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Basic Physician Training Written Prep > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

Anti DNA Topoisomerase I / Scl-70

A

Diffuse Cutaneous Systemic Sclerosis dcSSc

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2
Q

Anti-Centromere Ab (ACA)

A

Limited Cutaneous Systemic Sclerosis (lcSSc)
- only 5% of patients with dcSSc will be positive for ACA

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3
Q

Anti-RNA Polymerase III

A

If present in patients with dcSSc is associated with rapidly progressive skin involvement and high risk for renal crisis.

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4
Q

Classification of Inflammatory Myopathies

A

Idiopathic Inflammatory Myopathies:
- Polymyositis, Dermatomyositis with the subsets of Anti-Synthetase syndrome and Amyopathic Dermatomyositis (Anti MDA)

Immune Mediated necrotizing myopathy:
- Anti SRP and anti-HMGcoR

Inclusion Body Myositis

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4
Q

Classification of Inflammatory Myopathies

A

Idiopathic Inflammatory Myopathies:
- Polymyositis, Dermatomyositis with the subsets of Anti-Synthetase syndrome and Amyopathic Dermatomyositis (Anti MDA)

Immune Mediated necrotizing myopathy:
- Anti SRP and anti-HMGcoR

Inclusion Body Myositis

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5
Q

Polymyositis

A

By far the rarest form of myositis (5%)

Timeline: Subacute (weeks)
Clinical Features:
- proxima, symmetrical
Extra-Muscular:
- Lung - NSIP
- Heart - pericarditis, conduction block
- Oesophageal dysmotility

10-50 fold CK
No specific serology

Good response to immunosupression

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6
Q

Dermatomyositis

A

Subacute (weeks)
Similar pattern to PM.
Cutaneous findings:
- Gottron papules
- Heliotrope Rash
- V and Shawl Sign

Extra-muscular:
- ILD more common (20-25%)
- Cardiac - CHB, pericarditis

CK 10-15 fold
Anti-Mi2 Ab (good treatment response)
Anti-MDA5 (Rapidly progressive ILD)
Associated with malignancy (poor prognosis)

Good response to treatment apart from ILD or malignancy associated.

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7
Q

Immune mediated Necrotising Myositis

A

Rapidly progressive, acute presentation
Symmetrical, proximal
May be seen after statin exposure, but different to common statin induced myopathy.

CK 10-50 fold elevated
Anti-SRP Ab
Anti-HMGCR

Biopsy: Necrosis without inflammation

Immunosuppression, escalation in therapy may be needed.

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8
Q

Inclusion body myositis

A

Slowly progressive over years
Asymmetrical
Proximal + long finger extensors + knee extensors + dysphagia (cricopharyngeal)

CK only 10-15 fold

Male > Female
>50 years
cN1A Ab

Biopsy: “invasion” of muscle fibres, amyloid deposition

Slowly progressive, wheelchair within 10-15 years
Does not respond to Immunosuppression.

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9
Q

Anticentromere Ab

A

90% of LcSSc, associated with risk of PAH

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10
Q

Anti-Scl 70 (DNA topoisomerase 1)

A

dcSSc, risk of ILD

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11
Q

Anti-RNA Pol III

A

DcSSc, associated with rapidly progressive skin disease and high risk of scleroderma renal crisis

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12
Q

Anti-U3-RNP

A

dcSSc, associated with poor outcome and skeletal involvement

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13
Q

Pattern of arthritis is SSc

A

12-60% of patients with SSc will have joint involvement.
- Hand and wrist predominance, often affects DIP

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14
Q

Significance of tendon friction rubs

A

Can be felt or heard over a tendon, indicator of active disease.

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15
Q

Raynauds Phenomonon

A

Occurs in 95% of SSc.
Earliest manifestation
If you have Raynauds, an associated SSc Ab and nailfold capillary changes - 80% chance of developing SSc.

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16
Q

Scleroderma Renal Crisis:

A

5% of patients.
Anti-RNA Pol III gives increased risk
Use of glucocorticoids gives increased risk

Clinical: Hypertensive crisis

Tests: Micro-angiopathic haemolytic anaemia.

