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CTD Flashcards

1
Q

________is the most common autoimmune disease

A

Rheumatoid arthritis

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2
Q

Vasculitis is, in fact, a condition common to the
connective tissue disorders and to the so-called
_______

A

vasculitides

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3
Q

A classification of rheumatological pain

Hyperacute (red hot) joints

Crystals

1
2
3

A

Urate: gout
Calcium pyrophosphate
Hydroxyapatite

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4
Q

A classification of rheumatological pain

Hyperacute (red hot) joints

Pus
Example: ________

A

staphylococcal septic arthritis

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5
Q

A classification of rheumatological pain

Inflammation of joints

Symmetrical

Example:________

A

rheumatoid arthritis

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6
Q

A classification of rheumatological pain

Inflammation of joints

aSymmetrical

Example:________

A

Example: spondyloarthropathies

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7
Q

A classification of rheumatological pain

Non-inflammatory joint disorder

Typical:

Example_____

A

Primary osteoarthritis (e.g. in hands)

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8
Q

A classification of rheumatological pain

Non-inflammatory joint disorder

aTypical:

Example: _____

A

post-trauma, haemochromatosis

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9
Q

Joint and soft tissue inflammation

Connective tissue disorders

examples

1
2
3
4

A

SLE
Scleroderma
Polymyositis/dermatomyositis
Polyarteritis nodosa

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10
Q

Joint and soft tissue inflammation

Vasculitides

Example
1
2

A

Giant cell arteritis

Polymyalgia rheumatica

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11
Q

Non-articular (soft tissue) inflammation

Generalised

Examples:
1
2
3

A

fibrositis, fibromyalgia, polymyalgia

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12
Q

Non-articular (soft tissue) inflammation

Localised

Examples:
1
2

A

plantar fasciitis, epicondylitis

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13
Q

What type of Vasculitides

• giant cell arteritis/temporal arteritis/polymyalgia
rheumatica
• Takayasu arteritis
• Behçet syndrome

A

Large vessel predominantly

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14
Q

What type of Vasculitides

  • polyarteritis nodosa
  • Kawasaki disease
A

Medium vessel:

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15
Q

What type of Vasculitides

  • Henoch–Schönlein purpura
  • hypersensitivity vasculitis
  • essential cryoglobulinaemia
A

Small vessel (mainly):

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16
Q

What type of Vasculitides

  • Wegener granulomatosis
  • Churg–Strauss vasculitis
  • microscopic polyangiitis
A

Antineutrophil cytoplasmic antibody (ANCA) associated:

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17
Q

The term ‘__________is a generic
label applied to a group of disorders characterised by
inflammation, presumed initiated by an autoimmune
response to an autoantigen and perpetuated by
unknown factors

A

connective tissue disease’ (CTD)

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18
Q 
The CTDs comprise three classic conditions,
namely 
1
2
3
A

systemic lupus erythematosus (SLE),
scleroderma and
polymyositis/dermatomyositis

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19
Q

Mixed connective tissue disorder includes features
of all three disorders and is sometimes referred to as
______

A

‘overlap’ syndrome

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20
Q

Baseline workup for pts with CTD:

A

FBE, ESR, C-reactive

protein and rheumatoid factor

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21
Q

The _____ test is a generic term for

autoantibodies to several different cellular antigens

A

antinuclear

antibody (ANA)

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22
Q

ANA is Sn for ___

A

SLE

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23
Q

T or F

ANA is Sp for SLE

A

F

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24
Q

false positive for ANA

A

viral arthritis and others

e.g. Sjögren

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25
Q

When is ANA useful>

A

It is especially useful in the young
female presenting with fatigue, small joint arthralgia
and dermatological features of SLE.

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26
Q

The more specific antibodies for SLE, namely
to _________ and _______, should only be ordered if
there is a significantly positive ANA.

