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JL Musculoskeletal > Week 2 (2) > Flashcards

Week 2 (2) Flashcards

1
Q

What are SLE, Sjogrens syndrome, systemic sclerosis, dermatomyositis, polymyositis, MCTD and anti-phospholipid syndrome?

A

Connective tissue diseases

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2
Q

How are connective tissue diseases characterised?

A

by the presence of spontaneous over activity of the immune system

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3
Q

What part of the body does SLE affect?

A

Any

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4
Q

Give two pathogenic features of SLE?

A
  1. Immune system attacks body’s cells and tissue, resulting in inflammation and tissue damage
  2. Antibody-immune complexes precipitate and cause a further immune response
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5
Q

What is the female:male ratio for SLE?

A

9:1

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6
Q

What people is there a higher prevalence of SLE in?

A

Asians, afro-americans, afro-caribbeans and hispanic americans

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7
Q

In relation to genetic factors for SLE - what is there a high concordance in?

A

Monozygotic twins

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8
Q

Name a hormonal factor in the aetiology of SLE?

A

Incidence increased in those with higher oestrogen exposure

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9
Q

Name three environmental factors involved in the aetiology of SLE?

A
  1. Epstein-Barr Virus
  2. UV light
  3. Silica dust (cleaning powders, smoking)
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10
Q

In SLE pathogenesis - what happens to apoptosis?

A

Increased and defective

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11
Q

In SLE pathogenesis - what release nuclear material which act as potential autoantigens?

A

Necrotic cells

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12
Q

In SLE pathogenesis - what does autoimmunity possibly result by?

A

Extended exposure to nuclear and intracellular autoantigens

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13
Q

Give four mucocutaneous features of SLE

A
  1. Photosensitivity
  2. Malar rash
  3. Discoid lupus erythematosus
  4. Subacute cutaneous lupus
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14
Q

Give four musculoskeletal features of SLE?

A
  1. Non-deforming polyarthritis
  2. Deforming arthropathy - Jaccoud’s arthritis
  3. Erosive arthritis
  4. Myopathy - weakness, myalgia
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15
Q

Name five pulmonary features of SLE?

A
  1. Pleurisy
  2. Infections
  3. Diffuse lung infiltration and fibrosis
  4. Pulmonary hypertension
  5. Pulmonary infarct
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16
Q

Name four SLE cardiac features

A
  1. Pericarditis
  2. Cardiomyopathy
  3. Pulmonary hypertension
  4. Libman-Sachs endocarditis
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17
Q

In SLE - what presents with proteinuria, urine sediments, urine RBC and casts, hypertension, acute and chronic renal failure?

A

Glomerulonephritis

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18
Q

Give five neurological features in SLE

A
  1. Depression/psychosis
  2. Migraines
  3. Cerebral ischaemia
  4. Cranial or peripheral neuropathy
  5. Cerebellar ataxia
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19
Q

Name four haematological features of SLE

A
  1. Lymphadenopathy
  2. Leucopenia
  3. Anaemia
  4. Thrombocytopenia
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20
Q

In SLE, what does low complements, impaired cell mediated immunity, defective phagocytosis and poor antibody response to certain antigens result in?

A

Susceptibility to infection

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21
Q

Name three extrinsic factors in SLE which increased infection risk?

A
  1. Steroids
  2. Immunosuppressives
  3. Nephrotic system
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22
Q

Name two main screening tests for SLE?

A
  1. FBC

2. Renal function tests including urine examination

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23
Q

Name four immunological tests for suspected SLE?

A
  1. Anti- nuclear antibody
  2. Anti-dsDNA antibodies
  3. ENA
  4. Complement levels
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24
Q

Name three conditions other than SLE, where ANA is found in?

A
  1. RA
  2. HIV
  3. Hepatitis C
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25
Q

Name antibodies which need to be present other than ANA for SLE to be taken seriously?

