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MBChB YR2 MSK > Disease Profiles: Connective Tissue Diseases > Flashcards

Disease Profiles: Connective Tissue Diseases Flashcards

1
Q

What features would you expect to see on a lip gland biopsy of a patient with Sjogren syndrome?

A

Lymphocytic infiltrates

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2
Q

Which blood test value is decreased in anti-phospholipid syndrome?

A

Thrombocytopenia

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3
Q

Describe the typical symptoms of Sjogren syndrome

A

Dry eyes and mouth, vaginal dryness, joint pains, fatigue

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4
Q

Which antibodies indicate Sjogren syndrome?

A

Anti-Ro, anti-La

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5
Q

Which type of hypersensitivity reaction causes damage to tissues in SLE?

A

Type III - immune complex formation, complement activation and cytokine release

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6
Q

Define scleroderma renal crisis

A

Uncontrolled hypertension with proteinuria and rapidly worsening renal function, often occurs early in disease (presenting feature)

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7
Q

What is the first line management for Raynaud’s (other than avoiding cold)?

A

CCBs

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8
Q

How would you treat an SLE patient with severe organ disease (e.g. lupus nephritis, CNS lupus)?

A

IV steroids and cyclophosphamide

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9
Q

How would you manage a patient with Sjogren syndrome?

A

Symptomatic control e.g. tear and salivary replacement, analgesia

Hydroxychloroquine for arthralgia and fatigue

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10
Q

Which blood test value is increased in anti-phospholipid syndrome?

A

aPTT (partial thromboplastin time)

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11
Q

Which antibodies are associated with venous arterial thrombosis and recurrent miscarriage (antiphospholipid syndrome, can be secondary to SLE )?

A

Antiphospholipid antibodies (APLS) - lupus anticoagulant, anti-cardiolipin antibodies, anti-beta-2 glycoprotein 1 antibodies

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12
Q

How would you manage a patient with anti-phospholipid syndrome who is experiencing recurrent pregnancy loss?

A

LMWH and aspirin

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13
Q

Which complement components have a particular association with renal and haematological disease in SLE?

A

(Decreased) C3 and C4

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14
Q

Describe the common cutaneous manifestations of SLE

A

Malar rash, photosensitivity, non-scarring alopecia, oral/nasal ulcers, Raynauds

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15
Q

Which antibody is found in most patients with a connective tissue disease?

A

ANA

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16
Q

What causes anti-phospholipid syndrome?

A

Can be primary (idiopathic) or secondary to SLE or another rheumatic/autoimmune disorder

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17
Q

What is livedo reticularis?

A

Blood clots in capillaries lead to swelling of venules, resulting in a purplish, net-like discoloration of the skin

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18
Q

Which cutaneous feature is associated with antiphospholipid antibodies?

A

Livedo reticularis

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19
Q

Which disease of bone are SLE patients at increased risk of?

A

Avascular necrosis

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20
Q

In APS, what do the anti-phospholipid antibodies react against?

A

Proteins that bind to anionic phospholipids on plasma membranes

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21
Q

What is Jaccouds arthropathy?

A

Non-erosive reversible joint disorder that can occur after repeated bouts of arthritis, occurs in 10-35% of SLE patients

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22
Q

How should you monitor a patient with SLE?

A

Anti-dsDNA and complement

Urine dipstick

BP and cholesterol (due to increased CVD risk)

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23
Q

What is diffuse systemic sclerosis?

A

Skin changes develop rapidly and can involve the trunk

There is early significant organ involvement

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24
Q

How would you treat an SLE patient with mild-moderate disease (skin disease, arthralgia)?

A

Hydroxychloroquine, short course NSAIDs for symptomatic control, steroids (IA/topical)

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25
Q

What is catastrophic anti-phospholipid syndrome?

A

Rare form of APS involving multi organ infarctions over a period of days to weeks, often fatal

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26
Q

Which patient group does systemic sclerosis most commonly occur in?

A

Women 30-50 years

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27
Q

How can systemic sclerosis affect the face?

