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MSK- Rheumatology > Connective Tissue Diseases- APS, Sjogren's, Systemic Sclerosis > Flashcards

Connective Tissue Diseases- APS, Sjogren's, Systemic Sclerosis Flashcards

1
Q

What characterises APS?

A

Venous or arterial thrombosis and/or adverse pregnancy outcomes in the presence of anti-phospholipid antibodies

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2
Q

What are anti-phospholipid antibodies antibodies against?

A

Phospholipid binding proteins

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3
Q

What can recurrent emboli/thrombosis lead to?

A

Life-threatening pulmonary hypertension

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4
Q

What are the adverse pregnancy outcomes that can occur in anti-phospholipid syndrome?

A
  • Late spontaneous foetal loss (2nd/3rd trimester usually though can occur at any time) - Premature birth due to severe pre-eclampsia or placental insufficiency - Recurrent early foetal loss < 10 weeks
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5
Q

Is APS always associated with another condition?

A

Not necessarily, it can just exist on its own

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6
Q

What other conditions is APS often associated with?

A

SLE, or another rheumatic/autoimmune disease

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7
Q

What CV effects can APS have?

A

Increased frequency of stroke/MI, especially in younger patients

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8
Q

What may strokes occur secondary to in APS?

A

In situ thrombosis/embolism that originates from the valvular lesions of Libman-Sacks (sterile) endocarditis

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9
Q

What is catastrophic APS?

A

A rare, serious and often fatal manifestation of APS characterised by multi-organ infarctions over a period of days-weeks

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10
Q

What is the mortality rate of catastrophic APS?

A

50%

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11
Q

What haematological change does APS cause?

A

Thrombocytopenia or prolonged activated partial thromboplastin time (APTT)

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12
Q

What is the name of this rash, associated with APS?

A

Livedo Reticularis

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13
Q

What neurological symptom occurs with APS?

A

Migraine

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14
Q

What are the three main anti-phospholipid antibodies?

A
  • Anti-cardiolipin antibody
  • Lupus anticoagulant
  • Anti-beta2 glycoprotein
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15
Q

What type of antibody can anti-cardiolipin antibody/anti-beta2 glycoprotein be?

A

IgM or IgG

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16
Q

What is diagnostic of APS? (in terms of antibodies)

A

One or more of the antibodies must be positive on 2 occasions at least 12 weeks apart

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17
Q

What is the mainstay of treatment for APS?

A

Anticoagulation

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18
Q

What medication is used for APS? When should this be changed?

A

Warfarin, this is changed to LMWH during pregnancy (warfarin is teratogenic)

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19
Q

When is anticoagulation required in APS?

A

Only for patients who have had an episode of thrombosis

(DO NOT give unless this has happened, even if the patient is antibody positive)

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20
Q

What DMARD is used in APS?

A

Hydroxychloroquine

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21
Q

What is this clinical sign? When is it seen?

A

Bilateral parotid gland swelling- Sjogren’s Syndrome

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22
Q

What type of disease is Sjogren’s Syndrome?

A

Chronic autoimmune inflammatory disorder

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23
Q

What characterises Sjogren’s Syndrome?

A

Lymphocytic infiltrates in exocrine organs causing diminished lacrimal and salivary gland function resulting in dry eyes/mouth

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24
Q

Sjogren’s can be primary or secondary. What are two conditions it can be secondary to?