A
  1. Anti-dsDNA
  2. Anti-Sm
  3. Anti-Ro
  4. Anti-RNP
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26
Q

What antibody in SLE is highly specific, titre collerates with overall disease activity and may be associated with lupus nephritis?

A

Anti-dsDNA

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27
Q

Name two anti-ENA antibodies which are associated with SLE?

A

Anti-Ro

Anti-La

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28
Q

In SLE - anti-Sm (an anti-ENA) is highly speciic and is probably associated with what?

A

Neurological involvement

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29
Q

Name an anti-ENA associated with SLE which relates to sclerodermatous skin lesions, Raynauds and low grade myositis

A

Anti-RNP

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30
Q

In SLE - what does anti-dsDNA positvely correlate with?

A

Activity

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31
Q

In SLE - what does C3/C4 levels negatively correlate with?

A

Activity

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32
Q

Are NSAIDs given for SLE?

A

yes

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33
Q

Name an anti-malarial which is useful for arthritis, cutaneous manifestations and constitutional symptoms in SLE?

A

Hydroxychloroquine

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34
Q

In SLE - steroids are given, what are small doses for?

A

Skin rashes, arthritis and serositis

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35
Q

In SLE - steroids are given, what are moderate doses for?

A

Resistant serositis, haemoatologic abnormalities and class V GN

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36
Q

In SLE - steroids are given, what are high doses for?

A

Severe/resistant haematologic changes, diffuse GN and major organ involvement

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37
Q

Name two side effects for immunosuppressives used in SLE (azathioprine, cyclophosphamide, methotrexate, mycophenolate mofetil)?

A

All can cause bone marrow suppression

All potentially teratogenic

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38
Q

Name two biologics used in SLE?

A

Anti-CD20 (Rituximab)

Anti-Blys (Belimumab)

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39
Q

For mild SLE - waht 3 drugs are give?

A

HCQ, topical steroids and NSAIDS

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40
Q

For moderate SLE - what three drugs are given?

A

Oral steroids, azathioprine and methotrexate

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41
Q

For severe disease - what three drugs are given?

A

IV steroids, cyclophosphamide and rituximab

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42
Q

What do you need to have to be diagnosed with anti-phospholipid syndrome?

A

Positive anti-cardiolipin (anti-phospholipid) antibodies and/ or lupus anticoagulant activity and/or anti-beta2-glycoprotein on 2 occassions at least 12 weeks apart

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43
Q

Is arterial/venous thromosis and pregnancy loss with no other explanation a feature of anti-phospholipid syndrome?

A

Yes

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44
Q

Who does anti-phospholipid syndrome occur in?

A

Young women

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45
Q

Name five clinical features of anti-phospholipid syndrome?

A
  1. Superficial thrombophlebitis and livedo reticularis
  2. Mild/moderte thrombocytopenia
  3. Neurological features - migraine, transverse myelitis
  4. Libman-Sacks endocarditis
  5. Catastrophic anti-phospholipid syndrome
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46
Q

Give two treatments for anti-phospholipid syndrome?

A

Life-long anticoagulation

Aspirin and heparin during pregnancy

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47
Q

What connective tissue disease involves lymphocyte infiltration of exocrine glands causing xerostomia and keratoconjunctivitis sicca?

A

Sjogrens syndrome

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48
Q

What two antibodies are found in Sjogrens?

A

Anti-Ro

Anti-La

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49
Q

What ocular test is done in Sjogrens syndrome?

A

Schirmer

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50
Q

What is the peak age for primary sjogrens syndrome?

A

40-60 (m:f = 1:9)

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51
Q

What are five treatment options for Sjogrens syndrome?

A
  1. Eye drops
  2. Saliva replacement
  3. Pilocarpine
  4. Hydroxychloroquine
  5. Steroids and immunosuppression
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52
Q

What connective tissue disease is characterised by vasculopathy (Raynauds syndrome), inflammation and fibrosis?

A

Systemic sclerosis

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53
Q

What is systemic scerosis also known as?

A

CREST

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54
Q

What is CREST?

