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Rheum > General Rheumatology > Flashcards

General Rheumatology Flashcards

1
Q

Anti-Ro is associated with what foetal issue?

A

Foetal heart block.
Ab bind to developing cardiac conduction system resulting in destruction.
More associated with Ro-60.
HCQ may be useful.

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2
Q

What are the 4 types of spondyloarthritis?

A

Axial spondyloarthritis
Reactive arthritis
Psoriatic arthritisf
IBD-associated spondyloarthritis

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3
Q

What are the two most common peripheral manifestations of axial SAs?

A

Arthritis and enthesitis

  • Usually lower limbs, symmetrical
  • Swollen and painful
  • Common enthesitis location is calcaneus (Achilles and plantar fascia)
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4
Q

What is used to quantify disease severity in axial spondyloarthritis?

A

BASDAI and ASDAS

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5
Q

What is the sex distribution in AxSpA?

A

AxSpA 2-3:1 M:F

nrAxSpA 1:1

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6
Q

How does HLA-B27 status predict natural course?

A

If positive, disease onset is 5 years earlier on average..

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7
Q

What genetic associations have been identified in AxSpA?

A

HLA-B27
IL-23 receptor
Endoplasmic reticulum aminopetidase
(ERAP)

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8
Q

What interleukins are primarily involved in AxSpA?

A

IL-23 stimulates Th17 cells to produce IL-17.

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9
Q

How do ILs mediate OP in AxSpA?

A

IL-17 stimulates osteoclasts.

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10
Q

How do ILs mediate osteogenesis in AxSpA?

A

IL-22 release is stimulated by IL-23, and this stimulates osteogenesis.

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11
Q

What are the spondyloarthritic features in diagnostic criteria as per ASAS.

A 
Inflammatory back pain
Arthritis
Dactylitis
Enthesitis (heel)
Psoriasis
Crohn's/UC
Uveitis
Family Hx
HLA-B27
Elevated CRP
Response to NSAIDs
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12
Q

Below what age is back pain used for AxSpA Dx?

A

45yo and below

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13
Q

How long must back pain be present to be used as a primary diagnostic criterion in AxSpA?

A

3 months

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14
Q

What MRI sequences are used and what do they show in AxSpA?

A

STIR - bone marrow oedema

T1 - subchondral sclerosis, fatty lesions, cortical bone defects

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15
Q

What is the treatment algorithm for axial disease?

A
  1. NSAIDS + PT/exercise/rehab/self-help groups/patient associations
  2. TNF-alpha or IL-17 blocker
  3. Analgesics/surgery
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16
Q

How are peripheral manifestations treated?

A

Local steroids/DMARDs (SSZ, MTX)

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17
Q

What is ustekinumab?

A

mAb against the p40 subunit of IL-12 and IL-23.

Currently in clinical trails for AxSpA.

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18
Q

MCTD is associated with which Ab?

A

Anti-U1RNP

The only autoAb found in MCTD

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19
Q

MCTD is primarily a combination of which phenotypes?

A

SLE, polymyositis and scleroderma

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20
Q

What are the 3 main features of Adult Onset Stills?

A

Daily fevers, salmon-pink rash (macular or maculopapular cutaenous eruption) on trunk and extremities

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21
Q

Which 2 diseases present with severely elevated ferritin?

A

HLH and Adult Onset Stills Disease

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22
Q

Where is febuxostat metabolised?

A

Liver

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23
Q

How does pegloticase work?

A

Catalysis of metabolism of uric acid to allantoin, which is 10x more soluble in urine.

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24
Q

What are the muscles of the rotator cuff and what are their functions?

A

Supraspinatus - aBDucts arm before deltoid. Most commonly injured.
Infraspinatus - externally rotates arm
Teres minor - aDDucts and externally rotates arm.
Subscapularis - aDDucts and internally rotates the arm.

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25
Q

Which disorder is consistent with the “double contour sign” on US of a painful joint?

