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GCUH FRACP Writtens 2020 > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

What are the typical ages of patients affected with giant cell arteritis?

A

Primarily affects people aged >50

Peaks at around age 80

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2
Q

Which arteries are typically affected by GCA? Name 5 arteries/groups of arteries.

A

External carotid branches (e.g. temporal and occipital arteries)

Ophthalmic

Vertebral

Distal subclavian and axillary arteries

Thoracic aorta

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3
Q

What is the pathogenesis of GCA?

A

Antigenic stimulation via danger pattern recognition on vessel-specific Toll-like receptors on vascular dendritic cells in artery adventitia

CD83+ activated vascular dendritic cells produce T cell-attracting chemokines (CCL19 and CCL21) drawing T cells into the media

Peripheral blood monocytes differentiate into macrophages in vessel wall and produce IL-6 and IL-1B, as well as IFy, CXCL9, 10 and 11, PDGF, FGF and VEGF

IL-6 induces T cells to differentiate into Th17 cells

Vasc smooth muscle cells express NOTCH ligands, which interact with NOTCH receptors on T cells and amplify inflammatory response, switching to synthetic myofibroblasts that proliferate in intima, narrowing vessel lumen

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4
Q

What are the 4 different clinical patterns of GCA?

A

Isolated cranial GCA (headache, jaw claudication, scalp tenderness) - most cases

Symptomatic LVV (claudication, pulseless limb) with or without cranial signs

Isolated fever or inflammatory response

Isolated PMR with vasculitis on imaging

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5
Q

Do GCA patients with LVV require higher or lower doses of steroids?

A

Higher

Typically tend to be younger, less likely to present with headaches, lower risk of visual loss

However, higher risk of relapse

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6
Q

Which arteries are typically occluded in GCA that result in visual loss?

A

Posterior ciliary arteries, resulting in anterior ischaemic optic neuropathy

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7
Q

What are the diagnostic criteria for GCA as per the American College of Rheumatology?

A

3 of 5 present:

Age at disease onset >50 years

New headache - localised

Temporal artery abnormality

Elevated ESR by >50

Abnormal artery biopsy

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8
Q

What 3 acute phase reactants would you measure in GCA, and what are the estimated optimal cut-off levels?

A

ESR - 50

CRP - 20

Platelets - 300

CRP + platelets = good diagnostic utility for GCA

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9
Q

What is the most significant long term risk of GCA?

A

Aortic aneurysm

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10
Q

As well as steroids, what medications should be considered for treatment of GCA? Name 7.

A

Aspirin - positive role in cerebrovascular disease

Methotrexate

Cyclophosphamide

Azathioprine

Leflunomide

Tocilizumab (anti IL-6)

Ustekinumab

TNFa not recommended for GCA

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11
Q

What are the mandatory (3) and additional criteria for diagnosing polymyalgia rheumatica (6)?

A

Mandatory

  • age > 50
  • aching in both shoulders
  • abnormal CRP/ESR, or both

Additional (> 4 points w/o USS, > 5 with USS)

  • morning stiffness lasting >45 mins (2 points)
  • hip pain or reduced range of motion (1 point)
  • negative RF/CCP (2 points)
  • absence of peripheral synovitis (1 point)
  • USS findings - one shoulder with subdeltoid bursitis, biceps tenosynovitis, or glenohumeral synovitis, or at least one hip with synovitis or trochanteric bursitis (1 point)
  • subdeltoid bursitis, biceps tenosynovitis, or glenohumeral synovitis in both shoulders (1 point)
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12
Q

What are the cut-offs for PMR activity score (PMR-AS)?

A

PMR-AS < 7 is low disease activity

PMR-AS > 10 defines a flare and suggests adjustment in glucocorticoid dosage

PMR-AS > 17 is high disease activity

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13
Q

How is PMR treated?

A

Prednisolone 15 mg/day for 4 weeks, then reduced by 2.5 mg every 2 weeks to 10 mg and subsequent reduction by 1 mg per month

Also can give methotrexate to use less GC, particularly to reduced osteoporotic fracture risk

Etanercept has a modest benefit in GC naive PMR patients

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14
Q

Name 5 extra-articular features associated with axial sponyloarthritis.

A

Anterior uveitis - common

IBD

Psoriasis

Apical fibrosis

Aortic regurgitation

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15
Q

What are the typical XR changes you can expect to see in the spine with AS? Name 3.

