Rheumatology Flashcards

1
Q

3 antibodies in antiphospholipid syndrome

A
  • Anti-cardiolipin
  • Lupus anticoagulant
  • Anti Beta-2 glycoprotein
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2
Q

What is Felty’s syndrome

A

Splenomegaly
Leg ulcers
neutropenia

High risk triad seen in rheumatoid

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3
Q

Cardiac complication of rheumatoid arthritis

A

constrictive pericarditis

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4
Q

Which deletion mutation is protective for Ankylosing spondylitis

A

ERAP 1 deletion - as this protein is required to present peptides to HLA B27

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5
Q

Antibody in Scleroderma renal crisis

A

RNA polymerase 3

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6
Q

Management of GCA

A
  1. Steroids
    - IV methyl pred 3 days if visual symptoms.
    - 1mg/kg pred up to 60mg with a slow wean.
  2. Long term prophylaxis
    - Tocilizumab
    - Methotrexate
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7
Q

Anti SRP association

A

Necrotizing myositis

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8
Q

Anti TIF 1 association

A

Myositis with high risk malignancy

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9
Q

Anti MDA5/CADM

A

Rapidly progressive DM.

ILD, less muscle involvement.

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10
Q

Symptoms of anti-synthetase syndrome

A

Anti-Jo (anti-tRNA), myositis, Raynaud’s, mechanics hands, heliotrope rash, ILD, non-erosive arthritis.

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11
Q

Risk factor for recurrence of reactive arthritis

A

HLA B27 gene

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12
Q

Lofgrens syndrome

A

Seen in Sarcoidosis

  • swollen ankles
  • erythema nodosum
  • bilateral hilar lymphadenopathy
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13
Q

Which arthropathy commonly involves the enthesis

A

Reactive arthritis

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14
Q

DFS70 significance

A

indicates patient does NOT have systemic autoimmune rheumatic disease

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15
Q

All rheumatological diseases have NSIP pattern of ILD except

A

RA: Has UIP pattern

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16
Q

Environmental triggers for RA

A

Mucosal inflammation:

Gut microbiome
Periodontitis
Lung mucosa inflammation - smoking.

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17
Q

Significance of RF positive rheumatoid arthritis

A

More severe disease if RF positive

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18
Q

What is RS3PE

A

Remitting seronegative, symmetric synovitis with pitting oedema
“boxing glove appearance”
Responds well to steroids

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19
Q

Hand X-ray in rheumatoid arthritis

A
  • Symmetrical
  • *PIP and MCP joints involved, DIPs spared.
  • subchondral cysts and erosions
  • *juxta-articular osteoporosis
  • *ulnar deviation at MCP, radial deviation at wrist
  • boutonniere, hitchhikers thumb and swan neck deformity
  • erosion of medial distal radius

Other - atlantoaxial subluxation

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20
Q

Hand X-ray in Gout

A
  • Podagra - 1st metatarsophalangeal joint
  • usually a oligoarthropathy
  • joint effusion
  • punched out erosions with over-hanging edge
  • tophi
  • double halo sign on US

Note- there is NO osteopenia

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21
Q

Hand X-ray in Pseudogout

A
  • involves knee, wrist, elbow, shoulder
  • if hand - wrists and MCPs only
  • features of osteoarthritis in non-weight bearing joints
  • chondrocalcinosis
  • subchondral cysts

Single halo on US as calcifications are within the cartilage itself

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22
Q

Hand X-ray in Psoriatic arthritis

A
  • distal joint predominance
  • symmetric polyarthropathy
  • asymmetric oligoarthroapthy
  • enthesitis
  • *pencil in cup erosions
  • joint subluxation
  • *periostitis - new bone formation/ thickening
  • dactylitis - sausage finger
  • athritis mutilans - telescoping fingers

Other:

  • nail involvement
  • skin psoriasis
  • uveitis/ conjunctivitis
  • IBD
  • prostatitis
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23
Q

Hand X-ray in Psoriatic arthritis

A
  • distal joint predominance
  • symmetric polyarthropathy
  • asymmetric oligoarthroapthy
  • enthesitis
  • *pencil in cup erosions
  • joint subluxation
  • *periostitis - new bone formation/ thickening
  • dactylitis - sausage finger
  • athritis mutilans - telescoping fingers

