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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
What is remission in terms of SLE?
SLEDAI = 0 | HCQ but no steroids
26
What should all patients with anti-phosolipid antibodies be prescribed?
Antiplatelets/anticoagulants
27
What is second line for all severities of SLE after HCQ?
Glucocorticoids - route of administration/dose will vary depending on severity
28
In mild refractory or 1st line moderate disease after HCQ and GC what is the next possible treatment option?
Methotrexate/Azathioprine
29
What is given in moderate refractory SLE after HCQ and GC?
Belimumab
30
What is given in moderate disease fourth line?
Calcineurin Inhibitors
31
If calcineurin inhibitors don't work what is next line in moderate disease?
Mycophenolate Mofetil
32
What are the treatment options in severe disease after HCQ and GC?
Mycophenolate mofetil (1st) Cyclophosphamide (1st and refractory) Rituximab (refractory)
33
What is Sjogren's syndrome?
Chronic inflammatory autoimmune disorder with lymphocytic infiltration and fibrosis of exocrine glands
34
What are the symptoms of sjogren's?
``` Dry; eyes, mouth, throat, vagina Parotid gland enlargement Joint pain Fatigue Unexplained dental caries ```
35
What causes secondary sjogren's?
Rheumatoid arthritis | SLE
36
How is sjogren's diagnosed?
- Schimers test for conjunctival dryness - IgG and ESR - Anti-Ro/Anti-La antibodies
37
What are patients with sjogren's at increased risk of?
Lymphoma
38
How is sjogren's treated?
Symptomatic treatment NSAIDs Hydroxychloroquine for arthralgia Immunosuppressants in severe disease
39
What is systemic sclerosis?
multisystem autoimmune disease - vasculopathy - autoimmunity - fibrosis
40
What are the two types of systemic sclerosis?
Diffuse | Limited
41
Describe diffuse systemic sclerosis
``` Whole body affected Interstitial lung disease Pulmonary hypertension (SOB) Renal crisis Extremities above and below elbows and knees ```
42
Describe limited systemic sclerosis
``` Calcinosis Raynaud's Oesophageal dysmotility Sclerodactyly Telangiectasia Below elbows and knees + face ```
43
What antibody is associated with each type of systemic sclerosis?
Diffuse - Anti-topoisonmersase and Anti-Scl70 | Limited - Anti-centromere
44
How will systemic sclerosis present in the face?
small mouth, beaked nose, tight skin, telangiectasia
45
What GI symptoms can systemic sclerosis cause?
Dysphagia, GORD, malabsorption, fluctuation in bowel habits, small intestinal bacterial overgrowth, faecal incontinence
46
What cardio/resp systems can systemic sclerosis cause?
ILD, pulmonary hypertension, myocardial disease
47
Describe the renal problems caused by systemic sclerosis?
Scleroderma renal crisis and renal dysfunction
48
How does Raynaud's present?
Blanching Hyperaemia Acrocyanosis
49
What are the risk factors for renal crisis?
- anti-RNA polymerase antibody | - steroids
50
How does renal crisis present?
Uncontrolled hypertension, proteinuria, worsening renal function
51
How is renal crisis treated?
ACE inhibitors
52
What is the treatment for pulmonary fibrosis?
- mycophenolate mofetil - cyclophosphamide - rituximab - nitedanib (anti-fibrotic)
53
How is skin fibrosis treated?
Methotrexate | Mycophenolate mofetil
54
What is the treatment for vasculopathy?
``` Calcium channel blockers Prostacyclin infusion (iloprost) Endothelin receptor antagonist (bosentan) ```
55
What can pulmonary hypertension lead to?
Right heart failure - SOB on exertion
56
How is pulmonary hypertension treated?
PDE-5 Inhibitor Oxygen Endothelin receptor antagonist

MSK (22 decks)

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