A 
Calconosis
Raynaud's
Esophageal dysmotility
Sclerodactyly
Telangiectasia
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55
Q

What antibodies are associated with systemic sclerosis?

A

Anti-centromere antibodies

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56
Q

What antibody is in diffuse systemic sclerosis?

A

Anti-Scl-70

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57
Q

What two features characerise diffuse systemic sclerosis?

A
  1. Skin changes within 1 year of Raynauds

2. Truncal and acral skin involvement

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58
Q

What is the peak age and M:F ratio for systemic sclerosis?

A

25-55

1:4

59
Q

Name 6 treatments for systemic sclerosis?

A
  1. Calcium channel blockers
  2. Prostacyclin
  3. ACE inhibitors
  4. Prednisolone
  5. Immunosuppression
  6. Bosentan, Sildenafil
60
Q

Give five major criteria for MCTD?

A
  1. Severe myositis
  2. Pulmonary involvement
  3. Raynauds
  4. Swollen hands
  5. Sclerodactyly
61
Q

What anibody is found >1:10,000 in MCTD?

A

Anti-RNP

62
Q

Define vasculitis?

A

Inflammation of blood vessels, often with ischaemia, necrosis and organ involvement

63
Q

What does primary vasculitis result from?

A

Inflammatory response that targets the vessel walls and has no known cause - sometimes autoimmune

64
Q

What does secondary vasculitis result from?

A

Infection, drug, toxin or part of inflammatory disorder/cancer

65
Q

Name two large-vessel vascuslitises?

A
  1. Takayasu arteritis

2. Giant cell arteritis

66
Q

Name two medium-vessel vasculitis?

A
  1. Polyarteritis nodosa

2. Kawasaki disease

67
Q

What class are microscopic polyangitis, granulomatosis with polyangitis (WEgner) and eosinophilic granulomatosis with polyangitis (Churg-Strauss)?

A

ANCA-associated small-vessel vasculitis

68
Q

What class are cryoglobulinemic vasculitis, IgA vasculitis (Henoch), hypocomplementemic urticarial vasculitis and anti-GBM disease?

A

Immune complex small-vessel vasculitis

69
Q

What can small vessel vasculitis be divided into#?

A

ANCA-associated and non-ANCA associated

70
Q

In a pANCA small vessel vasculitis - if a granuloma is not present what is the diagnosis?

A

Microscopic polyangitis

71
Q

In a pANCA small vessel vasculitis - if a granuloma is present, and asthma is too what is the diagnosis?

A

Churg-Strauss

72
Q

In a pANCA small vessel vasculitis - if a granuloma is present and thre is no asthma what is the diagnosis?

A

Wegeners granulomatosis

73
Q

What is a non-ANCA associated small-vessel vasculitis where there is IgA dominant immune deposit?

A

Henoch-Schnlein purpura

74
Q

What large vessel vasculitis predominantly affects those under 40 and is commoner in females and asian populations?

A

Takayasu arteritis

75
Q

What large vessel vasculitis affects over 50s and typically causes temporal arteritis but the aorta and other vessels may be affected?

A

Giant cell arteritis

76
Q

What are both large vessel vasculitis conditions characterised by?

A

Granulomatous infiltration of walls of large vessels

77
Q

What are five clinical findings in large vessel vasculitis?

A
  1. Bruit - carotid artery
  2. BP difference
  3. Claudication
  4. Carotodynia or vessel tenderness
  5. Hypertension
78
Q

What is temporal arteritis commonly associated with?

A

Polymyalgia rheumatica

79
Q

What are three classic symptoms of temporal arteritis?

A
  1. Unilateral temporal headache
  2. Scalp tenderness
  3. Jaw claudication
80
Q

What is there risk of in temporal arteritis?

A

Blindness due to ischaemia of optic nerve

81
Q

What investigations should be done for temporal arteritis?

A
  1. ESR, plasma viscosity and CRP raised
  2. Biopsy - careful of skip lesions
  3. MRI - vessel wall thickening
82
Q

What is the management for temporal arteritis?

