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RACP Written Exam 2021 > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

Risk factors for RA for patients with genetic risk (HLA-D)

A

1) Smoking
2) Silica
3) Gingivitis/peridontitis

Increased production of citrillunated proteins which bind to the MHC on APC (where the shared epitope sits)
Anti - CCP indicates increased response to CCP in the body

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2
Q

Genes associated with RA

A

1) DRB1
2) STAT 4
3) PTPN22
4) PADI

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3
Q

Cytokines associated with local inflammation and damage in RA

A

TNF
Il-1
Il-6

Macrophage derived and targets for therapy

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4
Q

Common cell finding on atherocentesis of pannus in RA?

A

Polymorphs

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5
Q

Cells involved in the pathogenesis of RA

A 
Macrophages
t-Cells
Synovial Fibroblasts 
B Cells
PMN
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6
Q

Driver of boney erosion in RA

A

RANKL
Produced by T-cells in RA
Encourages osteoclast formation –> bone resorption

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7
Q

Cytokine targets of therapy in RA

A

Il-1:
TNF:
B cells:
Il-6R

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8
Q

Hallmark features of RA

A

1) Erosion - peri-articular (osteoclast)

2) Juxta-articular osteopenia

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9
Q

What proportion of patients with RA are seronegative?

A

One third

Therefore indicates need for diagnostic criteria

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10
Q

Hand findings of RA

A

1) DIP sparing
2) Swan neck
3) Boutonierres
4) Ulnar deviation

note SLE also gets DIP sparing but is passively correctable due to lack of radiological destruction of joints

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11
Q

What rheumatological diseases have positive RF?

A

1) RA (70-90%)
2) Sjogren’s syndrome (75-95%)
3) Cryoglobulinaemia (40-100%)
4) Undifferentiated connective tissue disorder
5) SLE
6) Polymyositis

Non-rheumatic
> Hepatitis C/B
> Viral infections
> Post vaccination

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12
Q

Anti-CCP in RA

A

Specific
Predictive of developing RA in asymptomatic individuals

Strongly associates with genetic risk (including shared epitope)

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13
Q

What indicates activity of RA

A

1) CRP
2) ESR
3) Swollen joint count

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14
Q

Pharmacological treatment of RA

A

Steroids
- Glucocorticoids
Steroid sparing agents/DMARD
> Non-biologic
- Methotrexate (gold standard): flare with discontinuation, improved mortality (decreases cvs/stroke). A/E: LFT changes, myelosupression, MTX lung
- Leflunoamide: Inhibits pyrimidine biosynthesis. A/E: pancytopenia/transaminitis/pneumonitis/diarrhoea
- Plaquenil
- Sulphasalazine
> Biologic (targeted)
- TNF blockers (Infliximab/Entanercept/Adalimumab): Quick onset. A/E Infection/TB/Congestive Heart Failure/De-myelinating disease/Lymphoma/SLE.
- IL-1 antagonists (Anakinra)
- I-6 antagonists (Tocalizumab) A/E diverticular disease and perforation
- B-cell depletion (Rituximab)
- CTLA4 Ig (Abatacept)
- JAK1/3 Inhibitor (Tofacitinib/Baracitinib) A/E immunosuppression/Zoster/VTE

Nb Methotrexate + Biologic
> Note do not use Leflunomide + Methorexate due to poor adverse outcomes
> No live vaccines for TNF blockade
> Addition of pred to any of these increases infection risk

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15
Q

Antidote to Leflunomide therapy in the context of myelosuppression

A

Oral cholestyramine

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16
Q

Precautions with Anti-TNF

A

1) No live vaccines
2) Not for patients with active or chronic infections
- chronic bronchiectasis
- can use in active TB but have to treat with 2 months prior
- healing osteomyelitis
3) Cancer within the last 5 years
4) Contraindicated in stage 4 NYHA heart failure

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17
Q

Adverse effects of IL-6 antagonist (Tocalizumab)

A

1) LFT derangement
2) Immunsupression and masking of infections
- decreased CRP
3) Increase total HDL cholesterol

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18
Q

Main cause of mortality in RA?

