Rheumatology Flashcards

1
Q

anti Sm

A

SLE

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2
Q

anti SSA/Ro & SSB/La

A

Sjogren syndrome

subacute cutaneous lupus erythematosus

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3
Q

anti dsDNA

A

SLE - prone to nephritis

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4
Q

anti U1RNP

A

mixed connective tissue disease (MCTD)

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5
Q

anti Jo-1, PL-7, PL-12

A

antisynthetase syndrome

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6
Q

anti Mi-2

A

dermatomyositis

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7
Q

anti centromere

A

limited cutaneous systemic sclerosis

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8
Q

anti Scl-70 (anti-topoisomerase)

A

diffuse cutaneous systemic sclerosis

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9
Q

associated complications?

  • anti centromere
  • anti Scl-70
  • anti RNA polymerase
A
  • anti centromere - primary PAH
  • anti Scl-70 = ILD
  • anti RNA polymerase - renal crisis, malignancy
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10
Q

HLA associated with:-

  • RA & SLE
  • SLE, SS, Sjogren’s
A

HLA-DRB1 - RA, SLE

HLA-DQ - SLE, SS, Sjogren’s

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11
Q

Which HLA more common in non-Caucasians with higher risk of lupus nephritis, neuropsych SLE, APLS

A

HLA-DRB11503, HLA-DRB108 in African american, Hispanic

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12
Q

How to distinguish Raynaud’s fr autoimmune CTD vs other causes?

A

Peri-ungal erythema/capillary dilatation & drop-out

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13
Q

The classes of lupus nephritis & respective management

A

Class 1: mesangial immune deposits without hypercellularity
Class 2: mesangial immune deposits with hypercellularity
Class 3: focal proliferative, <50% glomeruli
Class 4: diffuse proliferative, >50% glomeruli, segmental or global
Class 5: membranous
Class 6: advanced sclerosing lesions
Class 1&2 (no intensive tx), 3&4 (intensive tx), 5&6 (tx refractory)

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14
Q

Serology markers in SLE which correlate w disease activity

A

high dsDNA, low C3, C4

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15
Q

Serology marker in SLE which correlates w neonatal heart block

A

anti-Ro

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16
Q

anchor drug in SLE

A

Hydroxychloroquine

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17
Q

risk of Hydroxychloroquine requiring regular checks

A

Retinopathy

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18
Q

1st line choice for lupus nephritis

A

MMF

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19
Q

Haematological manifestations of SLE

A
  • TTP

- Macrophage activation syndrome (assoc in JIA); fever, high ferritin, low plt, low fibri, high tiglyceride, high AST

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20
Q

Cardiac involvement of SLE

A
  • Pericarditis

- Liebman-sachs endocarditis

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21
Q

ANA staining patterns

A
  • Speckled - non-specific
  • peripheral - SLE
  • Homogenous - SLE, RA, drug- induce lupus
  • Centromere - limited scleroderma
  • Nucleolar - diffuse sclerdoerma, SLE
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22
Q

Medications for SLE which are safe in pregnancy

A

hydroxychloroquine, azathioprine, steroids

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23
Q

When to consider systemic immunosuppressive therapy (CYC or MMF) in systemic sclerosis?

A
  • Pts w diffuse skin involvement that is severe & progressive
  • Pts w ILD
  • Pts w myocarditis
  • Pts w severe inflamm myopathy and/or arthritis
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24
Q

Risk factors leading to ILD in systemic sclerosis

A
  • early diffuse cutaneous
  • anti-Scl70
  • elevated CRP
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25
Q

Areas of skin distribution in limited cutaneous systemic sclerosis

A

below elbow & knee, face involvement & truncal sparing

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26
Q

Pharmacological mx of ILD in SS

A
  • 1st line MMF
  • CYC
  • Aza
  • Nintedanib (multi-targeted tyrosine kinase inhibitor - FGFR, PDGFR, VEGFR)
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27
Q

Pharmacological mx of PAH in SS

A

i) Endothelin receptor antagonist: ambrisentan, bosentan
ii) PDE-5 inhibitors: sildenafil, tadalfil
iii) Riociguat (stimulate guanylate cyclase -> cGMP)
iv) Severe class III/IV PAH: IV epoprostenol (prostacyclin antagonist)
v) Consider other prostacyclin analogues: iloprost
- Tx iron deficiency – better survival
- Consider lung transplantation

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28
Q

Risk factors of scleroderma renal crisis

A

RNA polymerase III Ab, tendon friction rubs

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29
Q

Usual trigger of scleroderma renal crisis

A

Steroid

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30
Q

anti HTN agent of choice for scleroderma renal crisis

A

Captopril

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31
Q

anti HTN agent to avoid for scleroderma renal crisis

A

beta-blocker

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32
Q

Non-pharmacological measures to prevent Raynaud’s

A
  • keep warm
  • avoid caffeine
  • smoking cessation
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33
Q