A

‘Beaked’ nose, puckered lips, lack of wrinkles

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28
Q

Define mixed connective tissue disease

A

Uncommon, specifically defined condition which features symptoms also seen in other connective tissue diseases - Raynauds, arthralgia/arthritis, myositis, sclerodactyly, pulmonary hypertension, ILD

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29
Q

What is the first line management for pulmonary hypertension in a systemic sclerosis patient?

A

PDE-5 inhibitor e.g. sildenafil

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30
Q

Which antibody is present in 60% of lupus patients and is highly specific?

A

anti-dsDNA

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31
Q

Which patient group does SLE most commonly occur in?

A

Women 20-40, especially in women of Afro-Caribbean, Hispanic American, Asian, and Chinese ethnicity

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32
Q

Define subacute cutaneous lupus

A

Small erythematous lesions on neck, shoulders and forearms; accounts for 10% of cases of SLE

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33
Q

Unexplained increase in dental caries is a possible sign for which disease?

A

Sjogren syndrome

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34
Q

Leukopenia, thrombocytopenia and haemolytic anaemia commonly occur in which disease?

A

SLE

35
Q

Name the antibody that if present in mother can be associated with neonatal lupus and congenital heart risk for baby

A

Anti-Ro

36
Q

Which antibody is associated with limited systemic sclerosis?

A

Anti-centromere antibody

37
Q

Bilateral enlargement of which gland is a sign of Sjogren syndrome?

A

Parotid

38
Q

How would you manage pulmonary hypertension in a systemic sclerosis patient?

A
  1. Immunosuppression e.g. mycophenolate mofetil, cyclophosphamide
  2. Rituximab
  3. Nintedanib (antifibrotic)
39
Q

Name the three factors which predispose to SLE

A

Genetic predisposition (HLA genes), hormonal factors (↑ oestrogen), environmental factors (e.g. infection, some medications)

40
Q

What is limited systemic sclerosis?

A

Skin involvement confined to face, hands, forearms and feet

Organ involvement tends to occur later

41
Q

Describe the cutaneous manifestations of systemic sclerosis

A

Thickening and hardening of the skin, sclerodactyly, digital ulcers

42
Q

Describe the arterial manifestations of anti-phospholipid syndrome

A

Increased frequency of stroke or MI

43
Q

How would you treat a patient with systemic sclerosis who has recurrent digital ulcers?

A

Endothelin receptor antagonist (bosentan)

44
Q

In SLE, what does low complement levels indicate?

A

Active disease - activation and consumption related to disease activity

45
Q

Which medication increases the risk of a renal crisis in a systemic sclerosis patient?

A

Steriods

46
Q

Describe the musculoskeletal manifestations of SLE

A

Arthritis of 2+ joints with 30 mins morning stiffness, arthralgia, myalgia

47
Q

What is Systemic Lupus Erythematosus (SLE)?

A

Systemic autoimmune condition that mainly involves the skin, joints, kidneys, blood cells and nervous system but can affect almost any organ system

48
Q

How would you manage skin fibrosis in a systemic sclerosis patient?

A

Mycophenolate or methotrexate

49
Q

What causes systemic sclerosis?

A

Chronic inflammation results in vascular damage and tissue fibrosis

50
Q

What is the diagnostic criteria for SLE?

A

≳4 critera (at least 1 clinical and 1 lab critera) OR biopsy-proven lupus nephritis with positive ANA or anti-DNA

51
Q

Which rheumatic autoimmune disease is associated with a photosensitive rash, particularly a malar rash?

A

SLE

52
Q

Describe the vascular manifestations of systemic sclerosis

A

Raynaud’s phenomenon - on exposure to cold, fingers go white, then blue, then red

53
Q

What lifestyle changes would you suggest for a patient with SLE?

A

Sun protection measures

54
Q

What causes autoimmunity in SLE?

A

Loss of immune regulation results in increased and defective apoptosis; the nuclear materials released from the necrotic cells act as auto-antigens

55
Q

How would you treat an SLE patient with unresponsive disease?