A

Cardiovascular mortality

> CRP related to risk

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19
Q

Extra-articular involvement in Ankylosing Spondylitis

A 
Acute Uveitis (25- 40%) 
Inflammatory bowel disease
Osteopenia
Neurological - Cauda equina, fracture, A-a subluxation 
Cardiac 
Respiratory - Apical fibrosis
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20
Q

Diagnosis of Ankylosing Spondylitis

A

1) Clinical
- Inflammatory lower back pain/stifness
- Restriction in lumbar forward flexion or lateral flexion
- Restriction in chest wall expansion
2) Radiological
- Bilateral grade 2 sacroilitiis
- Unilateral grade 3-4 sacroiliitis on xray

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21
Q

Secukinumab MOA and adverse effects

A

IL-17 blocker

Increases risk of fungal infections

Secukinumab - used in Ankylosing Spondylitis

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22
Q

When are DMARDS indicated in Ankylosing Spondylitis

A

For peripheral arthritis or extraarticular disease

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23
Q

Features with the highest LR in GCA

A 
Clinical
- Jaw claudication 
- Termporal artery thickening/pulse loss 
- Limb claudication 
Lab features
- Thrombocytosis >400 
- ESR >100
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24
Q

Interleukin implicated in GCA and associated treatment

A

Interleukin 6

Weekly Tocalizumab (MAB to IL6) can be used

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25
Q

Induction treatment for ACNA vasculitis

A

Severe (organ threatening): High dose glucocorticoids + Cyclophosphamide/Rituximab

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26
Q

Which ANCA vasculitis conveys greater risk of relapse

A

PR3 positivity

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27
Q

Maintenance therapy for ANCA vasculitis

A

Methotrexate or Azathioprine

Rituximab is superior to azathioprine or methotrexate in terms of reducing relapses however not PBS

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28
Q

Anti-synthesase syndrome ENA

A

Anti - jo

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29
Q

ENA which carries the highest risk of Scleroderma renal crisis

A

RNA polymerase III

Risk of scleroderma renal crisis

DO NOT USE STEROIDS IN THESE PATIENTS

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30
Q

Live vaccines

A

MMR
Zoster
Varicella

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31
Q

HLA loci commonly seen in RA and SLE

A

HLA-DRB1

R= Rheumatoid

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32
Q

HLA Loci commonly seen in SLE/SSC/Sjogrens

A

HLA-DQB1

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33
Q

Acquired risk factors for SLE

A

1) Oestrogen:progesterone ratio - higher the oestrogen = greater risk factor
2) UV light exposure
3) Infection with EBV/CMV
4) Microbiome

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34
Q

Best measure of disease activity in SLE

A

DsDNA

additionally supported by low levels of complement

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35
Q

Most specific ENA for SLE

A

Anti-smith

then DS DNA

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36
Q

What ANA does a patient need to have in order to qualify for SLE

A

1:80

As per the new guidelines

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37
Q

Best steroid sparing agent for skin and joint disease in lupus?

A

Methotrexate

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38
Q

Best steroid sparing agent for renal disease in lupus?

A

Mycophenolate (mainstay) or Azathioprine

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39
Q

Hydroxychloroquine adverse effects

A

Occular toxicity

- Once patients on for 5+ years need annual review with ophthalmologist

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40
Q

Treatment of lupus nephritis

A

First line) Mycophenolate or Cyclophosphamide

Second line) Azathioprine

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41
Q

MOA of Belimumab

A

MAB anti-BAFF
- inhibits B-cell survival

Used as add on therapy for high disease activity

42
Q

Which cancer is Sjogrens syndrome associated with

A

Non-Hodgkins lymphoma.

Higher risk

  • Lymphadenopathy
  • Raynauds
  • Salviary gland enlargement
  • Anti-Ro/SSA/La/SSB
  • RF
  • Monoclonal Gammopathy
  • C4 hypercomplimentaemia
43
Q

ENA’s associated with Sjogren’s sydnrome

A

Anti-La
Anti-SSA
Anti-SSB
Anti-Ro

44
Q

Which ENA is associated with Neonatal heart block

A

Anti La

Anti Ro

45
Q

ENA for diffuse skin disease scleroderma

A

SCL-70

At risk for ILD

46
Q

ENA for limited skin disease scleroderma

A

Centromere staining pattern

At risk for primary PAH

47
Q

Anti-synthesase syndrome clinical findings

A 
Myositis
ILD
Inflammatory arthritis
Raynauds
Mechanics hands 
Fever
48
Q

ENA associated with the Anti-synthesase syndrome

A

Anti-Jo 1

49
Q

MDA5 disease

A

Dermatomysositis findings with no myopathy (CK normal)

+ Ulceration of Gottrons papule

50
Q

Additional screening for patients with DM/PM

A

Age appropriate cancer screening (significant increased risk of malignancy)

51
Q

In Scleroderma what is anti-centromere ENA associated with?

A

Limited scleroderma and pulmonary hypertension

52
Q

In Scleroderma what is anti-topiromase ENA associated with?

A

Diffuse scleroderma and higher risk of severe ILD

53
Q

In Scleroderma what is anti-RNA-polymerase ENA associated with?

A

Diffuse scleroderma and scleroderma renal crisis

54
Q

Tocilizumab MOA

A

IL-6 receptor antagonist.