Tx: ACE inhibitor to control BP (captopril), continue even in the setting of rising Cr and dialysis as late improvement may occur.
Prophylaxis: Calcium channel blockers

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17
Q

Lung Involvement:

A

ILD in 50% of patients with dcSSc (85% if anti Scl 70 positive)
Pattern: NSIP > UIP
Tx: Nintedanib now approved for SSc with ILD

PAH in 10% of patients (more likely in anti-centromere +ve lcSSc
- WHO Group 1 with function class II-iV can have treatment with vasodilating therapy.

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18
Q

Anti-HMG Co-R AB

A

Immune mediated Necrotising myopathy
- Severe myopathy
- no extra-muscular involvement.

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18
Q

Treatment of PAH

A

Endothelin R antagonists:
- Bosentan, Ambrisentan (selective)
- Macitentan (non-selective)

PDE-5 Inhibitors
- Sildenafil, Tadalafil

Riociguat

Prostatcycline antagnosist
- iloprost, epoprosteonol

Combination therapy with ambrisentan and tadalafil better than mono-therapy.

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19
Q

Anti-Jo 1

A

Anti-synthetase Syndrome

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20
Q

Anti-Mi2

A

Classical Dermatomyositis

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21
Q

Anti-MDA5

A

DM with rapidly progressive ILD

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22
Q

Anti-SRP

A

Immune mediated Necrotising myopathy
- Severe myopathy
- no extra-muscular involvement.

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23
Q

Mixed Connective Tissue Disease

A

Features of at least 2 of:
- SLE, Polymyositis, SSc

Positive anti-U1-ribonucleoprotein (anti-U1-RNP)

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24
Q

Spondyloarthpropathies

A

Axial Spondyloarthropathies:
- Non-Radiographic SpA
- Radiographic SpA = Ankylosing Spondylitis

Peripheral Spondyloarthropathies:
- Reactive Arthritis
- Psoriatic Arthritis
- Enteric or IBD associated arthritis

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25
Q

Pathophysiology of Spondyloarthropathies

A

T cell Activation with Th17 phenotypes
- TNF-Alpha
- IL1, IL-17, IL23

Enthesitis –> bone erosion –>new bone formation. Causing boney proliferation and destruction.

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26
Q

HLAB27

A

Found in 6% of northern europeans.

Only 5% of those carrying it will develop at SpA, and not all those with SpA have it.

Sensitivity varies:
- 95% in AnkSpon
- 50-75% on IBD-Arthritis
-

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27
Q

Ankylosing Spondylitis Peripheral

A

Enthesitis

Asymmetical, Large joint Oligoarthritis (hips, shoulders)

Dactylitis uncommon

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28
Q

Ankylosing Spondylitis Extraarticular manifestations

A

Opthal: Anterior Uveitis (unilateral, recurrent)

Cardiovascular: Aortic valve disease (regurgitation), conduction disease

Pulmonary: Restrictive lung disease, rarely apical lung fibrosis

Bone: High risk of vertebral fractures

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29
Q

Psoriatic Arthritis

A

Occurs in 10-40% of patients with psoriasis.

Skin psoriasis occurs first or co-occurs in 90%.
therefore 10% of cases occur before skin (up to 10 years in advance)

No correlation between extent of joint involvement and severity of skin disease.

Nail involvement (pitting and onycholysis) is a risk factor for joint disease.

5 patterns:
- symmetrical polyarthritis
- asymetrical oligoarthritis
- DIP predominant
- spondyloarthritis
- arthritis mutilans

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30
Q

Psoriatic Extra-articular manifestations

A

Opthal: Conjunctivitis more common than uveitis

Skin and nail changes

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31
Q

IBD Associated Arthritis

A

4-46% of patients with IBD.

3 patterns:
- Sacroilitis (most common), strongly associated with HLAB27, not related to bowel flare.

  • Acute polyarticular peripheral arthritis, knees most commonly involved, may parallel exacerbations of bowel disease.
  • Chronic polyarticular peripheral arthritis, less than 5%, not correlated with bowel activity.
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32
Q

Reactive Arthritis

A

Occurs within 2 weeks following certain GI or GU infections.

Chlamydia trachomatis, Ureaplasma urealyticum in the urethra.

Campylobacter, E Coli, Salmonella, Shigella and Yersinia in the intestine.

Asymmetrical monoarticular or oligoarticular.
- Knee, ankle and wrist most common

Enthesitis very common 90% - achilles tendonitis or plantar fasciitis.