Other:

  • nail involvement
  • skin psoriasis
  • uveitis/ conjunctivitis
  • IBD
  • prostatitis
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24
Q

Hand X-ray in reactive arthritis

A

Triad - arthritis, conjunctivitis, urethritis (cervicitis in women)

  • HLAB27 positive
  • lower limbs > upper limbs
  • asymmetric
  • Enthesitis
  • floating osteophytes
  • *juxta-articular osteoporosis
  • *periostitis - new bone formation/ thickening
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25
Q

Hand X-ray in reactive arthritis

A

Reiters Triad - arthritis, conjunctivitis, urethritis (cervicitis in women)

  • HLAB27 positive
  • lower limbs > upper limbs
  • asymmetric
  • Enthesitis
  • floating osteophytes
  • *juxta-articular osteoporosis
  • *periostitis - new bone formation/ thickening
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26
Q

RA remission score

A

DAS28 <2.6

ACR20/ ACR50/ ACR70

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27
Q

Cytokine involved in Gout

A

IL-1

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28
Q

Hand X-ray in Osteoarthritis

A
  • Joint space narrowing
  • Osteophytes
  • Osteosclerosis
  • erosions and subchondral cysts
  • synovitis
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29
Q

Hand X-ray in lupus/SLE

A
  • correctable ulnar subluxation
  • no erosions

Also called Jaccoud’s arthropathy

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30
Q

Probenecid contraindications

A

Dont use in PMHx of renal calculi

Increases concentration of ABx through inhibiting tubular secretion

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31
Q

Drugs which cause Lupus

A

TNF inhibitors

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32
Q

Cardiac complications of Lupus

A

Pericarditis
Cardiomegaly
Conduction defects
Libman-Sacks (sterile) endocarditis

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33
Q

When to immunosuppress in lupus nephritis

A

Class 3,4,5.

Do not immunosuppress in 1,2 (too early) or 6 (too late)

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34
Q

Immunosuppression agents in lupus nephritis - in order

A
  1. IV pulse steroids
  2. Mycophenolate
  3. Belimumab (NOT RITUX) - Blocks B activating factor (BAFF)
  4. Cyclophosphamide/ Azathioprine
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35
Q

Genetic associations in Scleroderma

A

HLADRB1

STAT4

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36
Q

Other name for Anti-Scl70

A

Anti- topoisomerase

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37
Q

Best ACE-i for Scleroderma renal crisis

A

Captopril

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38
Q

Immune marker for inclusion body myositis

A

anti-CN1A

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39
Q

extra-muscular features of Myositis

A

Gottron’s papules
Poikiloderma (shawl sign)
Heliotrope rash

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40
Q

HLA type in Ankylosing spondylitis

A

HLAB27

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41
Q

Extra-articular manifestations of ankylosing spondylitis

A
  • anterior uveitis
  • IBD
  • psoriasis
  • aortic regurg
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42
Q

Second line Mx for ankylosing spondylitis (after NSAIDs, steroids, non-pharm)

A

TNF- alpha blockers - Adalimumab, Etanercept, Infliximab, (Golimumab for non-radiological AS)
IL-17 inhibitors - Secukinumab

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43
Q

Artery which causes vision loss in GCA

A

posterior ciliary artery

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44
Q

Management of PMR

A

15mg/day pred for 4 months then wean

45
Q

Leflunomide in pregnancy

A

Cease + cholestyramine washout

46
Q

First site of damage in knee OA

A

medial tibial-femoral cartilage

47
Q

Risk factors for future joint erosions in RA

A
  • number of swollen joints currently
  • number of erosions currently
  • RF/CCP/ESR/CRP levels
48
Q

beta-2-microglobulin is associated with which type of amyloidosis

A

dialysis associated

49
Q

TTR is associated with which type of amyloidosis

A

senile cardiac amyloid

50
Q

Management of Ank Spond

A
  1. Exercise
  2. NSAIDs
  3. TNF-a (NO role for DMARDs) - Cannot use in multiple sclerosis.
  4. IL-17 agents - Secukinumab
51
Q

What is rheumatoid factor?

