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Musculoskeletal > Connective Tissue Disease > Flashcards

Connective Tissue Disease Flashcards

1
Q

What are connective tissue disease characterized by?

A

the presence of spontaneous over-activity of thewh immune system

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2
Q

what type of hypersensitivity is SLE?

A

type III

immune complexes

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3
Q

who is affected more by lupus- males or females?

A

females

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4
Q

what are the 3 main environmental factors which could contribute to the development of SLE?

A
  • virus (eg eptein-barr virus)
  • UV light
  • silica dust (found in cigarette smoke)
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5
Q

why might defective apoptosis lead to the production of auto-antibodies?

A

cell death happens less efficiently so remnants are not cleared away quickly. This allows extended exposure to nuclear and intracellular antigens which might trigger the production of auto-antibodies.

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6
Q

where do the immune complexes get deposited in SLE?

A

in the wall of blood vessels

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7
Q

what is serositis?

A

inflammation of a lining ie pericarditis, peritonitis, pleuritis

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8
Q

what are the 5 main constitutional symptoms of SLE? (vague/non specific- most common symptoms)

A 
fever
malaise
poor appetite
weight loss
fatigue
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9
Q

what are the 7 main mucocutaneous features of SLE?

A
  • photosensitivity
  • malar rash
  • discoid lupus erythematosus
  • subacute cutaneous lupus
  • apthous ulcers
  • alopecia
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10
Q

what is typically spared in an SLE malar rash?

A

nasolabial folds

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11
Q

what is different about discoid lupus?

A

can be solely cutaneous symptoms

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12
Q

what are the 4 main musculoskeletal features of SLE?

A
  • non-deforming polyarthritis/polyarthralgia
  • deforming arthropathy (Jaccoud’s arthritis)
  • erosive arthritis
  • myopathy
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13
Q

how is non-deforming polyarthritis of SLE differentiated from rheumatoid arthritis even though they have the same distribution?

A

SLE polyarthritis has no sign of erosion on radiological investigation

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14
Q

What is Jaccoud’s arthritis?

A

a reversibly deforming arthropathy (not erosive)

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15
Q

what are the 5 main pulmonary features of SLE?

A
  • pleurisy (causing pleural effusion)
  • infections
  • diffuse lung infiltrate and fibrosis
  • pulmonary hypertension
  • pulmonary infarct (causing haemoptysis)
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16
Q

is a patient with SLE more likely to get a transudate or exudate pleural effusion?

A

exudate (due to inflammation, transudate more due to leakage)

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17
Q

what are the 4 main cardiac features of SLE?

A
  • pericarditis
  • cardimyopathy
  • pulmonary hypertension
  • Libman-Sachs endocarditis
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18
Q

what is Libman-Sachs endocarditis?

A

sterile (nonbacterial) endocarditis, valve vegetation but no microbes on lood cultuees

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19
Q

How do you detect glomerulonephritis in a patient with SLE?

A

urinalysis

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20
Q

what are the 5 main neurological features of SLE?

A
  • depression/psychosis
  • migraine
  • cerebral ischamie (TIA or CVA)
  • cranial or peripheral neuropathy
  • cerebellar ataxia
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21
Q

what are the 4 main haematological features?

A
  • lymphadenopathy
  • leukopaenia
  • anaemia
  • thrombocytopaenia
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22
Q

why do patients with SLE have a susceptibility to infection?

A
  • intrinsic factors (impaired immunity)

- extrinsic factors (ie immunosuppressive treatments)

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23
Q

what are the main immune tests to do in suspected SLE?

A
  • anti-nuclear antibody
  • anti-double stranded DNA antibody
  • anti-extractable nuclear antigens (eg anti-Ro, anti-La, anti-Sm and anti-RNP)
  • complement levels
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24
Q

which is more specific for lupus: anti-ds DNA or ANA

A

anti-ds DNA

rarely get false positives

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25
Q

which is antibody is present in more SLE patients- anti-ds DNA or ANA?

A

ANA

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26
Q

what antibody is important in pregnant patients with SLE because of pathogenic effects on foetus?

A

anti-Ro

anti-ENA

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27
Q

what can anti-Ro cause in a neonate?

