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RACP Written Exam 2021 > Rheumatology > Flashcards

Rheumatology Flashcards

(102 cards)

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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
Induction treatment for ACNA vasculitis
Severe (organ threatening): High dose glucocorticoids + Cyclophosphamide/Rituximab
26
Which ANCA vasculitis conveys greater risk of relapse
PR3 positivity
27
Maintenance therapy for ANCA vasculitis
Methotrexate or Azathioprine Rituximab is superior to azathioprine or methotrexate in terms of reducing relapses however not PBS
28
Anti-synthesase syndrome ENA
Anti - jo
29
ENA which carries the highest risk of Scleroderma renal crisis
RNA polymerase III Risk of scleroderma renal crisis DO NOT USE STEROIDS IN THESE PATIENTS
30
Live vaccines
MMR Zoster Varicella
31
HLA loci commonly seen in RA and SLE
HLA-DRB1 R= Rheumatoid
32
HLA Loci commonly seen in SLE/SSC/Sjogrens
HLA-DQB1
33
Acquired risk factors for SLE
1) Oestrogen:progesterone ratio - higher the oestrogen = greater risk factor 2) UV light exposure 3) Infection with EBV/CMV 4) Microbiome
34
Best measure of disease activity in SLE
DsDNA additionally supported by low levels of complement
35
Most specific ENA for SLE
Anti-smith then DS DNA
36
What ANA does a patient need to have in order to qualify for SLE
1:80 As per the new guidelines
37
Best steroid sparing agent for skin and joint disease in lupus?
Methotrexate
38
Best steroid sparing agent for renal disease in lupus?
Mycophenolate (mainstay) or Azathioprine
39
Hydroxychloroquine adverse effects
Occular toxicity | - Once patients on for 5+ years need annual review with ophthalmologist
40
Treatment of lupus nephritis
First line) Mycophenolate or Cyclophosphamide | Second line) Azathioprine
41
MOA of Belimumab
MAB anti-BAFF - inhibits B-cell survival Used as add on therapy for high disease activity
42
Which cancer is Sjogrens syndrome associated with
Non-Hodgkins lymphoma. Higher risk - Lymphadenopathy - Raynauds - Salviary gland enlargement - Anti-Ro/SSA/La/SSB - RF - Monoclonal Gammopathy - C4 hypercomplimentaemia
43
ENA's associated with Sjogren's sydnrome
Anti-La Anti-SSA Anti-SSB Anti-Ro
44
Which ENA is associated with Neonatal heart block
Anti La | Anti Ro
45
ENA for diffuse skin disease scleroderma
SCL-70 At risk for ILD
46
ENA for limited skin disease scleroderma
Centromere staining pattern At risk for primary PAH
47
Anti-synthesase syndrome clinical findings
``` Myositis ILD Inflammatory arthritis Raynauds Mechanics hands Fever ```
48
ENA associated with the Anti-synthesase syndrome
Anti-Jo 1
49
MDA5 disease
Dermatomysositis findings with no myopathy (CK normal) | + Ulceration of Gottrons papule
50
Additional screening for patients with DM/PM
Age appropriate cancer screening (significant increased risk of malignancy)
51
In Scleroderma what is anti-centromere ENA associated with?
Limited scleroderma and pulmonary hypertension
52
In Scleroderma what is anti-topiromase ENA associated with?
Diffuse scleroderma and higher risk of severe ILD
53
In Scleroderma what is anti-RNA-polymerase ENA associated with?
Diffuse scleroderma and scleroderma renal crisis
54
Tocilizumab MOA
IL-6 receptor antagonist. Reduces the total prednisone dose required to achieve remission over 52 week period
55
What does IL-17 therapy (Secukinumab) predispose a patient to?
Fungal infection, particularly candidiasis
56
Finding on MRI of the sacroiliac joints on MRI of patient with early AS
Subchondral bone marrow oedema
57
First and Second line therapy for axial ankylosing spondyloarthritis
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
Fragility fracture clinical feasture
Pain prodrome | Load bearing bone i.e femoral shaft
59
Risk factors for atypical femoral fracture
On bisphosphonates Asian Young Concurrent use of corticosteroids
60
Radiological features of an atypical fracture
Femoral Diaphysis: Distal to the lesser trochanter but proximal to supracondylar flare Travers fracture Beaking
61
Management of atypical femoral fracture
Cease bisphosphonate and avoid other antiresportives Continue calcium and vit D Anabolic bone meds such as teriperatide Minimise weight bearing exercises
62
Myocarditis and Myositis
Common | Associated with SRP, MDA5, Jo-1, SAE, PL-12, Ro as well as antimitochondrial antibodies
63
Best investigation of cardiac involvement in myositis
``` Cardiac MRI (non invasive) Endomyocardial Biopsy (invasive) ```
64
Inclusion Body Myositis clinical features (age, rapidity of onset, muscle distribution and labs)
- 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
Histological findings on Inclusion Body Myositis
Myodegeneration Rimmed vacuoles Ragged-red or ragged-blue fibres Congophilic amyloid deposits
66
Labs in Inclusion Body Myositis
Antibodies: IgG anti-cN1A CK: Normal or elevated
67
