Dunedin- Rheumatology Flashcards

1
Q

What is the larger contributor for a risk of rheumatoid arthritis ?

A

(1) Smoking (2) genetics

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2
Q

What HLA is associated with RA?

A

HLA-DRB1
MHC Class 2, Chromosome 6

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3
Q

Describe some genes associated with RA

A

PTPN22 SNP- together with HLA-DRB1 gives 50% genetic risk

IL2RA (chromosome 10p15)
PTPN2 (Chromosome 18p11)
Chromosome 12q24 region

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4
Q

Describe Mucosal origins hypothesis

A
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5
Q

Describe Anti-CCP

A

As sensitive as, and more specific than, IgM RF in early and fully established RA

Marker of erosive disease

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6
Q

What other things can cause rheumatoid factor to be high?

A

Chronic viral hepatitis
infection

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7
Q

When diagnosing RA, what is the time frame?

A

> 6 weeks

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8
Q

Co-morbidities and RA treatment

A

*dose reduction MTX when eFFR <60mls/min

*Contraindication MTX, LEF liver disease or transaminitis

*Contraindication: Chronic infection

  • Consideration: recent malignancy

*Manage actively: Hep B or Hep C

*Various specific CI- VTE and JAKI, CHF, and MS with TNFI

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9
Q

What is the first line treatment for RA?

A

Methotrexate with oral prednisone with weaning. if still active ADD cDMARDS, if still active ADD bDMARDS or tsDMARDS

(1) DMARDS (methotrexate, salazopyrine, hydoxychloroquine, leflunomide)

(2) Biologics (TNFalpha inhibitors, B-cell inhibition, Anti-IL-6 co stimulatory receptor blocker)

(3)targeted synthetic tsDMARDS (Janus kinase inhibitors)

glucocorticoids, NSAIDS

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10
Q

Any “cept” eg etanercept

A
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11
Q

What age group is at risk with JAK inhibitors?

A

> 65 years

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12
Q

Can immune checkpoint inhibitors be used in RA?

A

Yes

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13
Q

What is Peresolimab?

A

Peresolimab is a humanized IgG1 monoclonal antibody designed to stimulate the endogenous programmed cell death protein 1 (PD-1) inhibitory pathway.

can be used in mod-severe RA disease

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14
Q

Methotrexate and the lungs?

A

Methotrexate causes Acute PNEUMONITIS NOT ILD

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15
Q

What is the pathophysiology of scleroderma?

A

*Initial microvascular/endothelial damage

*Initial microvascular/endothelial damage, Followed by an autoimmune response with inflammation

*Finally characterized by diffuse fibrosis

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16
Q

What is the scleroderma spectrum

A

Limited cutaneous AKA CREST

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17
Q

Describe limited cutaneous systemic sclerosis vs diffuse cutaneous systemic sclerosis

A

Limited cutaneous: CREST
- calcinosis, Raynaud’s, oesophageal dysmotility, sclerydactyl, telangiectasia + PULMONARY ARTERY HTN

Diffuse cutaneous systemic sclerosis:
- extensive skin involvement: proximal limbs and trunk.
Renal disease
pulmonary interstitial fibrosis

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18
Q

What is the most common cause of death in diffuse cutaneous systemic sclerosis?

A

Pulmonary fibrosis

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19
Q

Discuss renal crisis in Diffuse sclerosis

A

*Precipitated by steroids
*Anti RNA Polymerase III
*abrupt onset of mod-severe hypertension
*urine sediment normal or reveals only mild proteinuria with few cells or casts
* progressive renal failure

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20
Q

Describe cardiac involvement in systemic sclerosis

A

*Ischaemia including in response to cold exposure
*contraction band fibrosis
*right ventricular changes with pulmonary HTN

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21
Q

Describe GI involvement in systemic sclerosis

A

*GAVE (watermelon stomach) with chronic blood loss
*oesophageal dysmotility and reflux
*‘shrinking stomach’ with early satiety
*small bowel dysmotility with bacterial overgrowth
*malabsorption
*volvulus
*Faecal incontinence
*cachexia

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22
Q

Describe auto-antibodies in scleroderma

A

Diffuse: Scl-70 (anti-topoisomerase), RNA polyermase III, U3-RNP

Limited: centromere, Th/To, U11/U12 RNP

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23
Q

Which antibody is associated with mixed connective tissue disease?