A

It is usually an IgM directed against the FC portion of IgG

52
Q

Dietary factor most affecting the risk of Gout

A

Beer

53
Q

Pathophysiology of Vasculitis

A
  1. vasculitis is a T-cell driven process that is triggered by exposure to antigens, most probably infectious agents. Dendritic APCs activate CD4+ T cells, which recruit macrophages and monocytes to the vessel walls. induce systemic inflammation via the release of cytokines, IL1/IL6. T cells also release interferon-γ, which is a key proinflammatory cytokine of GCA.

Platelet-derived growth factor and vascular endothelial growth factor play key roles in GCA.

  1. Immune complex formation -polyarteritis nodosa, cryoglobulinaemia and Henoch-Schönlein purpura.
54
Q

Drug used for refractory moderate SLE after hydroxychloroquine and steroids

A

Belimumab (BAFF/BLYS inhibitor)

55
Q

SE of hydroxychloroquine

A

Retinopathy

56
Q

Treatment of cutaneous SLE

A

First-line treatment of skin disease includes topical agents (GC and/or CNIs) and antimalarials, with or without systemic GC.

57
Q

Treatment of Lupus Nephritis

A

MMF and cyclophosphamide are the agents
of choice for induction treatment; low-dose CYC is preferred over highdose CYC as it has comparable efficacy and lower risk of gonadotoxicity.

Refractory disease - Rituximab

58
Q

HLA associated with Rheumatoid arthritis

A

HLA-DRB1

59
Q

What is Rheumatoid factor

A

An antibody against the FC portion of IgG

60
Q

HLA for Allopurinol hypersensitivity

A

HLAB5801

61
Q

T cell type and interleukin related to HLAB27 positivity

A

IL23, TH17.

62
Q

Reiters Syndrome

A

Reactive arthritis + Conjunctivitis + Urethritis

63
Q

Drug to avoid in IBD related arthropathy

A

IL17 inhibitors - Secukinumab

64
Q

Features of Jaccouds arthropathy and diseases associated

A
  • correctable ulnar subluxation
  • no erosions

Diseases - Rheumatic fever, SLE.

65
Q

Antibidy related to neural lupus

A

Ribosomal P antibodies

66
Q

Management of antiphospholipid syndrome

A

Aspirin

Hydroxychloroquine

67
Q

Condition causing watermelon stomach

A

GAVE - gastric antral vascular ectasia - associated with scleroderma.

68
Q

Management for Raynauds/ nail vasculopathies in Scleroderma

A

Hand warmers
CCBs
Sildenafil
Bosentan

69
Q

PRES ( Posterior reversible encephalopathy syndrome) symptoms as associations.

(same as reversible posterior leukoencephalopathy syndrome and brain capillary leak syndrome.

A

Symptoms - CCCV

  • cephalgia’s
  • convulsions
  • confusion
  • visual disturbance

Associations:

  • HTN
  • Immunosuppression
  • lupus nephritis/ GN
70
Q

Pattern of myopathy in inclusion body myositis

A

distal upper limb

proximal lower limb

71
Q

HLA in statin induced myositis

A

HLADRB1 11:01

72
Q

Bechets HLA association

A

HLAB51

73
Q

ACPA (CCP) is formed by which process

A

citrulination

74
Q

Immunosuppressants safe in pregnancy

A

P - pred
A - Aza
S - Sulfasalazine (but reduces male fertility)
H - Hydroxychloroqine.

75
Q

Contraindications to use of TNF inhibitors

A
  • demyelinating disease
  • septic arthritis <12 months
  • pregnancy
  • untreated TB
  • malignancy within 10 years
  • chronic cutaneous ulceration
  • indwelling catheter
  • CCF
  • infected prosthesis
  • recurrent chest infections.
76
Q

Familial Mediterranean fever symptoms

A

intermittant fever, abdominal pain, chest pain, erysipleas like skin lesion.

77
Q

Familial Mediterranean fever treatment

A

Colchicine

78
Q

Familial Mediterranean fever cause and marker

A

Marker - SAA amyloid protein

Cause - autosomal recessive mutation in pyrin gene MEFV

79
Q

Which infection is PAN associated with?

A

Hep B

80
Q

Treatment of PAN

A

Non-infection related –> Steroids + cyclophosphamide

Infection related –> treat infection + PLEX

81
Q

Which organ system does PAN not affect?