A

Gout

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26
Q

What is a significant level of positivity of ANA?

A

> 1:160

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27
Q

Which Ab in polymyositis and dermatomyositis are associated with a reduced risk of malignancy?

A

Jo-1, Mi-2

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28
Q

What is tocilizumab and what are the indications for it?

A

IL-6 receptor antagonist
Severe active RA and severe juvenile idiopathic arthritis
Also shown to provide better sustained response to 52 weeks in GCA

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29
Q

What is tofacitinib and what is it used for?

A

Janus kinase inhibitor

Rheumatoid arthritis

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30
Q

What is sensitivity of P-ANCA in MPA?

A

90%

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31
Q

Which digits are most commonly affected by dactylitis in psoriatic arthritis?

A

3rd and 4th toes

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32
Q

After ankylosing spondylitis, which spondyloarthritis has the strongest association with HLA-B*27?

A

Reactive arthritis > psoriatic > IBD

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33
Q

What is the treatment algorithm for psoriatic arthritis?

A

Mild oligoarticular - NSAIDs and intra-articular steroids
Severe symptoms - DMARDs
Peripheral arthritis - leflunomide
Skin and joint inflammation - TNF-alpha inhibitor, ustekinumab, secukinumab, ixekizumab (IL-17 blocker), PDE-4 inhibitors (apremilast)

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34
Q

What are the 5 subtypes of psoriasis?

A 
Plaque (ie psoriasis vulgaris)
Guttate (ie droplet)
Inverse (ie intertriginous or flexural)
Pustular - palmoplantar or generalised
Erythrodermic
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35
Q

Which genetic mutations predispose to pustular psoriasis?

A

IL-36RN recessive mutation and CARD14 gain of function mutation

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36
Q

Which medications can exacerbate psoriasis?

A

Beta blockers, lithium, antimalarials, NSAIDs

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37
Q

What proportion of psoriatic patients develop psoriatic arthritis?

A

Up to 30%

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38
Q

Which mutation is most strongly associated with heritability of psoriasis?

A

HLA-Cw6

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39
Q

What are the treatments for dermatological psoriasis?

A 
Topical glucocorticoids, coal tar, Vit D3, topical tacrolimus/pimecrolimus
Narrow band UVB or psoralen + UVA (PUVA)
MTX
Cyclosporin
Acitretin (retinoid)
Apremilast (PDE-4 inhibitor)
TNF-alpha inhibitors
Secukinumab
Ustekinumab
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40
Q

Which rash is typical of reactive arthritis?

A

Keratoderma blenorrhagica - brown macular rash usually on the palms and soles.

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41
Q

How does allopuinol interact with azathioprine?

A

Allopurinol inihibits xanthine oxidase, which breaks down AZA

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42
Q

What is the lifetime prevalence of anterior uveitis in akylosing spondylitis?

A

30-40%

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43
Q

Which subtypes of HLA-B27 are associated with ankylosing spondylitis?

A

B27:02
B27:04
B27:05
B27:07

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44
Q

What are the predictors of good response to TNF-alpha inhibitors?

A

Young age
Short duration of disease
High baseline inflammatory markers
Low baseline functional disability

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45
Q

In which subpopulation of spondyloarthropathies should etanercept be avoided?

A

Anterior uveitis

IBD

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46
Q

What are the contraindications to TNF inhibitors?

A 
High infective risk
CCF
SLE
MS
Cancer
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47
Q

Which patients should receive sulfasalazine in spondyloarthropathy?

A

Peripheral arthritis

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48
Q

What is secukinumab listed for under the PBS?

A

Ankylosing spondylitis

Psoriatic arthritis

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49
Q

What are the antisynthetase antibodies?

A 
Jo-1
PL-7
Pl-12
ZO
OJ
KE
KS
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50
Q

Which pulmonary function test measurement exhibits the highest degree of variability?

A

DLCO

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51
Q

How do lumacaftor and ivacaftor work?