A

U1-RNP

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24
Q

What antibody is associated with Myositis/Scleroderma

A

PMScl 100/75

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25
Q

What is the relevance of absent antibodies in scleroderma?

A

increased association with malignancy

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26
Q

Describe nail fold capillaroscopy

A
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27
Q

What is the Rodnan skin score?

A

used in scleroderma, treatment is based on it

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28
Q

Treating scleroderma

A

Scleroderma renal crisis: ACEI

Scleroderma inflammatory arthritis: Methotrexate

Skin involvement: Methotrexate, MMF, IV cyclophosphamide, HSCT

Lung disease or cardiac disease: MMF

Pulmonary arterial HTN: PDE5i, ERA (bosentan, ambrisentan)

raynauds or digital ulcers: CCB

GI: high dose PPI, promotability agents, oesophageal dilation

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29
Q

Digitial ulcers and scleroderma

A

strong association with pulmonary HTN

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30
Q

Describe spectrum of vasculitis

A
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31
Q

Out of the ANCA vasculitis, which ones produce granulmonas?

A

GPA (wegeners)
eosinophilic granulomatous w polyangitis (EGPA)

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32
Q

Differentiate between PR3-ANCA and MPO- ANCA

A

GPA: PR3-ANCA (sometimes MPA too)

MPA: MPO- ANCA

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33
Q

What lung disease is pANCA associated with vs cANCA?

A

pANCA- UIP
cANCA- NSIP

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34
Q

Urinary eosinophilia

A

interstitial nephritis NOT vasculitis

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35
Q

Erythema nodosum

A
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36
Q

Livido reticularis

A
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37
Q

Pyoderma gangrenosum

A
38
Q

Describe ANCA patterns in other disease

A

IBD can have ANCA
autoimmune hepatitis
infective endocarditis (c-ANCA)
Cystic fibrosis (pseudomonas aeruginosa)

39
Q

What drugs cause Drug-Induced ANCA?

A

Hydralazine
Carbimazole/propylthiouracil
minocycline
penicillamine

40
Q

Treating vasculitis:

A

Severe: IV Methylpred, cyclophosphamide, rituximab

Non-organ threatening: Pred + methotrexate, Aza, MMF

Maintenance of remission:
Ritux, MMF, Methotrexate, Aza, leflunamide

some evidence of mepoluzimab (IL-5), Plasma exchange, avacopan (C5a receptor inhibitor)

41
Q

How to determine severity of EGPA vasculitis?

A

(1) Age>65
(2) Renal impairment at presentation
(3) cardiac insufficiency
(4) Gastrointestinal involvement
(5) absence of ENT disease

42
Q

What is the gold standard for GCA?

A

Temporal artery biopsy >10mm sample

highly specific
reasonably useful within 2 weeks of starting steroids

43
Q

What is the biggest risk factor for GCA?

A

Age

44
Q

What is the temporal artery US sign in GCA?

A

*Halo sign
*compression test : thickened arterial wall remains visible on compression
*stenosis
*occlusion

45
Q

What is the management of GPA?

A

if vision threatened –> IV methylpred
or oral pred

methotrexate, leflunamide, tociluzimab

Large vessel involvement:
antiplatelet therapy in patients with critical/flow-limiting cerebral artery disease

46
Q

What is Anti-DFS70?

A

dense fine speckled
a type of ANA
usually a NEGATIVE associated with autoimmune rheumatic disease

47
Q

What are poor prognostic factors for SLE?

A

1) Age extremes. onset >50 years or <30 years
2) Haemolytic anaemia
3) Male
4) renal lupus within 1 year of presentation
5) neuropsychiatric lupus

48
Q

What are risk factors for SLE?

A

*Infection: CMV parvovirus, EBV
* Smoking
*Drugs- esp TNFis
* Sun exposure
* T and B cell receptor signalling PTP-N22 gene

49
Q

Describe some things involved in immunopathogenesis of SLE

A

apoptosis –> high cell turnover –> nucleic materials released from dying cells –> CRP and C1q assist removal of nucleic waste –> APC can take up the nucleic acids and present to T and B cells often in the kidney –> Neutrophil extracellular trans (NETOSIS) –> immune complex formation

50
Q

What are the main types of lupus?