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33
Q

Management of axial spondyloarthritis

A

Physical Therapy
NSAIDs
Anti-TNF: Infliximab, Adalinumab, Golimumab, Certolizumab, Etenercept
IL-17A inhibitors: Secukinumab, Ixekizumab
JAKi: Upadacitib

DMARDS for peripheral arthritis: Sulfasalazine and Methotrexate.

All have RCT evidence of both radiographic SpA and non-radiographic SpA. No head to head studies, choice depend on patient factors.

34
Q

Role of non-biological DMARDS like methotrexate in Ank Spon?

A

These is NO ROLE in axial arthritis, and does have some role in peripheral arthritis.

35
Q

Diagnostic Criteria for Ank Spon

A

New York Criteria:

Clinical:
- Restriction in lateral or forward flexion of spine
- reduced chest expansion
- Inflammatory lower back pain > 3 months

Radiological:
- Bilateral grade 4 sacro-ilitis on XR
- Unilater grade 3-4 sacro-illitis on XR

BUT this is now old, terminology is now “axial spondyloarthritis” which is split into radiographic and non-radiographic.

36
Q

IL-17A Therapy

A

Secukinumab and Ixekizumab:

Increased risk of UNcomplicated fungal infections (candida)

37
Q

JAK Inhibitor therapy for SpA

A

Upadacitinib
- Selective JAK inhibitor (JAK1, JAK3, less JAK2)

Risk of viral infection esp Herpes Zoster

38
Q

Diagnosis of Psoriatic Arthritis

A

1 - Presence of MSK inflammtation (either peripheral arthritis, axial arthritis or enthesitis)

2 - Plus:
- Skin psoriasis
- Family history of psoriasis
- Dactylitis
- Nail changes (pitting, onycholysis)
- RF negative
- Evidence of juxta-articular bone formation f XR

39
Q

Treatment options in psoriatic arthritis

A

NSAIDs and Physical Therapy - Symptom benefit

Local Corticosteroid injections for peripheral arthritis.

csDMARD: Sulfasalazine, MTX, Leflunamide. Not for axial disease.

Anti- TNF

Anti IL17A: Secukinumab, Ixekizumab

anti- IL12/23: Ustekinumab (p40 subunit)

JAK1/3i: Tofacitinib

anti-IL 23: Guselkumab (p19 subunit)

40
Q

Comparison of antiTNF Adalinumab with antiIL17A (Secukinumab, Ixekizumab)

A

Similar results for arthritis.

Anti-IL17A superior for skin.

41
Q

Is there a role for co-prescription of csDMARD and biologic DMARD?

A

No.
SEAM Trial.
TNF mono-therapy as effective as TNF + MTX.

42
Q

Giant Cell arteritis epidemiology

A

Female>male. Peak age 70-79 (80% of patients are > 70)

Association with HLA DRB1*04

Association with PMR:
- 50% of GCA will have PMR sx
- 10-15% of PMR will get GCA as some point.

43
Q

How to differentiate between CRAO and NAAION

A

Both present in older people with vascular risk factors.
Both present with acute monocular vision loss.
On exam : Both will have RAPD.

The differentiation is on fundoscopic examination:
- NAAION will show optic disc swelling
- CRAO will show cherry red spot

Management:
- NAAION, vascular risk factor management and exclude AAION
- CRAO - this is a stroke, work up and manage as per stroke

44
Q

Imaging in GCA

A

TA US: Halo sign has 90% specificity, 70% sensitivity.
-Useful for ruling out those with low pre test probability

CTA/MRI/PET may be considered to assess for large vessel involvement.

45
Q

GCA management

A

No visual sx –> PO steroids

Visual sx –> IV MP 3 days then PO

46
Q

GiACTA Study for GCA

A

Tocilizumab with steroids resulted in

47
Q

ANCA vasculitis - GPA vs MPA relapse rate?

A

Regardless of the clinical presentation - PR3 positive disease is more likely to relapse than MPO positive disease.

48
Q

CYCLOPS TRIAL

A

For treatment of ANCA Vasculitis:

PO and IV Ciclophosphamide are equivalent

49
Q

RAVE Study

A

For treatment of severe GPA/MPA

Rituximab induction vs PO Cyclophosphamide Induction

Rituximab non-inferior to cyclophosphamide - many would choose rituximab due to better side effect protocol.