Adverse effects of hydrocychloroquine
Retinal toxicity Ventricular arryhthmia QT prolongation Cardiomyopathy
68
Dermatomyositis clinical findings
Heliotrope rash Shawl rash Photosensitive violaceous eruption on the knuckles (Gotrons papule)
69
Histological finding on dermatomyositis
Perifascular, perimysial or perivascular B cell infiltrates accompanied with CD4 t-helper cells
70
Clinical significance of dermatomyositis and anti-TIF-1 or anti-NXp-2 autoantibodies
Increased risk of malignancy which require annual work up for malignancy for the first three years
71
Polymyositis clinical features
- Subacute onset - Proximal muscle weakness - Significantly elevated CK level (up to 50 times the normal)
72
Histological findings in polymyositis
Endomysial CD8cells invading healthy fibrinase
73
Clinical features of antisynthetase syndrome
Mechanics Hands Interstitial lung disease Arthritis Fever
74
Autoantibodies associated with necrotising autoimmune myositis
Anti-signal recognition particle (anti-SRP) | Anti-HMGCR antibody
75
Antibodies of the anti-sdnthetase syndrome
``` Anti-Jo Anti-PL12 Pl7 OJ EJ ```
76
Antibody stratification of dermatomyositis
Anti MI2: Classic dermatomyositis with good response to treatment Anti-SRP: Severe treatment resistant disease
77
Main adverse effect of Tofactinib (JAK1/3 inhibitor)
Herpes Zoster reactivation
78
Adverse effects of TNF in RA
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
Adverse effect of IL-6 blockade i.e Tociluzimab
Diverticular perforation
80
Types of cryoglobulinaemia and their association with rheumatoid factor
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
Clinical features of cryoglobulinaemia
Rash: Palpable purpura Renal Function: Haematuria Peripheral nerve involvement Nb: Lab will show disproportionately low C4 in the setting of normal C3.
82
Treatment of cryoglobulinaemia
Immediate: reduction of cryoglobulins themselves with glucocorticoids, cyclophosphamide, rituximab or plasmapharesis Long term: Clearance of the driving antigen therefore treating the underlying disease
83
Which disease is Polyarteritis Nodosa associated with?
Hepatitis B
84
Clinical features of PAN
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
Treatment of PAN
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
Nail Capillaroscapy findings in patients with Primary versus Secondary Raynauds Disease
Primary: Normal nailed capillaroscopy Secondary: Enlarged or distorted capillary loops and/or dropout or loss of loops suggest an underlying systemic rheumatic disease
87
Secondary causes of Raynauds Disease
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
Patient groups in whom Febuxostat may confer higher mortality
Ischaemic Heart Disease | Liver failure
89
Pathogenesis of Cheiroarthropathy (Limited Joint Mobility) in Type 1 and Type 2 Diabetics
Painless stiffening of the joints caused by deposition of abnormal collagen in the connective tissues around the joints --> stiffening of the joints
90
Cytokines responsible for the pathogenesis of GCA
Interleukin 6: Systemic Effects --> Inflammatory repsonse | Interleukin 12 and 18 --> Interferon Gamma --> Local Vascular Effects
91
More rapid radiological progression in AS is associated with?
``` Male Gender HLA B27 positivity More damage at baseline Higher inflammatory markers Higher Disease Activity States ```
92
Systemic sclerosis classifications
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
Treatment of scleroderma
Immunosuppresive therapy with Cyclophosphamide or Mycophenolate PAH: Ambrisentan (endothelin receptor antagonist) and Tadalafil (Phosphodiesterase type 5 inhibitor)
94
HLA epitopes more commonly associated with SLE
HLA-DRB1 HLA-DR/DQ Both double the risk of SLE
95
Treatment for scleroderma renal crisis
Oral Captopril
96
Antiphospholipid Syndrome and thrombosis
Venous more common than Arterial Venous most likely to be in the lower limbs Arterial most likely to be intracranial
97
Monitoring of disease activity in SLE
dsDNA Complement levels ESR
98
Clinical presentation of drug induced lupus
``` Fever Arthralgias Myalgias Rash Serositis ``` Nb: Cutaneous manifestations are much less common in drug-induced lupus
99
Specific association of SLE and sulphonamide drugs
30% of patients with SLE have an allergy to sulphonamides. Therefore caution is required when using these drugs
100
Drugs most likely to cause drug induced lupus?
Procainamide | Hydralazine
101
Laboratory findings in drug induced lupus
Positive ANA Anti-histone antibodies Anti Ro/SSA antibodies Nb: Most patients with drug induced lupus do not have anti ds-DNA antibodies
102
Belimumab is yes as and add on therapy for treatment of Lupus Nephritis. What is its mechanism of action
Monoclonal antibody to anti-BAFF (Blys) | which inhibits the survival of B cells.

RACP Written Exam 2021 (18 decks)

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