A

congenital heart block (in utero)

neonatal lupus

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28
Q

what happens to anti-ds DNA titre and complement titre when SLE starts to become active again?

A

anti-ds DNA titre increase

complement titre decreases

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29
Q

why do complement levels decrease in the body during active SLE?

A

complement is being used up

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30
Q

what is the drug treatment of SLE?

A
  • NSAIDs and simple analgesia
  • anti-malarials (hyroxychloroquine)
  • steroids
  • immunosuppressives
  • biologics (rituximab)
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31
Q

when are steroids used in SLE?

A

for acute flare up, never used long term

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32
Q

what is the function of rituximab (a biologic)?

A

B cell depletion therapy

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33
Q

what autoantibodies can be present in anti-phospholipid syndrome?

A

anti-cardiolipin antibodies
lupus anticoagulant
anti-beta2-glycoprotein

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34
Q

how do you diagnose anti-phospholipid syndrome?

A

1 laboratory sign AND 1 clinical sign

Lab sign:
-positive anti-cardiolipin
-lupus anticoagulant
-anti-beta2-glycoprotein
[on 2 occasions, 12 weeks apart]

Clinical sign:

  • arterial or venous thrombosis
  • pregnancy loss with no other explanation from 10 weeks +
  • 3 pregnancy losses with no other explanation below 10 weeks
  • pregnancy loss due to eclampsia, severe pre-eclampsia or placental insufficiency
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35
Q

why do patients with anti-phospholipid syndrome get blood clots when many have lupus anti-coagulant factor?

A

lupus anti-coagulant factor actually is a pro-coagulation factor in vivo

it only inhibits coagulation in vitro

36
Q

who tends to get anti-phospholipid syndrome?

A

young women

37
Q

what type of skin rash can be a feature of anti-phospholipid syndrome?

A 
livedo reticularis
(a vascularitic skin rash with a marbling appearance)
38
Q

what is the the platelet count like in patients with anti-phospholipid syndrome usually?

A

low

still risk of thrombosis

39
Q

what is the pharmacological treatment of anti-phospholipid syndrome?

A

for patients with prev episode of thrombosis- lifelong anti-coagulation (with heparin)

for patients with recurrent pregnancy loss- aspirin + heparin during future pregnancy

40
Q

What is Sjogren’s syndrome?

A

an autoimmune condition which causes a lymphocytic infiltration of exocrine glands leading to xerostomia and keratoconjuctivitis sicca
(+ potentially other areas of dryness)

41
Q

what are the 2 main autoantibodies associated with Sjogren’s syndrome?

A

anti-Ro

anti-La

42
Q

what test is useful for testing xerostomia in suspected sjogren’s sundrome?

A

schirmer test

43
Q

why can anti-Ro cause teratogenic effects?

A

anti-Ro can cross the placenta

44
Q

who tends to get primary sjogren’s syndrome?

A

middle-ages women

45
Q

what is the pharmacologicaly treatment of Sjogren’s syndrome?

A
  • punctal plugs + eye drops
  • saliva replacement
  • pro-salivation agents
  • hydroxychloroquine
  • steroids + immunosuppression (only for severe case)
46
Q

what is the function of pilocarpine?

A

stimulates salivation

47
Q

what type of patients is pilocarpine contra-indicated in?

A

asthmatic patients

48
Q

What is scleroderma/systemic sclerosis?

A

an autoimmune condition which causes fibrosis of the skin and internal organs

49
Q

why does fibrosis of the skin and internal organs occur in scleroderma/systemic sclerosis?

A

due to excess deposition of colllagen

50
Q

what vasculopathy of the hands occurs in almost every patient with scleroderma?

A

raynaud’s syndrome

51
Q

What are the 2 classifications of scleroderma/systemic sclerosis?

A
  1. localised scleroderma

2. systemic scleroderma

52
Q

what is the difference between localised and systemic scleroderma?

A

in localised scleroderma it is isolated to the skin, systemic scleroderma has systemic involvement

53
Q

what are the 2 types of localised scleroderma?

A

morphoea

linea scleroderma

54
Q

what are the 3 types of systemic scleroderma?

A

limited systemic sclerosis
diffuse systemic sclerosis
systemic sclerosis sine scleroderma

55
Q

What are the features of limited systemic sclerosis?