A

Steroids with starting dose of 40-60mg prednisolone

83
Q

What medium vessel disease is seen in children usually under 5 and can cause vasculitis of coronary arteries where aneurysms can develop?

A

Kawasaki disease

84
Q

What medium vessel vasculitiis is characterised by necrotising inflammatory lesions that affect arteries at vessel bifurcations, resulting in micro-aneurysm formation and aneurysms?

A

Polyarteritis nodosa

85
Q

What vasculitis is associated with hep B?

A

Medium vessel

86
Q

What vasculitis disease has the pathology of: granulomatous inflammation of respiratory tract, small and medium vessels. necrotising glomerulonephritis is common?

A

Granulomatosis with polyangiitis (GPA) or Wegener’s granulomatosis

87
Q

What vasculitis disease has the pathology of: eosinophilic granulomatous inflammation of respiratory tract, small and medium vessels and is associated with asthma?

A

Churg-Strauss syndrome or Eosinophilic granulomatosis with polyangitis (EGPA)

88
Q

What vasculitis disease has the pathology of: Necrotising vasculitis with few immune deposits, necrotising glomerulonephriotis is very common?

A

Microscopic polyangiitis (MPA)

89
Q

At what age does Wegeners or GPA present?

A

35-55

90
Q

What are sinusitis, nasal crusting, epitaxis, mouth ulcers, sensorinueural deafness, otitis media and deafness and saddle nose all ENT features of?

A

Wegeners or GPA

91
Q

Does Wegeners give cavitating nodules?

A

Yes

92
Q

Name two cutaneous features of Wegeners?

A

Palpable purpura and cutaneous ulcers

93
Q

What renal condition is seen in GPA or Wegeners?

A

Necrotising glomerulonephritis

94
Q

Name three nervous system features of Wegeners or GPA?

A
  1. Mononeuritis multiplex
  2. Sensorimotor polyneuropathy
  3. Cranial nerve palsy
95
Q

What condition are conjunctivitis, episcleritis, uveitis, optic nerve vasculitis, retinal artery occlusion and proptosis all ocular features of?

A

Wenegers or GPA

96
Q

What is the main difference between Churg-Strauss (EGPA) and Weneger (GPA)?

A

Presence of late onset asthma and high eosinophil count

97
Q

What are a group of autoantibodies against antigens in the cytoplasm of neutrophil granulocytes (most common type of white blood cell)?

A

Anti-neutrophil cytoplasmic antibodies (ANCAs)

98
Q

What is used to detect ANCA?

A

Immunofluoresence

99
Q

What is cANCA found in?

A

GPA or Wegeners

100
Q

What is pANCA found in?

A

MPA and EGPA (Churg-Strauss)

101
Q

What vasculitis is PR3 found mostly in?

A

GPA

102
Q

What vasculitis is MPO found mostly in?

A

MPA

103
Q

What is used to treat localised or early systemic ANCA associated vasculitis?

A

Methotrexate + steroids

104
Q

What is used to treat generalised/systemic ANCA associated vascusliits?

A

Cyclophosphamide + steroids

105
Q

What is used to treat refractory ANCA associated vasculitis?

A

IV immunoglobulins

ituximab

106
Q

Name an acute immunoglobulin A mediated disorder?

A

Henoch Schonlein Purpura (HSP)

107
Q

What conditions involves generalised vasculitis involving small vessels of the skin, GI tract, kidneys, the joints and rarely the lungs and CNS?

A

HSP

108
Q

Who is commonly affected by Henoch-Schonlein Purpura?

A

2-11 year old children

109
Q

What do more than 75% of patients have preceding HSP?

A

URTI, pharyngeal infection, GI infection - most common group A streptococcus

110
Q

5 features of presentation in HSP?

A
  1. Purpuric rash over buttocks nad lower limb
  2. Colicky abdo pain
  3. Bloody diarrhoea
  4. Joint pain
  5. Renal involvement
111
Q

How is HSP managed?