A
  • Lumacaftor potentiates transport of CFTR to the cell surface
  • Ivacaftor turns CFTR on when it reaches the membrane
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52
Q

What tricuspid regurgitation velocity should pulmonary HTN be suspected at?

A

> 2.9m/sec

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53
Q

Anti RNA polymerase III is associated with which condition?

A

Diffuse systemic sclerosis and scleroderma renal crisis

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54
Q

What dermatomyositis phenotype is associated with MDA-5 antibodies?

A
  • Cutaneous ulceration involving the Gottron’s papules, elbows, digital pulp, and nailfolds
  • Erythematous, painful palmar macules and papules
  • Alopecia
  • Oral ulcers
  • Arthritis
  • Amyopathic disease
  • Rapidly progressive intersitial lung disease
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55
Q

Which neurological disorders are most likely to demonstrate REM sleep behavious disorder?

A

DLB > MSA > Parkinson’s disease

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56
Q

Anti Mi-2 antibodies are associated with which disorder and what do they predict?

A

Dermatomysositis

Predicts good response to steroids

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57
Q

What are anti-SRP antibodies associated with?

A 
Necrotising myositis (polymyositis)
Early, rapid onset
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58
Q

What does histopath demonstrate in dermatomyositis and polymyositis?

A

Dermatomyositis - perimyopathic

Polymyositis - throughout muscle fibre

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59
Q

Which malignancies are most strongly associated with dermatomyositis?

A

Ovarian, breast and lung

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60
Q

Which myositis-specific antibody is most strongly associated with malignancy?

A

anti p155/140 antibodies (anti TIF-1)

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61
Q

Which patients with inclusion body myositis should be treated with IVIG?

A

Those with dysphagia

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62
Q

What test must be performed on all patients with limb girdle muscular dystrophy?

A

Echocardiogram

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63
Q

What % of ankylosing spondylitis patients have inflammatory bowel disease?

A

10% (Crohn’s > UC)

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64
Q

What is first line treatment in mild SLE?

A

NSAIDs

Hydroxychloroquine

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65
Q

What is Jaccoud’s arthritis?

A

Deforming, non-erosive, correctable arthropathy in SLE

66
Q

In SLE, what is leflunomide particularly good for treating?

A

Arthritis

67
Q

What are risk factors for hydroxychloroquine-induced retinal toxicity?

A

Renal impairment

Old age

68
Q

What is the mechanism of action of belimumab in SLE?

A

Blys inhibitor

69
Q

What is the usual first line treatment in lupus nephritis?

A

Cyclophsophamide

70
Q

What is anti-ribosomal P associated with?

A

Psychosis and severe depression in SLE

71
Q

What can be used to treat SLE in pregnancy?

A

Hydroxychloroquine

Azathioprine if necessary

72
Q

Which antibody is associated with fetal heart block?

A

SS-A

73
Q

Which antibody is associated with lung disease in scleroderma?

A

Scl-70

74
Q

What is the most common cause of death in scleroderma?

A

Interstitial lung disease

75
Q

What is the most common pattern on HRCT in scleroderma?

A

NSIP is most common

UIP less common but worse prognosis

76
Q

What should pulmonary hypertension in scleroderma be treated with?

A

Tadalafil and ambrisentan dual therapy

77
Q

What can precipitate scleroderma renal crisis and how is it treated?

A

Steroids can precipitate it

Treat with ACEI (ideally captopril)

78
Q

Which antibody is most assocaited with pulmonary hypertension in scleroderma?

A

Anti-centromere

79
Q

Which drugs may be used for scleroderma-related interstitial lung disease?

A

Cyclophosphamide
Mycophenolate
Prednisone (low dose)

80
Q

What aggravating factors for Raynaud’s disease should be avoided?

A

Smoking
Caffeine
Cold

81
Q

What are the 1st and 2nd line vasodilators in Raynaud’s syndrome?