A

respiratory system

82
Q

Extra-articular features of reactive arthritis

A
  • conjunctivitis
  • uveitis
  • urethritis
  • balanitis
  • keratoderma blennorrhagicum - (scaly palm and sole rash)
  • aortic incompetence
83
Q

Good prognostic marker in Rheumatoid

A

Acute onset with a few large joints affected only

84
Q

ACR criteria for SLE

A

MD SOAP BRAIN symptoms.
Alcopecia is NOT in the criteria

Malar rash, Discoid rash, Serositis, Oral ulcers, arthritis, photosensitive rash, Blood dyscrasias, Renal disease, ANA/ Autoantibodies, Immune markers, Neurological symptoms.

85
Q

Antibody in drug induced lupus

A

Anti-histone antibody

86
Q

AL amyloidosis features

A
Due to light chain deposition. 
deposits in kidney, heart and liver. 
Macroglossia (large tongue) 
Autonomic symptoms. 
Carpal tunnel
87
Q

AA amyloidosis features

A

Auto-immune, infection or malignancy related.

Produced by the LIVER.

88
Q

ATTR amyloidosis features

A

Deposits in the heart - particularly in elderly men.

89
Q

AF amyloidosis features

A

Inherited. Abnormal protein produced in the liver.

90
Q

Polymyositis antibody and histology

A

Anti Jo - associated with antisynthetase syndrome and ILD.

Histo - CD8 infiltration and increase MHC1 expression.

91
Q

Dermatomyositis antibody and histology

A

MDA5 - Rapidly progressive DM. ILD, less muscle involvement.
Mi2
TIF-1 –> highest risk for malignancy
Histo - wedge like infarcts and reduced capillaries

92
Q

Necrotizing myositis antibody and histology

A

Anti SRP
Anti HMGCoR
Histo - necrotic fibres with MACROPHAGES and COMPLEMENT.

93
Q

Inclusion body myositis antibody and histology

A

anti-CN1A
Histo - CD8 infiltration and increase MHC1 expression.
Ragged reg and ragged blue staining, Amyloid deposits, VACUOLES.

94
Q

Highest risk antibodies for mixed connective tissue disease

A

Anti-68 kD and A’ antibodies

U1RNP is second highest

95
Q

DMARD which affects male fertility

A

Sulfasalazine

96
Q

4 symptoms of IgA disease (HSP)

A
  1. Palpable purpura in patients with neither thrombocytopenia nor coagulopathy
  2. Arthritis/arthralgia
  3. Abdominal pain
  4. Renal disease
97
Q

Rheum condition with highest risk of Lymphoma

A

Sjogren’s

98
Q

Risk factors for ILD progression in Scleroderma

A
Disease duration <4 years
Diffuse cutaneous systemic sclerosis
Pulmonary function tests
- FVC <65%
- DLCO <55%
- >10% change
HRCT
- Interstitial lung disease affecting >20% of the lung
Serology
- Anti-topoisomerase I (anti-Scl-70) antibodies
99
Q

Most effective treatment for scleroderma ILD

A

Mycophenolate (better than cyclophosphamide)

100
Q

Single gene mutation that causes lupus (rare)

A

TREX1

101
Q

First cell involved in GCA

A

Dendritic cell - activates TLR and CD83

102
Q

gene associated with PAN

A

ADA2

103
Q

Treatment for Granulomatosis with polyangiitis

A

Cyclophosphamide
Azathioprine maintenance - 3 years
Prednisolone
Rituximab if treatment resistant

104
Q

Colchicine can treat which manifestations of Familial Mediterranean fever

A

Fever
Amyloidosis
Serositis

–> Does not work well for arthralgias

105
Q

Which feature of Rheumatoid arthritis is worsened by methotrexate

A

Rheumatoid nodules

106
Q

When can Rituximab be used in ANCA vasculitis

A
  1. PBS approved for induction

2. Trial proven for maintenance (not on PBS for maintenance)

107
Q

Which infection is increased by the use of TKIs

A

herpes zoster

108
Q

Rheumatoid antibodies - most specific, best NPV, best for monitoring disease activity.

A

most specific - anti Sm
best NPV - ANA
best for monitoring disease activity - dsDNA