A

1) Discoid lupus
2) Subacute cutaneous lupus
3) Systemic lupus with low risk of serious harm
4) Systemic lupus with risk of serious harm

51
Q

Describe the urine in systemic lupus with low risk of serious harm

A

no urinary casts, RBCs or proteinuria

52
Q

What type of arthritis does lupus cause?

A

non-erosive
small joint poly arthritis
correctable deformity (Jaccoud’s arthropathy)
tenosynovitis

53
Q

Describe symptoms of lupus with risk of serious harm

A

*Vasculitic rash
*neurology: seizures, psychosis, mononeuritis multiplex, peripheral or cranial neuropathy, organic brain syndrome, acute confusion
*lupus headache
*Endocarditis= liebmann-sachs, pericardial effusion
*renal involvement: nephritis, heavy proteinuria, reduced GFR
*pregnancy associated complications: pre-eclampsia, HELLP syndrome, neonatal heart block
*haemolytic anaemia

54
Q

describe lupus vasculitis

A
55
Q

Generally lupus spares the lung. true or false?

A

TRUE- however can get shrinking lung syndrome

56
Q

Describe lupus nephritis

A

Class 1- minimal mesangial
Class 2- mesangial proliferative
Class 3- Focal
Class 4- Diffuse

57
Q

Describe cerebral lupus

A

really bad headache. poor prognostic sign!
anxiety, depression
seizures
Focal neurological signs

investigate with PET scan and do formal cognitive testing

58
Q

What is the most common feature of lupus?

A

(1) Arthritis
(2) Rash
(3) serositis
(4) photosensitivity

59
Q

Relevant of Anti Ds-DNA in SLE

A

very specific 97%
titres correlate with disease activity
elevation correlates with lupus nephritis
can be seen in drug induced lupus

60
Q

ENA and their associations

A

Anti-Ro (60): SCLE, Sjogren’s syndrome
Anti-La: Sjögren’s syndrome and SLE
Anti- Sm: renal and neurological lupus (lupus with RSH)
AntiU1 RNP: MCTD, SLE

Anti Ro 60 is different to 52. Ro60 is specifically subcutaneous

61
Q

What drugs are likely to cause drug induced lupus?

A

high risk: procainamide, hydralazine
Low risk: PPI, anti-TNF, statins, chemotherapeutic drugs, ACEi, PTU, minocycline, methyldopa, D-penicillamine

tetracyclines

62
Q

Autoantibodies in drug induced lupus

A

Anti-histone
DsDNA in TNFi
ANCA (MPO)- hydralazine, minocycline and propylthiouracil

63
Q

What is the management of SLE?

A

flare: pred, NSAID, immunosuppressant (cytoxan, azathioprine, methotrexate,)

treating major organ involvement: cyclophosphamide, mycophenolate, calcineurin inhibitors

rituximab, obinutuzumab (both in renal lupus), belimumab , anifrolumab

JAKi, sexukinumab, ustekinumab, lenalidomide for severe cutaneous disease, targeted dendritic cell monoclonal ab: litifilimab

64
Q

What do you expect blood test in SLE?

A

Reduced CH50
Low C4 (risk factor, can be genetically determined)
C3- alternate pathway

High ESR, CRP can be normal

FBC: lymphopenia, neutropenia, thrombocytopaenia

Haemolytic

Urine; protein, granular cast, RBCs, WBCs

65
Q

Conventional DMARDS are only for peripheral SpA. True or false?

A

True

Also ustekinumab also only peripheral

66
Q

What is the diagnostic criteria of Spondylarthritis?

A

note sacroilitis can be on MRI or X-RI

67
Q

Treatment for Uveitis?

A

topical steroids

68
Q

Pathogenesis of axial spondylarthritis

A
69
Q

Infection associated arthritis

A
70
Q

What bacteria is associated with erythema nodosum?

A

campylobacter

71
Q

What does Keratoderma Blennorrhagicum look like in reactive arthritis?