A

1st line:

  • Nifedipine, amlodipine
  • Verapamil
  • Topical GTN

2nd line:

  • ARB (losartan)
  • SSRI (fluoxetine)
  • Phosphodiesterase inhibitors (sildenafil)
  • Alpha blockers (prazocin)
82
Q

What are the treatment options in critical ischaemia?

A

ET-1 receptor antagonist (bosentan)
Alprostadil
Sympathectomy

83
Q

What disease must be excluded in cryptogenic iron deficiency anaemia in diffuse scleroderma?

A

Gastric antral vascular ectasia

84
Q

What are the poor prognostic indicators in scleroderma?

A 
Age > 65
Scl-70 positive
Rapidly progressive skin disease
Cardiac
Lung
GI (except oesophagus)
Renal crisis
Tendon friction rubs
85
Q

Mixed connective tissue disease shares features of which 3 diseases?

A

SLE, scleroderma, polymyositis

- Often presents with fat fingers

86
Q

What is anti-CarP antibody specific for?

A

RA

87
Q

What is a classic renal disorder in Sjogren’s Syndrome?

A

Type 1 RTA

- Less commonly proximal RTA +/- Fanconi’s syndrome

88
Q

What isolated electrolyte abnormality can be caused by Sjogren’s Syndrome?

A

Hypokalaemia

89
Q

What types of GN are associated with Sjogren’s Syndrome?

A

Mesangioproliferative

Membranous

90
Q

What is the most common malignancy associated with Sjogren’s Syndrome?

A

MALT lymphoma

91
Q

What is the most common type of neuropathy associated with Sjogren’s Syndrome?

A

Painful neuropathy

92
Q

What happens to gammaglobulins in Sjogren’s Syndrome?

A

Elevated!

93
Q

What does parotid gland biopsy demonstrate in Sjogren’s Syndrome?

A

Diffuse infiltrate of lymphocytes

94
Q

What is Rose-Bengal stain used for?

A

To demonstrate keratitis in Sjogren’s Syndrome

95
Q

What changes would you expect in Sjogren’s Syndrome?

A

ESR elevated in >90% of Sjogren’s Syndrome

96
Q

Which autoimmune disease has the highest incidence of lymphoproliferative disease?

A

Sjogren’s Syndrome

97
Q

What are the predictors for lymphoma development in Sjogren’s Syndrome?

A

IL-18

Low C4

98
Q

Which HLA allele is associated with Sjogren’s Syndrome?

A

HLA DQA1∗0501

99
Q

What GI issues are associated with Sjogren’s Syndrome?

A

Oesophageal mucosa atrophy
Atrophic gastritis
Subclinical pancreatitis

100
Q

Name 4 dietary risk factors for gout

A

Beer and spirits
Fructose
Seafood
Red meat

101
Q

Name 4 factors associated with a lower risk of gout

A

Oestrogen
Low-fat dairy
Vitamin C
Coffee

102
Q

Where is URAT1 found, what is its function, and what are 3 common drugs that inhibit it?

A
  • Found in the proximal convoluted tubule
  • Reabsorbs urate
  • Inihibited by probenecid, losartan and fenofibrate
103
Q

What medications raise serum urate?

A 
Loop and thiazide diuretics
Low dose aspirin
Cyclosporin and tacrolimus
Pyrazinamide and ethambutol
Cytotoxic chemotherapy
Levodopa
Teriparatide
104
Q

Which cytokines are involved in the pathogenesis of gout?

A

IL-1beta
IL-6
TNF

105
Q

What do monosodium urate crystals look like under the microscope?

A 
Needle-shaped
Negatively birefringent (yellow when parallel to slow axis of compensator)
106
Q

Why is urate not accurate during an acute attack?

A

It may drop during an attack

107
Q

In which anatomical region might dual energy CT produce false positives in gout?

A

Costal cartilage

108
Q

What is the mechanism of action of canakinumab?

A

IL-1β inhibitor

109
Q

What are the serum urate targets for high and low urate load patients?

