertension (SOB)
Renal crisis 
Extremities above and below elbows and knees
42
Q

Describe limited systemic sclerosis

A 
Calcinosis
Raynaud's
Oesophageal dysmotility 
Sclerodactyly
Telangiectasia 
Below elbows and knees + face
43
Q

What antibody is associated with each type of systemic sclerosis?

A

Diffuse - Anti-topoisonmersase and Anti-Scl70

Limited - Anti-centromere

44
Q

How will systemic sclerosis present in the face?

A

small mouth, beaked nose, tight skin, telangiectasia

45
Q

What GI symptoms can systemic sclerosis cause?

A

Dysphagia, GORD, malabsorption, fluctuation in bowel habits, small intestinal bacterial overgrowth, faecal incontinence

46
Q

What cardio/resp systems can systemic sclerosis cause?

A

ILD, pulmonary hypertension, myocardial disease

47
Q

Describe the renal problems caused by systemic sclerosis?

A

Scleroderma renal crisis and renal dysfunction

48
Q

How does Raynaud’s present?

A

Blanching
Hyperaemia
Acrocyanosis

49
Q

What are the risk factors for renal crisis?

A
  • anti-RNA polymerase antibody

- steroids

50
Q

How does renal crisis present?

A

Uncontrolled hypertension, proteinuria, worsening renal function

51
Q

How is renal crisis treated?

A

ACE inhibitors

52
Q

What is the treatment for pulmonary fibrosis?

A
  • mycophenolate mofetil
  • cyclophosphamide
  • rituximab
  • nitedanib (anti-fibrotic)
53
Q

How is skin fibrosis treated?

A

Methotrexate

Mycophenolate mofetil

54
Q

What is the treatment for vasculopathy?

A 
Calcium channel blockers 
Prostacyclin infusion (iloprost)
Endothelin receptor antagonist (bosentan)
55
Q

What can pulmonary hypertension lead to?

A

Right heart failure - SOB on exertion

56
Q

How is pulmonary hypertension treated?

A

PDE-5 Inhibitor
Oxygen
Endothelin receptor antagonist

MSK (22 decks)

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