A 
double-stranded DNA (dsDNA) and extractable
nuclear antigens (ENA)
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27
Q

High sensitivity and specificity for SLE

(60%): found in rheumatoid arthritis

A

Anti-dsDNA

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28
Q 
Examples of ENA extractable
nuclear antigens (ENA)
1
2
3
4
5
6
A 
Smith (Sm)
U1 RNP
Ro (SSA)
La (SSB)
Scl-70 (antitopoisomerase)
Jo1
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29
Q 
Examples of ENA extractable
nuclear antigens (ENA)

Highly specific for SLE

A

Smith (Sm)

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30
Q 
Examples of ENA extractable
nuclear antigens (ENA)

Common in mixed connective tissue
disease, SLE

A

U1 RNP

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31
Q 
Examples of ENA extractable
nuclear antigens (ENA)

Common in Sjögren syndrome, SLE and
some other connective tissue diseases

A

Ro (SSA)

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32
Q 
Examples of ENA extractable
nuclear antigens (ENA)

Common in Sjögren syndrome, SLE (15%)

A

La (SSB)

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33
Q

Common in 20–30% of patients with scleroderma

A

Scl-70 (antitopoisomerase

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34
Q

Common in 30% of patients with polymyositis

A

Jo1

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35
Q

High sensitivity and specificity for CREST

syndrome

A

Anticentromere

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36
Q

High sensitivity and specificity for Wegener granulomatosis

A

Antineutrophil cytoplasm

ANCA

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37
Q

Diagnostic in antiphospholipid syndrome

A

Antiphospholipids

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38
Q

Present in 5–10% of SLE

A

Lupus anticoagulants

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39
Q

Examples of Antiphospholipids

A

Anti-cardiolipin

Anti-β2-GP1 antibodies

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40
Q

This syndrome may occur with SLE or in isolation and
is responsible for recurrent arterial and/or venous
thromboembolism, recurrent spontaneous abortions
or thrombocytopenia in the presence of antiphospholipid
antibodies but without features of SLE.

A

Antiphospholipid antibody

syndrome

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41
Q

Tx of APAS

A

aspirin 150–300 mg (o) daily

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42
Q

SLE (lupus), which is the commonest of the

connective tissue disorders, is described as the ‘____

A

great

pretender

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43
Q

T or F

In SLE

Milder manifestations outnumber more severe forms.

A

T

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44
Q

SLE

Mainly affects women in _______ period
(90% of cases

A

‘high oestrogen’

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45
Q

How to dx SLE

Fulfill __ of 11 criteria

A

4

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46
Q

SLE Features

  • _______rash
  • Discoid rash
  • Photosensitivity
  • Arthritis ________
  • Oral ulcers (usually _____)
  • Serositis (pleurisy or pericarditis)
  • Kidney features (________)
A

Malar (butterfly)

(non-erosive arthritis in ≥ 2 peripheral
joints)

painless

proteinuria or cellular casts

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47
Q

SLE Features

• Neurological features (intractable headache,
seizures or psychosis)
• Haematological features (______)
• Immunological features (positive _____ and ______ and
false positive syphilis serology)
• Positive antinuclear antibody (ANA) test

A

haemolytic anaemia,
leucopenia, lymphopenia or thrombocytopenia

anti-DNA,
antiphospholipid antibodies or anti-Sm tests

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48
Q

Dx of SLE

• _______—elevated in proportion to disease
activity
• _______—positive in 95% (perform first
key test)

A

ESR/CRP

ANA test

49
Q

Dx of SLE

• \_\_\_\_\_\_\_—90% specific for SLE but
present in only 60% (key test)
• \_\_\_\_\_\_\_\_—highly specific
• \_\_\_\_\_\_\_\_\_—positive in 50%
• \_\_\_\_\_\_—inefficient and not used
A

dsDNA antibodies

ENA antibodies, especially Sm

Rheumatoid factor

LE cell test

50
Q

For suspected SLE, the recommended approach is to

perform an ANA test. If positive then order ___ and _____

A

dsDNA

and ENA antibodies

51
Q

Tx of SLE

Based on severity and organ involved.
• Mild: _____ (for arthralgia)
• Moderate (especially skin, joint serosa involved):
_______

A

NSAIDs

low-dose antimalarials (e.g. hydroxychloroquine
up to 6 mg/kg once daily)

52
Q

Tx of SLE

Severe: _______ are the mainstay

___________ may be
used for severe arthralgia

A

corticosteroids (e.g. prednisolone 7.5–15 mg (o) daily):

immunosuppressive drugs (e.g. azathioprine,
methotrexate with folic acid, rituximab)
53
Q

Tx of SLE

Avoid drugs in those in clinical remission and
with normal _____ levels.