A

Squaring

Syndesmophytes (will progress to bridging syndesmophytes)

Romanus lesion (shiny corners)

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16
Q

When can patients with axial spondyloarthritis commence biologics?

A

After failing 12 weeks of NSAID and exercise therapy with on-going active disease

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17
Q

Name 6 biologics that can be used in the treatment of axial spondyloarthritis.

A

Adalimumab

Certolizuma pegol

Etanercept

Golimumab (funded by PBS)

Infliximab

Secukinumab is effective for AS (IL-17A inhibitor)

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18
Q

What are the PBS funding criteria for golimumab on non-radiographic axial spondyloarthropathy?

A

nr-axSpA as defined by ASAS criteria

Elevated inflammatory markers

Sacroiliitis with inflammation or oedema on non-contrast MRI

Bone marrow oedema on STIR and T1 images (non-contrast)

Failed NSAIDs and exercise for 3 months

BASDAI 4 or more

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19
Q

What are the classification criteria for axial spondyloarthritis?

A

Patients must have at least 3 months of back pain with age of onset < 45

Sacroiliitis on imaging plus at least 1 SpA feature OR HLA-B27 positive plus at least 2 other SpA features:

  • inflammatory back pain
  • arthritis
  • enthesitis
  • uveitis
  • dactylitis
  • psoriasis
  • Crohn’s/colitis
  • good response to NSAIDs
  • family history for SpA
  • HLA-B27
  • elevated CRP
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20
Q

What are the classification criteria for peripheral spondyloarthritis?

A

Arthritis, enthesitis or dactylitis PLUS

1 or more SpA features

  • uveitis
  • psoriasis
  • Crohn’s/colitis
  • preceding infection
  • HLA-B27
  • sacroiliitis on imaging

OR at least 2 other SpA features

  • arthritis
  • enthesitis
  • dactylitis
  • inflammatory back pain ever
  • family history for SpA
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21
Q

What is the definition of a positive MRI sacroiliac joint?

A

Subchondral bone marrow oedema

Acute (bilateral) sacroiliitis

STIR sequencing

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22
Q

How is psoriatic arthritis diagnosed?

A

Presence of established inflammatory articular disease (peripheral arthritis, enthesitis or axial) plus three points from:

  • present skin psoriasis (2 points)
  • past of family Hx of psoriasis (1 point)
  • dactylitis (1 point)
  • nail changes - pitting, onycholysis (1 point)
  • RF negative (1 point)
  • juxta-articular new bone formation on XR (1 point)
23
Q

What correlates better to psoriatic arthritis, skin or nail disease?

A

Nail disease

24
Q

Name 2 co-morbidities associated with psoriatic arthritis.

A

Cardiovascular disease

Metabolic syndrome

Obesity additionally is associated with worse outcomes

25
Q

What treatments can be given for psoriatic arthritis? 6 categories

A

NSAIDs - symptom relief

CsDMARDs - methotrexate, sulfasalazine, leflunomide (but little data for these)

Anti-TNF - adalimumab, certolizumab pegol, etanercept, golimumab, infliximab

Anti-IL17 - secukinumab, ixekizumab

Anti-p40 subunit IL12/23 - ustekinumab

Anti-p19 subunit IL23 - guselkumab

Also apremilast, a phosphodiesterase 4 inhibitor, but this is not PBS listed

26
Q

Name 5 organisms that can cause reactive arthritis.

A

Chlamydia

Shigella

Salmonella

Campylobacter

Yersinia

27
Q

Name 8 extra-articular manifestations of reactive arthritis.

A

Keratoderma blennorrhagica

Circinate balanitis

Mouth ulcers

Conjunctivitis

Uveitis

Aseptic urethritis

Cervicitis

Prostatitis

28
Q

What are the treatment options for reactive arthritis? 5 options.

A

If chlamydia - treat, contact tracing

NSAIDs

Intra-articular glucocorticoids (systemic if unwell)

DMARDs - sulfasalazine for chronic disease, or methotrexate

29
Q

Name 3 features of IBD that might predispose patients towards having spondyloarthritis.

A

Having other extra-intestinal features

Complications of bowel disease

Large bowel involvement (for Crohn’s)

30
Q

What is the most likely cause of death in systemic sclerosis?

A

Pulmonary arterial hypertension

31
Q

What pattern of interstitial lung disease is most likely to be seen in systemic sclerosis?