Reduces the total prednisone dose required to achieve remission over 52 week period

55
Q

What does IL-17 therapy (Secukinumab) predispose a patient to?

A

Fungal infection, particularly candidiasis

56
Q

Finding on MRI of the sacroiliac joints on MRI of patient with early AS

A

Subchondral bone marrow oedema

57
Q

First and Second line therapy for axial ankylosing spondyloarthritis

A

First Line: Nsaids and non-pharmacological
Second Line: TNF alpha blocker or anti IL-17

Nb for peripheral manifestations can consider DMARDS: Sulfasalazine or methotrexate
Nb: IL12/23 antibodies are NOT effective for AS

58
Q

Fragility fracture clinical feasture

A

Pain prodrome

Load bearing bone i.e femoral shaft

59
Q

Risk factors for atypical femoral fracture

A

On bisphosphonates
Asian
Young
Concurrent use of corticosteroids

60
Q

Radiological features of an atypical fracture

A

Femoral Diaphysis: Distal to the lesser trochanter but proximal to supracondylar flare
Travers fracture
Beaking

61
Q

Management of atypical femoral fracture

A

Cease bisphosphonate and avoid other antiresportives
Continue calcium and vit D
Anabolic bone meds such as teriperatide
Minimise weight bearing exercises

62
Q

Myocarditis and Myositis

A

Common

Associated with SRP, MDA5, Jo-1, SAE, PL-12, Ro as well as antimitochondrial antibodies

63
Q

Best investigation of cardiac involvement in myositis

A 
Cardiac MRI (non invasive) 
Endomyocardial Biopsy (invasive)
64
Q

Inclusion Body Myositis clinical features (age, rapidity of onset, muscle distribution and labs)

A
  • Age >50
  • Slow onset
  • Proximal and distal muscle weakness
  • Wasting of the quadriceps and forearms
  • Frequent falls due to atrophy
  • CK up to 10 times the upper limit of normal or normal

Nb: Associated with T-cell large granular lymphocytic leukaemia

65
Q

Histological findings on Inclusion Body Myositis

A

Myodegeneration
Rimmed vacuoles
Ragged-red or ragged-blue fibres
Congophilic amyloid deposits

66
Q

Labs in Inclusion Body Myositis

A

Antibodies: IgG anti-cN1A
CK: Normal or elevated

67
Q

Adverse effects of hydrocychloroquine

A

Retinal toxicity
Ventricular arryhthmia
QT prolongation
Cardiomyopathy

68
Q

Dermatomyositis clinical findings

A

Heliotrope rash
Shawl rash
Photosensitive violaceous eruption on the knuckles (Gotrons papule)

69
Q

Histological finding on dermatomyositis

A

Perifascular, perimysial or perivascular B cell infiltrates accompanied with CD4 t-helper cells

70
Q

Clinical significance of dermatomyositis and anti-TIF-1 or anti-NXp-2 autoantibodies

A

Increased risk of malignancy which require annual work up for malignancy for the first three years

71
Q

Polymyositis clinical features

A
  • Subacute onset
  • Proximal muscle weakness
  • Significantly elevated CK level (up to 50 times the normal)
72
Q

Histological findings in polymyositis

A

Endomysial CD8cells invading healthy fibrinase

73
Q

Clinical features of antisynthetase syndrome

A

Mechanics Hands
Interstitial lung disease
Arthritis
Fever

74
Q

Autoantibodies associated with necrotising autoimmune myositis

A

Anti-signal recognition particle (anti-SRP)

Anti-HMGCR antibody

75
Q

Antibodies of the anti-sdnthetase syndrome

A 
Anti-Jo
Anti-PL12
Pl7
OJ
EJ
76
Q

Antibody stratification of dermatomyositis

A

Anti MI2: Classic dermatomyositis with good response to treatment
Anti-SRP: Severe treatment resistant disease

77
Q

Main adverse effect of Tofactinib (JAK1/3 inhibitor)

A

Herpes Zoster reactivation

78
Q

Adverse effects of TNF in RA

A

TB reactivation
Demyelination
Exacerbation of heart failure (cannot use in patients with NYHA class 4 HF)
Malignancies (Lymphoma, Melanoma) - need to avoid if malignancy in the last 5 years

79
Q

Adverse effect of IL-6 blockade i.e Tociluzimab

A

Diverticular perforation

80
Q

Types of cryoglobulinaemia and their association with rheumatoid factor

A

Type 1: A/W Haematological malignant - RF negative. Monoclonal IgM
Type 2: A/W Hepatitis C - RF positive. Polyclonal IgG + Monoclonal IgM
Type 3: A/W Autoimmune disease - RF positive. Polyclonal IgG + Polyclonal IgM

81
Q

Clinical features of cryoglobulinaemia

A

Rash: Palpable purpura
Renal Function: Haematuria
Peripheral nerve involvement

Nb: Lab will show disproportionately low C4 in the setting of normal C3.