A 
CREST syndrome
C- calcinosis
R- raynauds
E- esophageal dysmotility
S- sclerodactyly
T- telangiectasia
\+ pulmonary hypertension
56
Q

what is calcinosis?

A

depositions of calcium nodules in the sin

57
Q

what is raynauds?

A

an exaggerated vasoconstrictive process in the hands in the cold

58
Q

what is sclerodactyly?

A

skin thickening on the fingers

59
Q

what is telangiectasia?

A

dilated capillaries on the hands, face and mucous membranes

blanche

60
Q

what antibodies are associated with limited systemic sclerosis?

A

anti-centromere antibodies

61
Q

What is diffuse systemic sclerosis?

A

systemic sclerosis with early significant organ involvement and skin changes

62
Q

what antibodies are associated with diffuse systemic sclerosis?

A

anti-Scl-70 antibodies

63
Q

what is systemic sclerosis sine scleroderma?

A

systemic sclerosis with organ involvement but no skin changes

64
Q

who is more likely to get scleroderma- M or F?

A

F

65
Q

what are the treatments for scleroderma?

A

target manifestations-
pulmonary hypertension: calcium channel blockers, bosentan, sildenafil, iloprost
interstitial lung disease: immunosuppression
reflux disease: PPI
others:
ACE inhibitors
prednisolone

66
Q

what is the main con of using steroids in the treatment of scleroderma?

A

may provoke a hypertensive crisis

67
Q

What is the autoantibody associated with mixed connective tissue disease?

A

anti-RNP

68
Q

what is the autoantibody associated with polymyositis?

A

anti-Jo-1

69
Q

for all connective tissue disease, what does the treatment hugely depend on? (what is the one exception to this)

A

depends on patients manifestations (which are very variable)

hydroxychloroquine in SLE is the one exception- ALWAYS used if possible

70
Q

in connective tissue disease, what are the main risk factors to try and reduce?

A

cardiovascular risk factors

71
Q

what connective tissue disease is associated with increased lymphoma risk?

A

Sjogren’s syndrome

72
Q

what are the 3 phases of cutaneous involvement of scleroderma and what does this lead to?

A
  1. oedematous
  2. indurative
  3. atrophic

skin becomes thickened and tight

73
Q

compare limited and diffuse systemic sclerosis in terms of location of skin changes?

A

limited- skin involvement confined to face, hands, forearms and feet

diffuse- can involve trunk (devlops more rapidly)

74
Q

compare limited and diffuse systemic sclerosis in terms of development of organ involvement?

A

limited- organ involvement occurs late

diffuse- early organ involvement

75
Q

compare limited and diffuse systemic sclerosis in terms of antibody association?

A

limited- anti-centromere antibody

diffuse- anti-Scl-70 antibody

76
Q

what is the treatment for raynauds/digital ulcers in scleroderma?

A

calcium channel blockers
iloprost
bosentan

77
Q

what is the treatment for renal involvement in scleroderma?

A

ACE inhibitors

78
Q

what is the treatment for acid reflux in scleroderma?

A

proton pump inhibitos

79
Q

what is the treatment for interstitial lung disease in scleroderma?

A

immunosupprssion

usually with cyclophosphamide

80
Q

What is catastrophic anti-phospholipid syndrome?

A

a rare, serious and often fatal manifestation of APS characterised by multiorgan infarcations over a period of days-weeks

81
Q

how can you distinguish between a malar rash and acne rosacea?

A

malar rash- sparing of the nasolabial folds

malar rash forms where sun has fallen on the face- photosensitivity

82
Q

what treatments are used for mild SLE disease?

A

hydroxychloroquine
NSAIDs
topical steroids

83
Q

what treatments are used for moderate SLE disease?

A

oral steroids
axathioprine
methotrexate

84
Q

what treatments are used for severe SLE disease?

A

cyclophosphamide
IV Ig
Rituximab

85
Q

what are the 2 reasons a patient with diffuse scleroderma might have shortness of breath?

A

-pulmonary fibrosis
or
-pulmonary hypertension

86
Q

what investigations tests for pulmonary hypertension?

A

echocardiogram

Musculoskeletal (16 decks)

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