A

< 0.36 mmol/L for low urate load

< 0.30 mmol/L for high urate load

110
Q

How long should colchicine cover be given with commencement of allopurinol?
At what eGFR is it contraindicated?

A

6 months

Contraindicated when eGFR < 45

111
Q

Which HLA allele is assocaited with hypersensitivity reactions to allopurinol, and in which population is it more common?
What are the other risk factors for allopurinol hypersensitivity?

A
  • HLA-B*58:01
  • More common in Han Chinese, Thai, Korean

Risk factors:

  • Renal impairment
  • Thiazide diuretics
  • Higher initial dose
112
Q

Which inflammasome is activated in gout and pseudogout?

A

NLRP3 - results in release of IL-1β

113
Q

Do men, or women, develop OA at a later age?

A

Women

114
Q

Which joints are most commonly affected in erosive OA and what is a specific treatment option for it?

A

Distal interphalangeal joints

Methotrexate may be used

115
Q

Which joint is the most symptomatic when radiographic OA is present?

A

Knee

116
Q

Compare inta-articular steroids vs hyaluronan in OA

A

Steroids:
- Short term pain relief for 1-4 weeks

Hyaluronan:
- Better pain relief at 12-26 weeks compared with steroids

117
Q

Do chondroitin and glucosamine improve pain and radiological progression?

A

Mild improvement in pain

No improvement in radiological progression

118
Q

Which anatomical areas affected by OA have been shown to benefit from topical capsaicin?

A

Hand and knee OA

119
Q

What is the benefit of doxycycline in OA?

A

Reduced progression of joint space loss

- Thought to be via inhibition of metalloproteinases

120
Q

What is the main risk associated with strontium?

A

Thromboembolism

121
Q

Which HLA alleles are associated with an increased risk of rheumatoid arthritis?

A

HLA-DR1 and DR4

122
Q

In Sjogren’s Syndrome, what is flat purpura suggestive of?

A

Hypergammaglobulinaemia

123
Q

A single-nucelotide polymorphism in which gene increases risk of anti-CCP antibodies?

A

PTPN22

124
Q

What is the function of IL-8 in the pathogenesis of rheumatoid arthritis?

A

Attracts neutrophils into synovial fluid

125
Q

What are the components of the diagnostic criteria for rheumatoid arthritis?

A

Number of joints
anti-CCP or RF positivity
ESR or CRP elevation
Duration > 6 weeks

126
Q

Periarticular osteopenia is characteristic of which disease?

A

Rheumatoid arthritis

127
Q

Name 8 diseases with an increased risk of atlanto-axial subluxation

A 
Rheumatoid arthritis
SLE
Psoriatic arthritis
Reactive arthritis
Ankylosing spondylitis
Down syndrome
Marfan disease
Neurofibromatosis type 1
128
Q

What are the 3 main features in Felty’s Syndrome?

A

Rheumatoid arthritis
Neutropenia
Splenomegaly

129
Q

What is first line treatment in rheumatoid arthritis?

What is second line is this is contraindicated?

A

1st line:
- Methotrexate

2nd line:
- Leflunomide, sulfasalazine

Add leflunomide/sulfasalazine if not responding to methotrexate monotherapy

130
Q

What is the mechanism of action of leflunomide?

A

Inihibition of DHODH, thereby inhibiting pyrimidine synthesis and suppressing activated T cells

131
Q

What are the 2 most significant side effects of leflunomide?

A

Pneumonitis

Peripheral neuropathy

132
Q

What should be done if a patient on leflunomide becomes pregnant?

A

Cholestyramine washout

133
Q

Which is associated with slower radiological progression in rheumatoid arthritis when comparing TNF inhibitors with methotrexate?

A

TNF inhibitors

134
Q

What TB treatment must be completed prior to TNF inhibitor commencement?

A

Isoniazid for 9 months if Quantiferon Gold of Mantoux tests are positive

135
Q

Which TNF inhibitor is most strongly associated with demyelination?