82
Q

Treatment of cryoglobulinaemia

A

Immediate: reduction of cryoglobulins themselves with glucocorticoids, cyclophosphamide, rituximab or plasmapharesis
Long term: Clearance of the driving antigen therefore treating the underlying disease

83
Q

Which disease is Polyarteritis Nodosa associated with?

A

Hepatitis B

84
Q

Clinical features of PAN

A

Non-specific inflammatory symptoms
Rash: Livido reticularis, purpura or painful subcutaneous nodules
Peripheral Nerve Involvement: Mononeuritis Multiplex
Renal involvement
GIT: Chronic or intermittent ischaemic pain (abdominal angina)

Nb: Symptoms arise secondary to vessel narrowing and or aneurysmal rupture.

85
Q

Treatment of PAN

A

Induction for severe disease (any end organ dysfunction): High dose glucocorticoids + cyclophosphamide
If Hep B +: Plasma exchange and antiviral therapy
If Hypertensive: ACE inhibitor

Remission-maintenance: 18 months of azathioprine/methotrexate

86
Q

Nail Capillaroscapy findings in patients with Primary versus Secondary Raynauds Disease

A

Primary: Normal nailed capillaroscopy
Secondary: Enlarged or distorted capillary loops and/or dropout or loss of loops suggest an underlying systemic rheumatic disease

87
Q

Secondary causes of Raynauds Disease

A

Rheumatoid Arthritis: Postive anti-CCP
Mixed Connective Tissue Disease: Anto-U1 RNP
Polymyositis: Anti-Jo
Dermatomyositis: Anti-MI2 (Good) and Anti-SRP (Bad)
Sjogrens Syndrome: Anti-centromere, Anti-topoisomerase and Anti-RNA Polymerase III

88
Q

Patient groups in whom Febuxostat may confer higher mortality

A

Ischaemic Heart Disease

Liver failure

89
Q

Pathogenesis of Cheiroarthropathy (Limited Joint Mobility) in Type 1 and Type 2 Diabetics

A

Painless stiffening of the joints caused by deposition of abnormal collagen in the connective tissues around the joints –> stiffening of the joints

90
Q

Cytokines responsible for the pathogenesis of GCA

A

Interleukin 6: Systemic Effects –> Inflammatory repsonse

Interleukin 12 and 18 –> Interferon Gamma –> Local Vascular Effects

91
Q

More rapid radiological progression in AS is associated with?

A 
Male Gender
HLA B27 positivity 
More damage at baseline
Higher inflammatory markers
Higher Disease Activity States
92
Q

Systemic sclerosis classifications

A

Limited: Below knee and elbow. Face Involvement with truncal sparing - associated with anti-centromere and PAH
Diffuse: Affecting all areas - associated with anti-topoisomerase and ILD

93
Q

Treatment of scleroderma

A

Immunosuppresive therapy with Cyclophosphamide or Mycophenolate

PAH: Ambrisentan (endothelin receptor antagonist) and Tadalafil (Phosphodiesterase type 5 inhibitor)

94
Q

HLA epitopes more commonly associated with SLE

A

HLA-DRB1
HLA-DR/DQ

Both double the risk of SLE

95
Q

Treatment for scleroderma renal crisis

A

Oral Captopril

96
Q

Antiphospholipid Syndrome and thrombosis

A

Venous more common than Arterial
Venous most likely to be in the lower limbs
Arterial most likely to be intracranial

97
Q

Monitoring of disease activity in SLE

A

dsDNA
Complement levels
ESR

98
Q

Clinical presentation of drug induced lupus

A 
Fever
Arthralgias
Myalgias
Rash
Serositis

Nb: Cutaneous manifestations are much less common in drug-induced lupus

99
Q

Specific association of SLE and sulphonamide drugs

A

30% of patients with SLE have an allergy to sulphonamides. Therefore caution is required when using these drugs

100
Q

Drugs most likely to cause drug induced lupus?

A

Procainamide

Hydralazine

101
Q

Laboratory findings in drug induced lupus

A

Positive ANA
Anti-histone antibodies
Anti Ro/SSA antibodies

Nb: Most patients with drug induced lupus do not have anti ds-DNA antibodies

102
Q

Belimumab is yes as and add on therapy for treatment of Lupus Nephritis. What is its mechanism of action

A

Monoclonal antibody to anti-BAFF (Blys)

which inhibits the survival of B cells.

RACP Written Exam 2021 (18 decks)

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