A

complement

54
Q

Tx of SLE

Other treatments, such as ___ and _____, are available for severe disease

A

plasma exchange and

immunosuppressive regimens

55
Q

Keep in mind antiphospholipid antibody

syndrome, especially with __ and ___

A

recurrent fetal loss and

thrombotic episodes

56
Q

This can present as a polyarthritis affecting the
fingers of the hand in 25% of patients, especially
in the early stages. Soft tissue swelling produces a
‘sausage finger’ pattern.

A

Scleroderma (systemic sclerosis

57
Q

Scleroderma mainly affects
the skin, presenting with _______ in
over 85% of patient

A

Raynaud phenomenon

58
Q

There are three clinical variants of scleroderma:
1 limited cutaneous disease, for example,_______
2 cutaneous with limited organ involvement ______
3 diffuse systemic disease ____

A

morphea
(CREST)
(systemic sclerosis)

59
Q

Sx of pts with Scleroderma

  • ____ facies (mouth puckered)
  • Dysphagia and diarrhoea ____
  • _____ dysmotility
  • Respiratory symptoms:____
  • Cardiac symptoms: pericarditis, etc.
  • Look for tight skin on chest ___
A

‘Bird-like’

(malabsorption)

pulmonary fibrosis

(Roman breastplate

60
Q

finger discomfort + arthralgia + GORD

(± skin tightness

A

scleroderma

61
Q

Dxtics of pts with scleroderma

\_\_\_\_\_\_\_may be raised
•\_\_\_\_\_\_\_ anaemia may be
present
\_\_\_\_\_\_—up to 90% positive (relatively specific)
\_\_\_\_\_—positive in 30%
\_\_\_\_\_\_\_—specific (positive
in 90% with limited disease and 5% with
diffuse)
A

ESR

Normocytic normochromic

  • ANA test
  • Rheumatoid factor
  • Anticentromere antibodies
62
Q

Dxtics of pts with scleroderma

• ______antibody is
specific but only positive in 20–40%
• Skin biopsy—increase in _____

A

Antitopoisomerase I (anti-Scl-70)

dermal collagen

63
Q

drugs to avoid in scleroderma

Avoid vasospasm (no smoking, ___ and ______

A

beta blockers,

ergotamine):

64
Q

______ may help Raynaud

A

calcium channel blockers such as

nifedipine

65
Q

Tx of scleroderma

____ can help if there is significant
systemic or cutaneous involvement

A

D-penicillamine

66
Q

Localised scleroderma

• ______—plaques of erythema with violaceous
periphery, feels hard; mainly on trunk
•______—may be ‘en coup de sabre’ (a sabre stroke)

A

Morphea

Linear

67
Q

Clinical features of CREST

A
  • Calcinosis
  • Raynaud phenomenon
  • Oesophageal dysmotility
  • Sclerodactyly
  • Telangiectasia
  • Anticentromere antibody (invariably positive)
68
Q

____ is an uncommon systemic disorder
with inflammation of skin and muscle whose main
feature is symmetrical muscle weakness and wasting
involving the proximal muscles of the shoulder and
pelvic girdles.

A

Polymyositis

69
Q

PM

Peak incidence_______years
• Female to male ratio = ____

A

40–60

2:1

70
Q

PM

Consider associated malignancy:_______ and _____

A

lung and ovary

71
Q

weakness + joint and muscle pain +

violaceous facial rash

A

DM

72
Q

The distinctive rash shows features of photosensitivity. What is this called?

