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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
Q

Who is Sjogren’s most common in?

A

Women in their 50s/60s

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26
Q

Besides dry eyes, mouth and throat, what are some other clinical features of Sjogren’s?

A
  • Vaginal dryness
  • Parotid gland enlargement
  • Joint pains
  • Fatigue
  • Unexplained dental caries
27
Q

What are two examples of further organ involvement of Sjogren’s?

A
  • Interstitial lung disease
  • Peripheral neuropathy
28
Q

Sjogren’s increases your risk of what cancer?

29
Q

What are the main auto-antibodies associated with Sjogren’s?

A

Anti-Ro, Anti-La

30
Q

Besides the antibodies, what three other things could be seen on a blood test for Sjogren’s?

A
  • Rheumatoid factor
  • High IgG
  • Raised inflammatory markers
31
Q

Besides blood tests, what other investigations can be used for Sjogren’s?

A
  • Salivary gland ultrasound
  • Labial (salivary) gland biopsy
32
Q

Four out of what six things is diagnostic of Sjogren’s?

A
  • Ocular signs
  • Ocular symptoms
  • Oral signs
  • Oral symptoms
  • Positive salivary gland biopsy
  • Positive anti-Ro/anti-La antibodies
33
Q

What test is this? What does it detect?

A

Schirmer’s Test for ocular dryness

34
Q

What are the mainstay of treatment for Sjogren’s?

A
  • Artificial tear and saliva supplements
  • Cyclosporine eye drops
35
Q

What DMARD drug can be used in Sjogren’s? When is it used?

A
  • Hydroxychloroquine
  • If there is arthralgia and fatigue
36
Q

When should immunosuppressants be used in Sjogren’s?

A

Only if there is major organ involvement

37
Q

What does pilocarpine do? What is its side-effect?

A

Can stimulate salivary gland function

Causes flushing

38
Q

What are the red areas known as?

A

Telangectasia

39
Q

What is this clinical sign known as?

A

Sclerodactyly

40
Q

What does the autoimmunity of Systemic Sclerosis cause?

A

Vascular endothelial changes and fibrosis

41
Q

What are the major characteristics of Systemic Sclerosis?

A

Raynaud’s, fibrosis and subsequent atrophy of the skin and subcutaneous tissue

42
Q

What are the major causes of death from systemic sclerosis?

A

Renal and lung involvement (especially pulmonary hypertension)

43
Q

Excess of what contributes to skin and organ changes?

44
Q

What are the three stages of cutaneous involvement in systemic sclerosis?

A

1) Oedematous
2) Indurative
3) Atrophic

45
Q

What are major cutaneous features of systemic sclerosis?

A

Centrally located skin sclerosis that affects the arms/face/neck

46
Q

What are minor features of systemic sclerosis?

A

Sclerodactyly, atrophy of the fingertips, bilateral lung fibrosis

47
Q

What makes a diagnosis of systemic sclerosis?

A

1 major and 2 minor criteria

48
Q

What is calcinosis?

A

Subcutaneous deposits of calcium that may be seen in the digits in systemic sclerosis

49
Q

What can gut involvement in systemic sclerosis lead to?

A

Dysphagia, malabsorption and bacterial overgrowth of the small bowel

50
Q

What defines diffuse systemic sclerosis?

A
  • Skin involvement proximal to the forearms and involving the torso
  • Rapid skin changes
  • Early organ involvement
51
Q

What is the main antibody associated with diffuse systemic sclerosis?

What other 2 antibodies can be present?

A
  • Anti-SCL-70 (main)
  • Anti-topoisomerase
  • Anti-RNA III Polymerase
52
Q

What defines limited systemic sclerosis?

A
  • Skin involvement only involving the forearms, hands, feet and face
  • Late organ involvement
53
Q

What antibody is associated with limited systemic sclerosis?

A

Anti-centromere

54
Q

What tests are performed as monitoring in patients with systemic sclerosis?

A
  • Pulmonary function tests
  • ECHO
  • Renal function tests
55
Q

What is used to treat the Raynaud’s in systemic sclerosis?

A

Ca++ channel blockers and phosphodiesterase inhibitors

56
Q

When is immunosuppression given in systemic sclerosis?

A

If there is advanced lung disease

57
Q

What is used to treat pulmonary hypertension in systemic sclerosis?

A

Ca++ blockers and endothelin receptor antagonists

58
Q

What drugs are used to treat GI symptoms in systemic sclerosis?

A

PPIs, H2 antagonists

Antibiotics if there is bacterial overgrowth

59
Q

What drug is used if there is renal involvement in systemic sclerosis?

A

ACE Inhibitors

60
Q

Mixed connective tissue disease involves features of what diseases?

A
  • SLE
  • Polymyositis
  • Systemic sclerosis
61
Q

What is the biggest recognised complication of MCTD and how is it screened for?

A

Pulmonary hypertension- ECHO

62
Q

What antibody is associated with mixed connective tissue disease?

63
Q

What major organ involvement should be screened for in MCTD?

A

Interstital lung disease

64
Q

What is the treatment for MCTD?

A

Depends on symptoms and whether or not there is major organ involvement

MSK- Rheumatology (12 decks)

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