A
72
Q

DISH vs Ankylosing spondylitis

A
73
Q

What test is best to look for active inflammatory lesions in Ankylosing spondylarthritis?

A

MRI- T2 for bone oedema
T1- for sclerosis, erosions and fatty lesions

however some can have positive MRI findings and be healthy

74
Q

What is gold standard for detecting structural changes (erosions) in ankylosing spondylarthritis?

A

CT scan

75
Q

Treatment of spondylarthritis?

A
76
Q

Regarding spondylarthritis, if Uveitis or IBD what drugs to use?

A

TNF inhibitors but NOT etanercept

77
Q

Regarding spondylarthritis, if psoriasis what drugs to use?

A

IL-17 eg secukinumab, ixekizumab

can use IL-12/23 –> not good for axial disease

78
Q

First like for axial spondylarthritis?

A

Should use NSAID. trial 2 different ones! if still not working then biologic

79
Q

Describe myositis specific antibody subset

A
80
Q

What lung problem is associated with Anti-synthetase syndrome?

A

Pulmonary interstitial fibrosis:

BOOP (bronchiolitis obliterates organising pneumonia) AKA Cryptogenic organising pneumonia (COP)

81
Q

Antibodies associated with dermatomyositis

A

Anti-Mi2
TIF-1y
MDA-5
Mj

82
Q

Sights of dermatomyositis?

A

heliotrope rash
gottrons papules
mechanisms hands
shawl rash
peau d’orange

83
Q

How to treat immune mediated inflammatory myopathies?

A

IV methylprednisone then oral prednisone
methotrexate (1st choice)
maybe Aza, MMF, calcineurin inhibitors

84
Q

Antibodies in anti-phospholipid syndrome

A

anticardiolipin
lupus anticoagulant
B2GP1
Anti-domain 1 B2GP1
anticomplex phosphatidylserine-prothrombin- anti-PS/PT

84
Q

Primary thromboprophylaxis of anti phospholipid syndrome

A

Low dose aspirin for:
*triple positive antibodies asymptomatic patients with no hx of VTE
*women with pure obstetric APS and positive antibodies
*Patients with SLE and one or more ACL ab

beneficial in reducing risk of arterial embolism
borderline effect in reducing VTE
as effective as low intensity warfarin

Hydroxychloroquine: in SLE
LMWH: triple positive or high risk (postpartum, post surgical, immobilisation)

85
Q

Secondary thromboprophylaxis in anti-phospholipid syndrome

A
  • if provoked can do 6 months of anticoagulation then aspirin
  • otherwise warfarin
  • CANNOT USE RIVAROXOBAN

can possibly do warfarin + aspirin if arterial thrombosis

86
Q

What is Catastrophic APS syndrome (CAPS) and how to treat it?

A

Clinically:
* overwhelming widespread thrombosis: renal, cardiac, cutaneous, cerebral, pulmonary and gastrointestinal thrombi
*multiple organ failure occurring within 1 week of presentation
* adult respiratory distress syndrome
*aPL antibodies

Pathophysiology
* acute endothelial injury, cytokine storm, systemic inflammation, and a microangiopathic thromboses

Management:
1) full dose anticoagulation (start with IV heparin)
2) high dose glucocorticoids (methylpred)
3) Plasma exchange

If infection too –> add IVIG
if SLE too –> cyclophosphamide
If MAHA too –> add rituximab
possible role for eculizumab

87
Q

Ehlers danlos syndrome associated with lens dislocation. True or false

A

false
marfans is associated with lens dislocation

88
Q

Describe discoid lupus

A

80% of lesions above the neck
scarring
depigmentation
alopecia
mucosal lesions
can progress to SLE (5-25%)

89
Q

Describe subacute cutaneous lupus (SCLE)

A

Photo-sensitive, non-scarring rash
pleurisy
oral ulceration
arthritis/arthalgia
tenosynovitis

90
Q

Describe the patterns of ANA

A

homogenous- SLE
- Anti-dsDNA and anti-histone

Speckled- SS, MCTD
- anti-Ro/La, anti-Sm, anti-RNP

Nucleolar- scleroderma (diffuse cutaneous)

Cytoplasmic- myositis