Pharmacological mx of Raynaud’s

A

1st: Nifedipine
2nd: Sildenafil, topical nitrate, alpha blocker, SSRI
Severe: IV iloprost

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34
Q

Gut involvement in SS

A
  • GORD 90% pts
  • bloating, small intestinal bacterial overgrowth
  • Faecal incontinence 30% - overflow diarrhoea
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35
Q

Cardiac complications in SS

A
  • Myopericarditis
  • CAD
    HTN cardiomyopathy
  • Arrhythmia (cardiac fibrosis)
  • Heart failure
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36
Q

The 4 types of inflammatory myopathies

A

Polymyositis(PM)
Dermatomyositis (DM)
iii) Inclusion body myositis
iv) Immune-mediated necrotizing myopathy (IMNM)

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37
Q

Which inflammatory myopathy M>F

A

Inclusion body myositis (IBM) M>F

PM & DM - F:M 2:1

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38
Q

Cells involved in the 4 types of inflammatory myopathies

A

DM: CD4 plasmacytoid dendritic cells
IMNM: macrophages
PM & IBM: endomysial CD4 T cells, cytotoxic CD8 T cells, myeloid dendritic cells, plasma cells

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39
Q

which complication is related to anti MDA5 & anti synthetase ab in DM/PM

A

ILD - poor prognosis

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40
Q

hyperkeratosis “mechanic’s hands”, ILD, myositis, polyarthralgia, fever & Raynaud. anti Jo-1 ab.

A

anti-synthetase syndrome

41
Q

antibodies related to Immune-mediated necrotizing myopathy

A

Anti -SRP, anti-HMGCR

*necrotising myopathy

42
Q

antibody related to classic DM with mild disease

43
Q

antibodies related to severe DM with a/w cancer

A

anti-TIF1 & anti NXP-2

44
Q

Insidious – prox leg weakness up to 5yrs, asymmetric, distal finger flexor weakness, muscle atropphy, dysphagia (cricopharyngeal muscle 1/3rd to half of pts), CK not useful to monitor disease (usually low), less responsive to immunosuppressive tx

A

Inclusion body myositis

45
Q

Gene mutations which confer risk of RA

A

DRB1, STAT4, PADI, PTPN22

46
Q

Cytokines involved in pathogenesis of RA

A

TNF, IL-1, IL-6 (Macrophage-derived)

47
Q

Predominant cells in RA synovial fluid

A

neutrophils

48
Q

Hallmark of RA joint in X-ray

A

juxta-articular osteopaenia & erosion

49
Q

Joint findings in RA

A

Small joints (sparing of DIP), symmetrical, associated nodules

50
Q

Joint findings in SLE

A

passively correctible deformity (Jaccoud arthropathy) - minimal erosion in imaging

51
Q

Joint findings in gout

A

tophi, DIP not spared, punched out erosions in imaging

52
Q

Joint findings in psoriatic arthritis

A

erosion + new bone = pencil in cup, Nail change, sausage fingers

53
Q

Joint findings in OA

A

Heberden’s nodes (osteophytes), subchondral sclerosis

54
Q

What are the abs involved in RA?

A

IgM against Fc portion of IgG

55
Q

Non-rheumatic dx with RF +ve

A

Hep C, Hep B, Viral infection

56
Q

Best predictors of severity of RA

A

Erosions

anti-CCP (but does not reflect current disease activity thus not used to assess progress)

57
Q

Factors indicating current activity of RA

A

CRP, ESR, swollen joint count

58
Q

Anchor drug in RA

A

Methotrexate

59
Q

Mechanism of action of MTX

A

irreversibly binds to & inhibits dihydrolate reductase

60
Q

Rescue therapy for MTX toxicity

A

Folinic acid (leucovorin)

61
Q

Mechanism of action of folinic acid

A

supplies cofactor blocked by MTX, displaces MTX fr intracellular binding sites, restores active folate stores for DNA synthesis

62
Q

Rescue therapy for leflunomide toxicity

A

Cholestyramine

63
Q

Mechanism of action of leflunomide

A

Inhibits pyrimidine biosynthesis

64
Q

Precautions of toxicity for TNF blocker

A

TB risk, demyelination (hx of optic neuritis), lymphoma, melanoma & non-melanoma, CHF (esp stage IV NYHA)

65
Q

What is the tx for latent TB before commencing anti-TNF

A

Isoniazid for 4-6 weeks

66
Q

Mechanism of action & main risks of tofacitinib vs baracitinib

A

tofacitinib - JAK 1/3 inhibitor, high infection risk

baracitinib - JAK 1/2 inhibitor, VTE risk

67
Q

Use of Yttrium in RA

A

radioisotope ->chemical synovectomy

68
Q

Features of Spondylarthritis

A
  1. Onset age <40
  2. Insidious onset
  3. Improvement w exercise
  4. No improvement w rest
  5. Nocturnal pain that improves on waking
    (Responds to NSAIDs)
69
Q

