CTD Flashcards
________is the most common autoimmune disease
Rheumatoid arthritis
Vasculitis is, in fact, a condition common to the
connective tissue disorders and to the so-called
_______
vasculitides
A classification of rheumatological pain
Hyperacute (red hot) joints
Crystals
1
2
3
Urate: gout
Calcium pyrophosphate
Hydroxyapatite
A classification of rheumatological pain
Hyperacute (red hot) joints
Pus
Example: ________
staphylococcal septic arthritis
A classification of rheumatological pain
Inflammation of joints
Symmetrical
Example:________
rheumatoid arthritis
A classification of rheumatological pain
Inflammation of joints
aSymmetrical
Example:________
Example: spondyloarthropathies
A classification of rheumatological pain
Non-inflammatory joint disorder
Typical:
Example_____
Primary osteoarthritis (e.g. in hands)
A classification of rheumatological pain
Non-inflammatory joint disorder
aTypical:
Example: _____
post-trauma, haemochromatosis
Joint and soft tissue inflammation
Connective tissue disorders
examples
1
2
3
4
SLE
Scleroderma
Polymyositis/dermatomyositis
Polyarteritis nodosa
Joint and soft tissue inflammation
Vasculitides
Example
1
2
Giant cell arteritis
Polymyalgia rheumatica
Non-articular (soft tissue) inflammation
Generalised
Examples:
1
2
3
fibrositis, fibromyalgia, polymyalgia
Non-articular (soft tissue) inflammation
Localised
Examples:
1
2
plantar fasciitis, epicondylitis
What type of Vasculitides
• giant cell arteritis/temporal arteritis/polymyalgia
rheumatica
• Takayasu arteritis
• Behçet syndrome
Large vessel predominantly
What type of Vasculitides
- polyarteritis nodosa
- Kawasaki disease
Medium vessel:
What type of Vasculitides
- Henoch–Schönlein purpura
- hypersensitivity vasculitis
- essential cryoglobulinaemia
Small vessel (mainly):
What type of Vasculitides
- Wegener granulomatosis
- Churg–Strauss vasculitis
- microscopic polyangiitis
Antineutrophil cytoplasmic antibody (ANCA) associated:
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
connective tissue disease’ (CTD)
The CTDs comprise three classic conditions, namely 1 2 3
systemic lupus erythematosus (SLE),
scleroderma and
polymyositis/dermatomyositis
Mixed connective tissue disorder includes features
of all three disorders and is sometimes referred to as
______
‘overlap’ syndrome
Baseline workup for pts with CTD:
FBE, ESR, C-reactive
protein and rheumatoid factor
The _____ test is a generic term for
autoantibodies to several different cellular antigens
antinuclear
antibody (ANA)
ANA is Sn for ___
SLE
T or F
ANA is Sp for SLE
F
false positive for ANA
viral arthritis and others
e.g. Sjögren
When is ANA useful>
It is especially useful in the young
female presenting with fatigue, small joint arthralgia
and dermatological features of SLE.
The more specific antibodies for SLE, namely
to _________ and _______, should only be ordered if
there is a significantly positive ANA.
double-stranded DNA (dsDNA) and extractable nuclear antigens (ENA)
High sensitivity and specificity for SLE
(60%): found in rheumatoid arthritis
Anti-dsDNA
Examples of ENA extractable nuclear antigens (ENA)
1 2 3 4 5 6
Smith (Sm) U1 RNP Ro (SSA) La (SSB) Scl-70 (antitopoisomerase) Jo1
Examples of ENA extractable nuclear antigens (ENA)
Highly specific for SLE
Smith (Sm)
Examples of ENA extractable nuclear antigens (ENA)
Common in mixed connective tissue
disease, SLE
U1 RNP
Examples of ENA extractable nuclear antigens (ENA)
Common in Sjögren syndrome, SLE and
some other connective tissue diseases
Ro (SSA)
Examples of ENA extractable nuclear antigens (ENA)
Common in Sjögren syndrome, SLE (15%)
La (SSB)
Common in 20–30% of patients with scleroderma
Scl-70 (antitopoisomerase
Common in 30% of patients with polymyositis
Jo1
High sensitivity and specificity for CREST
syndrome
Anticentromere
High sensitivity and specificity for Wegener granulomatosis
Antineutrophil cytoplasm
ANCA
Diagnostic in antiphospholipid syndrome
Antiphospholipids
Present in 5–10% of SLE
Lupus anticoagulants
Examples of Antiphospholipids
Anti-cardiolipin
Anti-β2-GP1 antibodies
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.
Antiphospholipid antibody
syndrome
Tx of APAS
aspirin 150–300 mg (o) daily
SLE (lupus), which is the commonest of the
connective tissue disorders, is described as the ‘____
great
pretender
T or F
In SLE
Milder manifestations outnumber more severe forms.