A

RA and SLE

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25
Who is Sjogren's most common in?
Women in their 50s/60s
26
Besides dry eyes, mouth and throat, what are some other clinical features of Sjogren's?
- Vaginal dryness - Parotid gland enlargement - Joint pains - Fatigue - Unexplained dental caries
27
What are two examples of further organ involvement of Sjogren's?
- Interstitial lung disease - Peripheral neuropathy
28
Sjogren's increases your risk of what cancer?
Lymphoma
29
What are the main auto-antibodies associated with Sjogren's?
Anti-Ro, Anti-La
30
Besides the antibodies, what three other things could be seen on a blood test for Sjogren's?
- Rheumatoid factor - High IgG - Raised inflammatory markers
31
Besides blood tests, what other investigations can be used for Sjogren's?
- Salivary gland ultrasound - Labial (salivary) gland biopsy
32
Four out of what six things is diagnostic of Sjogren's?
- Ocular signs - Ocular symptoms - Oral signs - Oral symptoms - Positive salivary gland biopsy - Positive anti-Ro/anti-La antibodies
33
What test is this? What does it detect?
Schirmer's Test for ocular dryness
34
What are the mainstay of treatment for Sjogren's?
- Artificial tear and saliva supplements - Cyclosporine eye drops
35
What DMARD drug can be used in Sjogren's? When is it used?
- Hydroxychloroquine - If there is arthralgia and fatigue
36
When should immunosuppressants be used in Sjogren's?
Only if there is major organ involvement
37
What does pilocarpine do? What is its side-effect?
Can stimulate salivary gland function Causes flushing
38
What are the red areas known as?
Telangectasia
39
What is this clinical sign known as?
Sclerodactyly
40
What does the autoimmunity of Systemic Sclerosis cause?
Vascular endothelial changes and fibrosis
41
What are the major characteristics of Systemic Sclerosis?
Raynaud's, fibrosis and subsequent atrophy of the skin and subcutaneous tissue
42
What are the major causes of death from systemic sclerosis?
Renal and lung involvement (especially pulmonary hypertension)
43
Excess of what contributes to skin and organ changes?
Collagen
44
What are the three stages of cutaneous involvement in systemic sclerosis?
1) Oedematous 2) Indurative 3) Atrophic
45
What are major cutaneous features of systemic sclerosis?
Centrally located skin sclerosis that affects the arms/face/neck
46
What are minor features of systemic sclerosis?
Sclerodactyly, atrophy of the fingertips, bilateral lung fibrosis
47
What makes a diagnosis of systemic sclerosis?
1 major and 2 minor criteria
48
What is calcinosis?
Subcutaneous deposits of calcium that may be seen in the digits in systemic sclerosis
49
What can gut involvement in systemic sclerosis lead to?
Dysphagia, malabsorption and bacterial overgrowth of the small bowel
50
What defines diffuse systemic sclerosis?
- Skin involvement proximal to the forearms and involving the torso - Rapid skin changes - Early organ involvement
51
What is the main antibody associated with diffuse systemic sclerosis? What other 2 antibodies can be present?
- Anti-SCL-70 (main) - Anti-topoisomerase - Anti-RNA III Polymerase
52
What defines limited systemic sclerosis?
- Skin involvement only involving the forearms, hands, feet and face - Late organ involvement
53
What antibody is associated with limited systemic sclerosis?
Anti-centromere
54
What tests are performed as monitoring in patients with systemic sclerosis?
- Pulmonary function tests - ECHO - Renal function tests
55
What is used to treat the Raynaud's in systemic sclerosis?
Ca++ channel blockers and phosphodiesterase inhibitors
56
When is immunosuppression given in systemic sclerosis?
If there is advanced lung disease
57
What is used to treat pulmonary hypertension in systemic sclerosis?
Ca++ blockers and endothelin receptor antagonists
58
What drugs are used to treat GI symptoms in systemic sclerosis?
PPIs, H2 antagonists Antibiotics if there is bacterial overgrowth
59
What drug is used if there is renal involvement in systemic sclerosis?
ACE Inhibitors
60
Mixed connective tissue disease involves features of what diseases?
- SLE - Polymyositis - Systemic sclerosis
61
What is the biggest recognised complication of MCTD and how is it screened for?
Pulmonary hypertension- ECHO
62
What antibody is associated with mixed connective tissue disease?
Anti-RNP
63
What major organ involvement should be screened for in MCTD?
Interstital lung disease
64
What is the treatment for MCTD?
Depends on symptoms and whether or not there is major organ involvement

MSK- Rheumatology (12 decks)

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