A

IV immunoglobin, rituximab

56
Q

What is the percentage chance that a patient with discoid lupus erthematosus will develop SLE?

A

10-15%

57
Q

How can anti-phospholipid syndrome affect pregnancy?

A

Late spontaneous miscarriage is common, recurrent early fatal loss is also possible

58
Q

What causes Raynaud’s phenomenon?

A

Vascular spasms that reduce the blood supply to the fingers, usually when the hands get cold

59
Q

Which antibodies are associated with anti-phospholipid syndrome?

A

Lupus anticoagulant, anti-cardiolipin antibodies and anti-beta 2 glycoprotein

60
Q

Describe the non-specific manifestations of systemic sclerosis

A

Fatigue, weakness, joint pain and stiffness

61
Q

Name the antibody which is highly specific for lupus but only positive in ~30% of patients

A

Anti-Smith

62
Q

Which investigation should be performed to confirm lupus nephritis after a positive urine dip stick?

A

Kidney biopsy

63
Q

How would you manage GI involvement in a systemic sclerosis patient?

A

PPIs

64
Q

Which antibody is associated with lupus nephritis?

A

anti-dsDNA

65
Q

Which antibody is associated with fibrosis complications in diffuse systemic sclerosis?

A

Anti-topoisomerase (SCL-7)

66
Q

How should patients with systemic sclerosis be monitored?

A

Echo for pulmonary hypertension

PFTs, then high res CT if needed, for pulmonary fibrosis

67
Q

Which antibody is associated with scleroderma renal crisis?

A

Anti RNA polymerase III

68
Q

Which antibody is associated with mixed connective tissue disease?

A

Anti-RNP

69
Q

What is Sjogren syndrome?

A

Chronic inflammatory autoimmune disease that occurs mainly in middle aged women, characterised by lymphocytic infiltrates in exocrine organs

70
Q

What is Schirmers test?

A

Test for ocular dryness used to investigate Sjogren syndrome

71
Q

Which antibodies are associated with diffuse systemic sclerosis?

A

Anti-SLC-50 and anti-RNA polymerase III

72
Q

How would you treat an SLE patient with moderate-severe disease (inflammatory arthritis or organ involvement)?

A

Hydroxychloroquine, immunosuppressants or short course oral steroids to induce remission in acute flares, treat organ complications appropriately

73
Q

When would you consider additional immunosuppression (other than HCQ) in a patient with Sjogren syndrome?

A

If there is organ involvement e.g. interstitial lung disease

74
Q

Why should you always perform a urine dip stick in any patient with suspected SLE?

A

Anti-dsDNA antibodies are toxic to the kidney - can cause lupus nephritis

75
Q

What is systemic sclerosis?

A

Systemic connective tissue autoimmune disease characterised by vasculopathy, autoimmunity and fibrosis

76
Q

Describe the venous manifestations of anti-phospholipid syndrome

A

DVT/PE, recurrent PE can cause pulmonary hypertension, livedo reticularis

77
Q

Which form of malignancy are patients with Sjorgen syndrome at increased risk of?

A

Lymphoma

78
Q

What is anti-phospholipid syndrome?

A

Autoimmune disorder that manifests clinically as recurrent venous or arterial thrombosis and/or fetal loss

79
Q

Define discoid lupus erthematosus

A

Erythematous raised scaling plaques with active inflammation triggered by UV light exposure

80
Q

What causes Sjogren syndrome?

A

Can be primary disease (unknown cause) or secondary to e.g. RA, SLE

81
Q

How would you manage a patient with anti-phospholipid syndrome?

A

If thrombosis - anticoagulation (acute and prophylaxis)

If never had thrombosis - no anticoagulation

82
Q

How would you treat a renal crisis in a systemic sclerosis patient?

A

ACEi, sometimes dialysis

83
Q

Which patient group does Sjogren syndrome most commonly occur in?

A

Middle aged females

84
Q

Why is it rare to treat only skin fibrosis in a systemic sclerosis patient?

A

Progressive skin fibrosis is usually a marker of progressive lung fibrosis

MBChB YR2 MSK (36 decks)

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