T
SLE
Mainly affects women in _______ period
(90% of cases
‘high oestrogen’
How to dx SLE
Fulfill __ of 11 criteria
4
SLE Features
- _______rash
- Discoid rash
- Photosensitivity
- Arthritis ________
- Oral ulcers (usually _____)
- Serositis (pleurisy or pericarditis)
- Kidney features (________)
Malar (butterfly)
(non-erosive arthritis in ≥ 2 peripheral
joints)
painless
proteinuria or cellular casts
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
haemolytic anaemia,
leucopenia, lymphopenia or thrombocytopenia
anti-DNA,
antiphospholipid antibodies or anti-Sm tests
Dx of SLE
• _______—elevated in proportion to disease
activity
• _______—positive in 95% (perform first
key test)
ESR/CRP
ANA test
Dx of SLE
• \_\_\_\_\_\_\_—90% specific for SLE but present in only 60% (key test) • \_\_\_\_\_\_\_\_—highly specific • \_\_\_\_\_\_\_\_\_—positive in 50% • \_\_\_\_\_\_—inefficient and not used
dsDNA antibodies
ENA antibodies, especially Sm
Rheumatoid factor
LE cell test
For suspected SLE, the recommended approach is to
perform an ANA test. If positive then order ___ and _____
dsDNA
and ENA antibodies
Tx of SLE
Based on severity and organ involved.
• Mild: _____ (for arthralgia)
• Moderate (especially skin, joint serosa involved):
_______
NSAIDs
low-dose antimalarials (e.g. hydroxychloroquine
up to 6 mg/kg once daily)
Tx of SLE
Severe: _______ are the mainstay
___________ may be
used for severe arthralgia
corticosteroids (e.g. prednisolone 7.5–15 mg (o) daily):
immunosuppressive drugs (e.g. azathioprine, methotrexate with folic acid, rituximab)
Tx of SLE
Avoid drugs in those in clinical remission and
with normal _____ levels.
complement
Tx of SLE
Other treatments, such as ___ and _____, are available for severe disease
plasma exchange and
immunosuppressive regimens
Keep in mind antiphospholipid antibody
syndrome, especially with __ and ___
recurrent fetal loss and
thrombotic episodes
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.
Scleroderma (systemic sclerosis
Scleroderma mainly affects
the skin, presenting with _______ in
over 85% of patient
Raynaud phenomenon
There are three clinical variants of scleroderma:
1 limited cutaneous disease, for example,_______
2 cutaneous with limited organ involvement ______
3 diffuse systemic disease ____
morphea
(CREST)
(systemic sclerosis)
Sx of pts with Scleroderma
- ____ facies (mouth puckered)
- Dysphagia and diarrhoea ____
- _____ dysmotility
- Respiratory symptoms:____
- Cardiac symptoms: pericarditis, etc.
- Look for tight skin on chest ___
‘Bird-like’
(malabsorption)
pulmonary fibrosis
(Roman breastplate
finger discomfort + arthralgia + GORD
(± skin tightness
scleroderma
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)
ESR
Normocytic normochromic
- ANA test
- Rheumatoid factor
- Anticentromere antibodies
Dxtics of pts with scleroderma
• ______antibody is
specific but only positive in 20–40%
• Skin biopsy—increase in _____
Antitopoisomerase I (anti-Scl-70)
dermal collagen
drugs to avoid in scleroderma
Avoid vasospasm (no smoking, ___ and ______
beta blockers,
ergotamine):
______ may help Raynaud
calcium channel blockers such as
nifedipine
Tx of scleroderma
____ can help if there is significant
systemic or cutaneous involvement
D-penicillamine
Localised scleroderma
• ______—plaques of erythema with violaceous
periphery, feels hard; mainly on trunk
•______—may be ‘en coup de sabre’ (a sabre stroke)
Morphea
Linear
Clinical features of CREST
- Calcinosis
- Raynaud phenomenon
- Oesophageal dysmotility
- Sclerodactyly
- Telangiectasia
- Anticentromere antibody (invariably positive)
____ 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.
Polymyositis
PM
Peak incidence_______years
• Female to male ratio = ____
40–60
2:1
PM
Consider associated malignancy:_______ and _____
lung and ovary
weakness + joint and muscle pain +
violaceous facial rash
DM
The distinctive rash shows features of photosensitivity. What is this called?
Heliotrope
Ddx for PM or DM
statin-induced necrotising
myositis (↑ CK levels)
Dx of PM and DM
- Muscle enzyme studies (___ and ___
- Biopsies—_____
- EMG studies—show characteristic pattern
serum creatine kinase
and aldolase)
skin and muscle
The under-diagnosed syndrome of dry eyes
(keratoconjunctivitis sicca) in the absence of
rheumatoid arthritis or any other autoimmune
disease is known as ______
primary Sjögren syndrome (SS):
Types of SS
• _______—limited or multisystem
•_______—occurs in association with other
CTDs including rheumatoid arthritis (accounts
for 50%)
primary SS
secondary SS
Clinical features of SS
• Increased dental caries; denture dysfunction • Salivary gland enlargement • \_\_\_\_\_\_\_→ chronic dry cough; hoarseness \_\_\_\_\_\_\_\_\_ • \_\_\_\_\_\_\_