A

Non-specific interstitial pneumonitis

32
Q

Name 2 high risk phenotypes in systemic sclerosis for the development of ILD.

A

Early dc-SSc and anti-Scl70

Early dc-SSc and elevated CRP

33
Q

How is SSc-ILD graded via HRCT?

A

<20% = limited disease

> 20% = extensive disease

Indeterminate percentage + FVC 70% or above = limited disease

Indeterminate percentage + FVC <70% = extensive disease

34
Q

How often should patients with early diffuse SSc with ILD be monitored?

A

Use spirometry and DLCO

Every 3-4 months for 3-5 years after disease onset, then yearly

No advantage in serial HRCT scans if PFTs are stable

35
Q

What are the options for treatment of SSc-ILD?

A

Mycophenolate 3g/day or if intolerant, mycophenolic acid

Oral or IV cyclophosphamide for 6-12 months followed by MMF or azathioprine for severe or progressive disease unresponsive to MMF

Consider nintedanib

36
Q

Which antibodies would you expect to see in scleroderma renal crisis?

A

Anti-RNA polymerase III

37
Q

Which drug can precipitate scleroderma renal crisis?

A

Cyclosporin A

38
Q

Name 4 classes of drugs that can be used in SSc-PAH for treatment.

A

PDE-5 inhibitors (sildenafil, tadalafil)

Endothelin receptor antagonists (ambrisentan, bosentan, macitentan)

Prostacyclin analogues (iloprost, treprostinil, IV epoprostenol for class III or IV disease)

Riociguat

No role for CCB monotherapy

39
Q

What is the treatment of scleroderma renal crises?

A

Mainstay of treatment is ACEi

Benefit of combining this with bosentan or plasma exchange

If CNS involvement (encephalopathy) - add nitroprusside

40
Q

Name 6 non-pharmacological therapies for treating Raynaud’s phenomenon.

A

Treat underlying cause e.g. cryoglobulinaemia

Keep hands warm/hand warmers

41
Q

Name pharmacological therapies for Raynaud’s phenomenon (3 stages).

A

First line -

42
Q

Which SSc patients benefit from autologous HSCT?

A

Non-smokers non-responsive to standard treatment along with either:

  • dcSSc within the first 4-5 years with mild-to-moderate organ involvement
  • lcSSc with progressive visceral involvement
43
Q

Name 2 complications from SSc affecting the small bowel

A

Pseudo-obstruction

Bacterial overgrowth

44
Q

Name 1 complication from SSc affecting the liver.

A

Primary biliary cirrhosis

45
Q

Name 2 complications from SSc affecting the large bowel.

A

Wide-mouth diverticulae

Faecal incontinence

46
Q

Name 3 diseases that can result following TREX1 gene mutations.

A

SLE

Aicardi-Goutiere’s syndrome - early onset encephalopathy

RVCL - retinal vasculopathy with cerebral leukodystrophy

47
Q

Name 4 complement deficiencies that can predispose patients to SLE.

A

C1q

C2

C4A

C4B

90% risk of SLE in homozygotes for C1q deficiency

48
Q

Name 3 drugs that can cause drug-induced lupus where you are more likely to see anti-histone antibodies.

A

Procainamide

Hydralazine

Isoniazid

49
Q

Name 3 features of Jaccoud’s arthropathy.

A

Ulnar deviation

Swan-neck deformity

Tenosynovitis

50
Q

What is the most common manifestation of cardiac disease in SLE?

A

Pericarditis

51
Q

What are the two most specific auto-antibodies seen in SLE, and what manifestations are they associated with?

A

Anti-Smith - renal and CNS disease

Anti-dsDNA - disease activity, nephritis, TNFi induced disease

52
Q

Name 4 triggers for SLE.

A

UV light/phototherapy

Sulphonamides

Stress

Oestrogen-containing preparations

53
Q

What is belimumab, and what is it used in?

A

Used in SLE

mAb against BAFF/BLyS (B-cell activating factor/B lymphocyte stimulator)

54
Q

Name 7 contra-indications to pregnancy in SLE.

A

Severe restrictive lung disease (FVC > 1L)

Severe pulmonary hypertension (sPAP >50)

Advanced renal insufficiency (>2.8 Cr mg/L)

Advanced heart failure

Previous severe pre-eclampsia or HELLP

Stroke within last 6 months

Severe disease flare within 6 months

GCUH FRACP Writtens 2020 (14 decks)

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