A

Etanercept

136
Q

Which subpopulation of rheumatoid arthritis patients benefit most from abatacept?

A

Anti-CCP positive patients

- Should be given with methotrexate

137
Q

Which serious GI side effect is associated with tocilizumab?

A

Bowel perforation - contraindicated in diverticulitis

138
Q

What are the options in rheumatoid arthritis refractory to methotrexate and a TNF inhibitor?

A

Abatacept
Tocilizumab
Rituximab
Toficitinib

139
Q

Which patients benefit most from rituximab?

A

Malignancy
RF or anti-CCP positive
Rheumatoid lung disease

140
Q

What does histology show in inclusion body myositis?

A

Changes of polymyositis

Red ragged fibres (abnormal mitcochondrial aggregates)

141
Q

What does histology show in dermatomyositis and polymyositis?

A

DM:

  • Perifascicular atrophy
  • Predominance of CD4 lymphocytes

PM:
- Predominance of CD8 lymphocytes

142
Q

What are the primary cells involved in necrotising autoimmune myositis?

A

Macrophages

143
Q

Which anti-synthetase antibody is most strongly associated with interstitial lung disease?

A

Anti-OJ

144
Q

What is the mechanism of action of secukinumab?

A

Anti IL-17

145
Q

What is the treatment algorithm for ankylosing spondylitis?

A

Physiotherapy
NSAIDs

TNF inhibitor or secukinumab if unresponsive to above

146
Q

What are the risk factors for Diffuse Idiopathic Skeletal Hyperostosis?

A 
Obesity
T2DM
Older age
Hypoparathyroidism
Retinoic acid
147
Q

What is the first movement usually lost in OA of the hip?

A

Internal rotation

148
Q

What are the characteristics of anterior uveitis and what rheumatological conditions are associated with it?

A

Painful eye
Small pupil
Photophobic
Usually reduced vision

Associated with:

  • Sarcoidosis
  • HLA-B27
  • Behcet’s disease
149
Q

Which immunoglobulin class acts as a B cell receptor?

A

IgD

150
Q

What are the markers of activity in SLE?

A 
Rising dsDNA
Low C3 and C4 levels
Worsening leukopenia, anaemia, thrombocytopenia
Increasing ESR
Increasing or cellular casts, haematuria
151
Q

Which forms of arthritis commonly affect the distal interphalangeal joints of the feet?

A

Psoriatic arthritis
OA
Crystal arthropathies

  • NEVER rheumatoid
152
Q

What is the leading cause of death in scleroderma?

A

Respiratory failure

153
Q

Which factors increase risk of frozen shoulder?

A

Diabetes
Trauma
Protease inhibitors

154
Q

What is the triad associated with gonococcal arthritis?

A

Arthritis
Tenosynovitis
Dermatitis - usually papules, pustules, or vesicles

155
Q

What white cell count is suggestive of septic arthritis?

A

> 50,000 x 10^6/L

156
Q

Which 2 arthitides are most strongly associated with dactylitis?

A

Psoriatic and reactive arthritis

157
Q

Which conditions are associated with avascular necrosis?

A 
Steroids
EtOH
SLE
Antiphospholipid antibodies
Sickle cell disease
HIV
Trauma
158
Q

How does MTX work?

A

MTX blocks dihydrofolate reductase, which converts dihydrofolate to tetrahydrofolate
Tetrahydrofolate is necessary for purine and pyramidine synthsis as well as thymidine synthesis (used in DNA).

159
Q

Which conditions are associated with CPPD?

A 
Haemochromatosis
Hyperparathyroidism
Hypothyroidism
Hypophosphataemia
FHH
Hypomagensaemia
Gitelman's syndrome
X-linked hypophosphataemic rickets
160
Q

What is the mechanism of action of risankizumab?

A

mAb to the p19 subunit on IL-23, which prevents downstream IL-17 production

Rheum (3 decks)

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