50
Q

Role of PLEX in ANCA associated vasculitis?

A

PEXIVAS Trial showed no benefit of PLEX for treatment of severe GPA/MPA including in the subset of people with pulmonary haemorrhage.

PLEX MAY have a role in:
- Double positive ANCA Anti-GBM disease
- Severe renal impairment (controversial)
- Severe pulmonary haemorrhage (contraversial)

51
Q

Avacopan

A

Avacopan is a C5a Receptor inhibitor.

ADVOCATE trial compared Avacopan vs prednisolone taper in ANCA associated vasculitis.

Avacopan was non-inferior to PNL at 26 weeks and superior to PNL at 52 weeks.

52
Q

Treatment of non-Severe MPA/GPA?

A

Methotrexate + low dose pred

53
Q

Remission maintenance for MPA/GPA?

A

Relapse is common, more common with PR3+ than MPO+

Traditionally Aza, MTX, Cyclophosphamide used.

Rituximab 6 monthly infusions is superior for remission maintenance.

54
Q

Remission maintenance for MPA/GPA?

A

Relapse is common, more common with PR3+ than MPO+

Traditionally Aza, MTX, Cyclophosphamide used.

Rituximab 6 monthly infusions is superior for remission maintenance.

55
Q

Treatment of EGPA?

A

Can be ANCA negative or ANCA MPO positive (is almost never PR3 positive)

Mepolizumab (Anti-IL5) is superior to placebo but prognosis is still poor.

56
Q

Rheumatoid Arthritis HLA

A

Shared epitope = a 5 amino acid sequence commonly encoded by the HLA DR locus, especially HLA-DRB101 and HLA-DRB104

57
Q

Risk factors for RA

A

HLA DRB1*01/04
STAT4
PADI
PTPN22
Smoking
Periodontitis
Gut dysbiosis
Anti-CCP

58
Q

Key Cytokines in RA

A

IL-1, IL-6, TNF

59
Q

Key Cell in RA

A

MAcrophage, T cell, B cell
PMN is the most abundant cell in the synovial fluid

60
Q

XR features of RA

A

Joint space narrowing
subchondral osteopenia
DIP sparing

61
Q

Rheumatoid factor

A

Ab against Fc portion of IgG

Utility in RA - RF positive disease is associated with more rheumatoid nodules, vasculitis, and worse prognosis.

Highly associated with RA, Sjogren Syndrome, Cryoglobulinaemia.

Also seen in non-rheumatic disease:
- Hepatitis B and C
- Endocarditis
- Viral infection
- Other chronic infections

62
Q

AntiCCP/ACPA

A

High specific for RA (90%)
Predictive or developing RA in asymptomatic and early undifferentiated arthritis.

Sensitivity 70%

Marker of severe and erosive disease.

63
Q

Methotrexate MOA

A

Inhibits purine (adenine and guanine) nucleotide synthesis by inhibition of enzyme dyhydrofolate reductase (DHF).

64
Q

Methotrexate adverse effects?

A

Pancytopenia
Liver Transaminitis
Pneumonitis

Give Folinic Acid rescue if sepsis + leukopenia

65
Q

Leflunomide MOA

A

Inhibits pyrimidine (cytosine, thymine, uracil) synthesis.

66
Q

Leflunomide adverse effects?

A

Pancytopenia, transaminitis, pneumonitis.

Cholestyramine rescue if sepsis and leucopenia.

66
Q

Leflunomide adverse effects?

A

Pancytopenia, transaminitis, pneumonitis, diarrhoea

Cholestyramine rescue if sepsis and leucopenia.

67
Q

Methotrexate and Leflunomide

A

Think of the two as brothers:
MTX - purine synthesis
LFN - Pyrimidine synthesis

Same side effect profile, but LFN more likely to cause diarrhoea.

Rescue if sepsis and leukopenia:
- MTX - Folinic Acid
- LFN - Cholestyramine binds LFN

68
Q

Etanercept

A

Anti TNF however is a soluble TNF receptor - structurally it is a Fc portion of human Ig fused to a TNF receptor.

Weekly subcut injection, shorter halflife of 4 days.

69
Q

Infliximab vs Adalinumab

A

Infliximab is a chimeric (75% humanised, 25% murine) mAb, Adalinumab is 100 humanised mAb.