A

Heliotrope

73
Q

Ddx for PM or DM

A

statin-induced necrotising

myositis (↑ CK levels)

74
Q

Dx of PM and DM

  • Muscle enzyme studies (___ and ___
  • Biopsies—_____
  • EMG studies—show characteristic pattern
A

serum creatine kinase
and aldolase)

skin and muscle

75
Q

The under-diagnosed syndrome of dry eyes
(keratoconjunctivitis sicca) in the absence of
rheumatoid arthritis or any other autoimmune
disease is known as ______

A

primary Sjögren syndrome (SS):

76
Q

Types of SS

• _______—limited or multisystem
•_______—occurs in association with other
CTDs including rheumatoid arthritis (accounts
for 50%)

A

primary SS

secondary SS

77
Q

Clinical features of SS

• Increased dental caries; denture dysfunction
• Salivary gland enlargement
• \_\_\_\_\_\_\_→ chronic dry cough; hoarseness
\_\_\_\_\_\_\_\_\_
• \_\_\_\_\_\_\_
A

Xerotrachea

• Dyspareunia

Arthralgia ± non-erosive arthritis

78
Q

In SS,

Although considered benign can transform into
________ (44 times risk).

A

non-Hodgkin lymphoma

79
Q

dry eyes + dry mouth + arthritis

A

Sjögren syndrome

80
Q

Dx of SS

Autoantibody tests—
Schirmer’s tear test

A

positive ANA (ENA), Ro (SSA), La (SS-B)

81
Q

Raynaud phenomenon

It is classified as either
1
2

A

primary (without associated disease) or

secondary (when associated with any CTD

82
Q

Patients with primary Raynaud may progress to a
_____ but the likelihood is low (5–15%) and the delay
to diagnosis is long (average of 10 years)

A

CTD

83
Q

________is a clinical syndrome of episodic
arteriolar vasospasm usually involving the fingers
and toes (one or two at a time). It may also involve
the nose, ear or nipple

A

Raynaud

84
Q

The _________ are
a heterogeneous group of disorders involving
inflammation and necrosis of blood vessels, the
clinical effects and classification depending on the
size of the vessels involved

A

vasculitides or vasculitis syndromes

85
Q

___________ is the common type

encountered in practice

A

Small vessel vasculitis

86
Q

Small vessel vasculitis

Skin lesions are usually associated with these
disorders and the most common presentation is
painless, palpable purpura, such as occurs with
______

A

Henoch–Schönlein purpura

87
Q

If a serious________ disease is suspected,
early diagnosis is life-saving because of sinister kidney
damage

A

ANCA-associated

88
Q

Known as ‘pulseless disease’ or ‘aortic arch syndrome’,
this vasculitis involves the aortic arch and other major
arteries

A

Takayasu arteritis

89
Q

Takayasu arteritis

It typically affects young Japanese female adults.

Features include absence of_____ and _____

A

peripheral pulses and hypertension

90
Q

The hallmark is necrotising vasculitis of the
small and medium arteries leading to skin nodules,
infarctive ulcers and other serious manifestations

A

Polyarteritis nodosa

91
Q

Association with PAN

The cause is unknown but associations are found
with

1
2
3

A

drug abusers (especially adulterated drugs),
B-cell lymphomas, other drugs and hepatitis B surface
antigen

92
Q

Special features of PAN

_______ or polyarthritis
• ______ along arterial lines
• ______ and skin ulcers
• Kidney impairment and hypertension

A

Migratory arthralgia

Subcutaneous nodules

Livedo reticularis

93
Q

Special features of PAN

  • Cardiac disorders: arrhythmia, failure, infarction
  • Diagnosis confirmed by ______
  • ESR raised
  • Treatment with ___ and _____
  • Death is usually from kidney disease
A

biopsy or angiogram

corticosteroids and immunosuppressants

94
Q

arthralgia + weight loss + fever (± skin

lesions)

A

polyarteritis nodosa

95
Q

The clinical syndromes are polymyalgia

rheumatica and temporal arteritis. What is the basic pathology?

A

Giant cell arteritis and polymyalgia rheumatica

96
Q

The clinical
manifestations of polymyalgia rheumatica invariably
precede those of temporal arteritis, of which there is
about a_____ association.