A

Symptoms resolve within 8 weeks

MUST DO URINALYSIS to screen for renal involvement

112
Q

In inflammatory arthritis - what are paracetamol, opiate compounds, NSAIDs and atypical analgesics used for?

A

Symptom relief

113
Q

Name three DMARDs used in inflammatory arthritis?

A
  1. Methotrexate
  2. Sulphasalazine
  3. Hydroxychloroquine
114
Q

What analgesic is commonly used in fibromyalgia?

A

Amitryptiline

115
Q

What class of drug is diclofenac?

A

NSAID

116
Q

What drugs are NSAIDs which selectively target cyclooxygenase-2, an enzyme responsible for inflammation and pain?

A

Cox 2 inhibitors

117
Q

If a patient was at risk of peptic ulcers - what NSAID would you give?

A

Cox 2 inhibitor

118
Q

What NSAID increases the risk of CV events?

A

Cox 2 inhibitors

119
Q

What drugs used in inflammatory arthritis are slow acting, purely anti-inflammatory with no direct analgesic effect and reduce rate of joint damage?

A

DMARDs

120
Q

When using DMARDs for inflammatory arthritis - what must be monitored?

A

WCC

121
Q

When should inflammatory arthritis patients be started on DMARDs?

A

Within 3 months

122
Q

What is the first choice DMARD in most cases?

A

Methotrexate

123
Q

Name a few side effects of methotrexate?

A

Leucopenia, hepatitis, pneumonitis, rash/mouth ulcers, nausea, TERATOGENIC

124
Q

What drug must be stopped in males and females at least 3 months before conception - and what must be started instead?

A

-Methotrexate

Sulphasalasine

125
Q

What drug is an azo ester of sulfapyridine and 5-aminosalicylic acid and is often used in combination with methotrexate in early inflammatory arthritis?

A

Sulfasalazine

126
Q

What are a few side effects of sulfasalazine?

A

Nausea, rash/mouth ulcers, neutropenia, hepatitis, reversible oligozoospermia

127
Q

What DMARD used in SLE, Sjogrens syndrome and RA causes irreversible retinopathy?

A

Hydroxychloroquine

128
Q

If patients suffer side effects for most DMARDs what other options are available?

A 
Sodium aurothiomalate (gold) IM - bone marrow suppression, GN, rash
Penicillamine oral
129
Q

What type of drugs are licensed for RA, psoriatic arthritis and ankylosing spondylitis but are also mroe effective in combo with DMARDS

A

Anti-TNF

130
Q

Give two adverse effects of anti-TNF?

A

Major infection risk (TB)

Contraindicated in pulmonary fibrosis and heart failure

131
Q

What is the criteria for use of ANTI-TNF?

A

High disease activity score and use of previous standard DMARDs

132
Q

What biologic inhibits IL-6?

A

Tocilizumab

133
Q

What biologic inhibits IL-12 and IL-23?

A

Ustekinumab

134
Q

What biologic blocks full activation of T cells?

A

Abatacept

135
Q

What biologic is monoclonal antibody against B cells?

A

Rituximab (CD20)

136
Q

In RA what biologic is NOT used?

A

Ustekinumab

137
Q

In psoriatic arthritis what two biologics are used?

A

Anti-TNF

Ustekinumab

138
Q

In ankylosing spondylitis what biologic is used/

A

Anti-TNF

139
Q

In Connective Tissue Disease -what biologic is used?

A

Rituximab

140
Q

Name a xanthine oxidase inhibitor?

A

Allopurinol

141
Q

Give an adverse affect of allopurinol?

A

Rash

142
Q

Name a few adverse effects of corticosteroids?

A

Weight gain, muscle wasting, skin atrophy, osteoporosis, diabetes, hypertension, cataract, glaucoma, fluid retention

143
Q

What should the dose be like for corticosteroids?

A

Low and for a short time

JL Musculoskeletal (35 decks)

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