Infliximab has halflife of 10 days and is given IV 8 weekly

Adalinumab has a halflife of 14 days and is given sub cut fornightly.

70
Q

Precautions for TNF blockade?

A

TB
Congestive heart failure
Demyelinating disease
Lymphoma
Lupus like syndrome of ANA/dsDNA positive
Cancer in last 5 years

71
Q

Adverse effects of TNF blockage?

A

Infections (normal bacterial)
Herpes Zoster
Chronic HSV (ophthalmic)
Melanoma

72
Q

Immunosuppression drugs and risk of skin cancer?

A

TNF blockage - melanoma

Thiopurines - non-melanoma SC

73
Q

Management of TB if wanting to start anti-TNF?

A

Latent TB:
- Isoniazid for 4-6 weeks before starting TNF, and continue for 9 months treatment.
- Latent TB is not a contraindication to anti-TNF
- liver enzyme derangement is a common problem with isoniazid

Active TB:
- Treat with standard therapy RIPE
- Should have at least 2 months treatment by a TB speciality prior to anti-TNF therapy

74
Q

Tocilizumab

A

iL-6R mAb (Blockage)

A/E:
- LFT derangement, especially in combo with MTX
- Increased total and HDL(but decreased CRP leads to decreased CVS risk)
- Diverticular perforation (rare)

75
Q

Rituximab

A

-imab (chimeric Ab)

IV Infusion

90% of B cells depleted in 3 months (measure CD 19+ levels)

Does not deplete early pre-B cells or Plasma cells, therefore no fall in Ig initially.

B cells are restored between 6-18 months later.

76
Q

Abatacept

A

CTLA-Ig fusion protein, therefore it acts as CTLA would do. Binds to B7 (CD80/86) on APC and prevents B7 binding to CD28 (co-stimulation).

This is opposite to the effect of Anti-CTLA4 mAb used in cancer immunotherapy.

77
Q

Tofacitinib

A

Inhibits Jak 1 and Jak 3

Blocking the JAK-STAT pathway will block signalling of multiple cytokines, therefore these are more immunosuppression than IL inhibitors.

Risk of herpes zoster reactivation. Give shingrix vaccine.

Other JAK inhibitors:
- Bari (JAK 1/2)
- Tofa (JAK1/3)
- Upa (JAK1) more selective

78
Q

RA in pregnancy:

A

Methotrexate - cease >6 weeks prior to conception, cannot use if breastfeeding (same for leflunamide - brothers)

Sulfasalazine, low dose pred, and Aza are all safe to continue.
- remember small increase risk of cleft lip with prednisolone.

Certalizumab does not cross placenta and therefore can be used.
- other anti-TNF cannot be used.

79
Q

Management of RA drugs peri-operatively:

A

csDMARDs can be continued through the peri-operative period.

bDMARDs should be held. Schedule surgery at the end of the dosing interval for that medication and hold until external wound healing is complete (usually 2 weeks)

JAKi - hold for 3 days pre-operatively .

80
Q

Perioperative management of patients on glucocorticoids:

A

HPA suppressed = >20mg prednisolone for 3 weeks, or cushingoid appearance.

Intermediate HPA suppressed = between 5-15mg prednisolone for > 3 weeks. Need to do AM cortisol and then ACTH testing to assess HPA axis.

HPA non-suppressed = Any dose of steroid for <3 weeks, or <5mg day doses for any duration. These patients will not be HPA suppressed.

80
Q

Perioperative management of patients on glucocorticoids:

A

HPA suppressed = >20mg prednisolone for 3 weeks, or cushingoid appearance.

Intermediate HPA suppressed = between 5-15mg prednisolone for > 3 weeks. Need to do AM cortisol and then ACTH testing to assess HPA axis.

HPA non-suppressed = Any dose of steroid for <3 weeks, or <5mg day doses for any duration. These patients will not be HPA suppressed.

81
Q

Urate lowering therapy

A

Allopurinol
- Competitive inhibitor of XO
- Risk of DRESS with HLAB 5801 (common in Han Chinese)

Febuxostat
- non-competitive inhibitor
- Use in Han Chinese
- Increased risk of cardiovascular events

Basic Physician Training Written Prep (20 decks)

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