A

20%

97
Q

Clinical features (polymyalgia rheumatica)
• Pain and stiffness in ______
• ________l distribution
Early morning stiffness

A

proximal muscles of shoulder and pelvic girdle, cervical spine

Symmetrical

98
Q

Ddx for polymyalgia rheumatica

A

Differential diagnosis: polymyalgic onset rheumatoid

arthritis

99
Q 
malaise + painful shoulder girdle +
morning stiffness (>50 years
A

polymyalgia

rheumatica

100
Q

Temporal arteritis

  • Headache—_____ (CHAPTER 56)
  • Temporal tenderness
  • Loss of pulsation of ____
  • Jaw claudication
  • _______ is diagnostic
A

unilateral, throbbing

temporal artery

Biopsy of artery (5 cm)

101
Q

fatigue + headache + jaw claudication

A

temporal arteritis

102
Q

T or F

No specific test for polymyalgia rheumatica

A

T

103
Q

Temporal arteritis dxtics

ESR—______
• _______—elevated
• Mild anaemia (normochromic, normocytic)

A

extremely high, around 100

C-reactive protein

104
Q

Treatment—for uncomplicated disease of temporal arteritis

Prednisolone
• Starting dose
— temporal (giant cell) arteritis: ______
— polymyalgia rheumatica: ___

A

40–60 mg (o) daily initially for 2–4 weeks ( + aspirin
100 mg/day) then gradual reduction according to ESR/CRP

15 mg (o) daily for 2–4 weeks, then taper

105
Q

Treatment—for uncomplicated disease of temporal arteritis

Taper down gradually to the minimum effective dose (often <5 mg daily) according to the clinical response and the _____.

Aim for treatment for_____

A

ESR and CRP

2 years.

106
Q

Treatment—for uncomplicated disease of temporal arteritis

If complicated (e.g. evolving visual loss) give IV
\_\_\_\_\_\_ for 3 days prior to oral agents
A

methylprednisolone

107
Q

Treatment—for uncomplicated disease of temporal arteritis

___ and ____can be used as
steroid-sparing agents.

A

Azathioprine or methotrexate

108
Q

In giant cell arteritis, a delay in diagnosis after
presenting with amaurosis fugax and non-specific
symptoms can have tragic consequences, in the form of
ischaemic events such as _____ and ______

A

blindness and strokes.

109
Q

_____ is a systemic (multiorgan) vasculitis
of unknown aetiology, affecting veins and arteries of
all sizes

A

Behçet syndrome

110
Q

In Behcet,

The main feature is painful oral ulceration
and the hallmark is the ____reaction whereby
simple trauma such as a pinprick can cause a papule
or pustule to form within a few hours at the site

A

‘pathergy’

111
Q

In Behcet,

• Male to female ratio = 2:1
• Recurrent \_\_\_\_\_\_
• \_\_\_\_\_\_\_
• Ocular symptoms—pain, reduced vision, floaters
(ocular inflammation)
A

oral and/or genital ulceration

Arthritis (usually knees)

112
Q

In Behcet,

Associated problems/complications: repeated
_____ → blindness, colitis, venous
thrombosis, meningoencephalitis

A

uveitis and retinitis

113
Q

In Behcet,

Treatment:

A

high-dose steroids and specific ulcer

treatment. DMARDs may be required.

114
Q

this rare vasculitis of unknown cause has a classic
triad: upper respiratory tract (URT) granuloma,
fleeting pulmonary shadows (nodules) and
glomerulonephritis

A

Wegener granulomatosis

115
Q

Other term for Wegener granulomatosis

A

‘granulomatosis with polyangiitis

116
Q

CXR of Wegener

A

Chest X-ray points to diagnosis—multiple nodes,

cavitations

117
Q

T or F,

In Wegener: Antineutrophil antibodies (c-ANCA) are a useful diagnostic marker (not specific)

A

T

118
Q

Wegener granulomatosis

Better prognosis with early diagnosis and
treatment with _____

A

cyclophosphamide

MC (90 decks)

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