Axial features of spondyloarthropathy

A
  • Inflammatory back pain
  • Buttock pain - alternating, poorly localised
  • Restriction in spinal movement
70
Q

Areas affected in enthesitis

A

Achilles, plantar fascia, chest wall, pelvic brim

71
Q

Extra-articular Involvement of RA

A
  • Acute uveitis
  • IBD
  • Osteopenia
  • Neurological: cauda equina, fracture, A-a subluxation
  • CVD risk, aortic regurg, conduction disturbance
  • Chest wall restriction, apical fibrosis
  • Secondary amyloidosis
72
Q

1st line mx for spondyloarthritis

A

NSAIDs, exercise, physical tx

73
Q

2nd line tx for axial spondyloarthropathy

A

TNF blocker, IL-17 blocker

*no role for local steroids & DMARDs for axial SpA

74
Q

5 distinct patterns of psoriatic arthritis

A
  1. Asymmetric oligo/mono
  2. Polyarthritis - symmetric
  3. Spondylo-arthritis - axial, AS-like
  4. DIP with nail disease
  5. Arthritis mutilans
75
Q

Main risk factor of GCA

76
Q

HLA association with GCA

A

HLA-DRB1*04

77
Q

Pathophysiology of GCA

A

Dendritic cell in adventitia activated –> recruit CD4 T cells & macrophages & migrate to media
IL-6 (->Th17 effect) & IFN-gamma (macrophage activation -> granuloma)

78
Q

Specific symptom suggesting GCA

A

jaw claudication

79
Q

Extra-cranial sx of GCA

A

aortic involvement (aneurysm), subclavian stenosis/occlusion, cough

80
Q

Options of mx in GCA

A

Glucocorticoids

  • GCA without visual sx - Pred 40-60mg
  • GCA with visual sx -> IV methyl pred for 3/7 then Pred

Tocilizumab - Anti-IL6

81
Q

Sjogren’s risk to which malignancy?

A

Non-Hodgkin lymphoma

82
Q

Mechanism of action of sulfasalazine

A

suppress TNF alpha, induces apoptosis of inflamm cells

83
Q

Mechanism of action of hydroxychloroquine

A

suppress TNFalpha, induces apoptosis of inflamm cells

84
Q

Medication to avoid with MTX

A

trimethoprim

85
Q

Rheumatic meds which are safe for pregnancy

A
  • glucocorticoids
  • azathioprine
  • cyclosporin/tacrolimus
  • sulfasalazine
  • anti TNFs
  • IVIG
86
Q

Which TNF blocker safe to be used throughout pregnancy?

A

certolizumab

87
Q

When to stop infliximab/adalimumab & Etanercept during pregnancy?

A

Stop infliximab/adalimumab at 20 weeks

Etanercept at 30-32 weeks during pregnancy

88
Q

Which rheumatic med is NOT recommended for men trying to conceive?

A

cyclophosphamide

*MMF, MTX, LFL, HCQ can be used

89
Q

Inborn errors of metabolism leading to primary urate overproduction

A
  • Accelerated purine synthesis (PRPP synthase enzyme hyperactivity)
  • Impaired purine salvage (HGPRT1 deficiency) - Lesch-Nyhan syndrome
  • Hereditary defects of energy metabolism - glucose-6 phosphatase deficiency
90
Q

Food to avoid in hyperuricaemia

A
  • Seafood & red meat (high in purine)
  • Fructose (alters hepatic metabolism to increase purines)
  • Alcohol (increases ATP degradation & purine turnover)
91
Q

Most common sites of subcutaneous tophus

A
Fingers -IP joints damaged by osteoarthritis.
Wrists
Olecranon bursae
Ulnar aspect of the forearm
Helix of the ear
92
Q

Joint aspiration findings for dx of gout

A

intra-cellular needle-shaped, negatively birefringent crystals.

93
Q

Imaging options for gouty arthritis

A
  • X-ray: Overhanging edge with sclerotic margin
  • U/sound: double contour sign, hyperechoic aggregates, tophi
  • Dual energy CT: Erosions, MSU crystal deposition
94
Q

HLA associated with Allopurinol hypersensitivity syndrome

A

HLA B*5801 (Han Chinese, Thai)

95
Q

Which part of nephron is involved in urinary excretion of uric acid by uricosuric agents

A

inhibit URAT1 & GLUT9 in prox tubule

96
Q

what’s the duration for gout flare prophylaxis?

97
Q

antibody associated with drug-induced lupus

A

anti-histone antibodies

98
Q

medications a/w drug-induced lupus

A
  • hydralazine
  • procainamide
  • isoniazid
  • methyldopa
  • chlorpromazine
  • quinidine
  • minocycline