Xerotrachea
• Dyspareunia
Arthralgia ± non-erosive arthritis
In SS,
Although considered benign can transform into
________ (44 times risk).
non-Hodgkin lymphoma
dry eyes + dry mouth + arthritis
Sjögren syndrome
Dx of SS
Autoantibody tests—
Schirmer’s tear test
positive ANA (ENA), Ro (SSA), La (SS-B)
Raynaud phenomenon
It is classified as either
1
2
primary (without associated disease) or
secondary (when associated with any CTD
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)
CTD
________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
Raynaud
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
vasculitides or vasculitis syndromes
___________ is the common type
encountered in practice
Small vessel vasculitis
Small vessel vasculitis
Skin lesions are usually associated with these
disorders and the most common presentation is
painless, palpable purpura, such as occurs with
______
Henoch–Schönlein purpura
If a serious________ disease is suspected,
early diagnosis is life-saving because of sinister kidney
damage
ANCA-associated
Known as ‘pulseless disease’ or ‘aortic arch syndrome’,
this vasculitis involves the aortic arch and other major
arteries
Takayasu arteritis
Takayasu arteritis
It typically affects young Japanese female adults.
Features include absence of_____ and _____
peripheral pulses and hypertension
The hallmark is necrotising vasculitis of the
small and medium arteries leading to skin nodules,
infarctive ulcers and other serious manifestations
Polyarteritis nodosa
Association with PAN
The cause is unknown but associations are found
with
1
2
3
drug abusers (especially adulterated drugs),
B-cell lymphomas, other drugs and hepatitis B surface
antigen
Special features of PAN
_______ or polyarthritis
• ______ along arterial lines
• ______ and skin ulcers
• Kidney impairment and hypertension
Migratory arthralgia
Subcutaneous nodules
Livedo reticularis
Special features of PAN
- Cardiac disorders: arrhythmia, failure, infarction
- Diagnosis confirmed by ______
- ESR raised
- Treatment with ___ and _____
- Death is usually from kidney disease
biopsy or angiogram
corticosteroids and immunosuppressants
arthralgia + weight loss + fever (± skin
lesions)
polyarteritis nodosa
The clinical syndromes are polymyalgia
rheumatica and temporal arteritis. What is the basic pathology?
Giant cell arteritis and polymyalgia rheumatica
The clinical
manifestations of polymyalgia rheumatica invariably
precede those of temporal arteritis, of which there is
about a_____ association.
20%
Clinical features (polymyalgia rheumatica)
• Pain and stiffness in ______
• ________l distribution
Early morning stiffness
proximal muscles of shoulder and pelvic girdle, cervical spine
Symmetrical
Ddx for polymyalgia rheumatica
Differential diagnosis: polymyalgic onset rheumatoid
arthritis
malaise + painful shoulder girdle + morning stiffness (>50 years
polymyalgia
rheumatica
Temporal arteritis
- Headache—_____ (CHAPTER 56)
- Temporal tenderness
- Loss of pulsation of ____
- Jaw claudication
- _______ is diagnostic
unilateral, throbbing
temporal artery
Biopsy of artery (5 cm)
fatigue + headache + jaw claudication
temporal arteritis
T or F
No specific test for polymyalgia rheumatica
T
Temporal arteritis dxtics
ESR—______
• _______—elevated
• Mild anaemia (normochromic, normocytic)
extremely high, around 100
C-reactive protein
Treatment—for uncomplicated disease of temporal arteritis
Prednisolone
• Starting dose
— temporal (giant cell) arteritis: ______
— polymyalgia rheumatica: ___
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
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_____
ESR and CRP
2 years.
Treatment—for uncomplicated disease of temporal arteritis
If complicated (e.g. evolving visual loss) give IV \_\_\_\_\_\_ for 3 days prior to oral agents
methylprednisolone
Treatment—for uncomplicated disease of temporal arteritis
___ and ____can be used as
steroid-sparing agents.
Azathioprine or methotrexate
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 ______
blindness and strokes.
_____ is a systemic (multiorgan) vasculitis
of unknown aetiology, affecting veins and arteries of
all sizes
Behçet syndrome
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
‘pathergy’
In Behcet,
• Male to female ratio = 2:1 • Recurrent \_\_\_\_\_\_ • \_\_\_\_\_\_\_ • Ocular symptoms—pain, reduced vision, floaters (ocular inflammation)
oral and/or genital ulceration
Arthritis (usually knees)
In Behcet,
Associated problems/complications: repeated
_____ → blindness, colitis, venous
thrombosis, meningoencephalitis
uveitis and retinitis
In Behcet,
Treatment:
high-dose steroids and specific ulcer
treatment. DMARDs may be required.
this rare vasculitis of unknown cause has a classic
triad: upper respiratory tract (URT) granuloma,
fleeting pulmonary shadows (nodules) and
glomerulonephritis
Wegener granulomatosis
Other term for Wegener granulomatosis
‘granulomatosis with polyangiitis
CXR of Wegener
Chest X-ray points to diagnosis—multiple nodes,
cavitations
T or F,
In Wegener: Antineutrophil antibodies (c-ANCA) are a useful diagnostic marker (not specific)
T
Wegener granulomatosis
Better prognosis with early diagnosis and
treatment with _____
cyclophosphamide
