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BPT > Rheumatology > Flashcards

Rheumatology Flashcards

Revise rheum

1
Q

Markers associated with diffuse cutaneous systemic sclerosis

A

Scl-70 (anti-topoisomerase) - ILD
Anti-RNA polymerase I, III - renal crises
ANA with nucleolar pattern

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2
Q

Markers associated with limited cutaneous systemic sclerosis

A

Anti-centromere

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3
Q

What is the prevalence of pulmonary arterial hypertension in diffuse and limited cutaneous systemic sclerosis

A

~10% in both

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4
Q

Antibodies in systemic sclerosis

A

Scl-70 - speckled (dcSSc)
RNA polymerase III -fine speckled nucleolar (dcSSc)
Anti-centromere (lcSSc)
U1-RNP (both)
Th/T0 (both, rare) - a/w PAH and worse prognosis
PM-Scl (both)

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5
Q

What is the added significance of RNA Polymerase III in dcSSc

A

Renal, skin involvement

Malignancy within 2-5 years of diagnosis

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6
Q

Which autoantibody in SSc is associated with ILD

A

Scl-70 (topoisomeraise 1)

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7
Q

What disease has the highest mortality amongst autoimmune rheumatic diseases?

A

Systemic sclerosis

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8
Q

What is the leading cause of death in SSc

A

Cardiopulmonary manifestations

prior to ACE-I, renal crisis was the leading

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9
Q

What is the pulmonary feature most common in Systemic Sclerosis

A

Non specific Interstitial Pneumonitis&raquo_space; Usual interstitial pneumonitis

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10
Q

Typical findings on HRCT for NSIP

A

Ground glass

Fine fibrosis

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11
Q

Typical findings on HRCT for UIP

A

Peripheral reticulo-nodular opacities
Honey-combing
Traction bronchiectasis

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12
Q

What is the high risk phenotype for SSc-ILD

A
  1. Early dcSSc and anti-Scl 70

2. Early dcSSc and elevated CRP

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13
Q

Pulmonary screening for patients with early dSSc with ILD

A

Spirometry and DLCO every 3-4 months for 3-5 years after disease onset, then yearly

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14
Q

First line therapy for SSc ILD

A

1st line: MMF or PO/IV cyclophos followed by MMF

Azathioprine if unable to tolerate MMF

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15
Q

Mechanism of action of Nintedanib

A

Tyrosine kinase to:

  • Fibroblast growth factor (FGF)
  • Platelet derived growth factor (PDGF)
  • Vascular endothelial growth factor (VEGF)
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16
Q

What concurrent issue worsens survival in SSc Pulmonary Arterial Hypertension?

A

Iron deficiency HR 0.34

17
Q

What is scleroderma renal crisis

A

Life-threatening hyper-reninaemic, rapidly progressive renal impairment, usually occuring within 5 years of disease onset. A/w abrupt onset moderate to severe HTN, normal urine sediment or mild proteinuria, progressive renal failure.
Occurs in 10-20% of pts with dSSc and less in limited

18
Q

Risk factors for scleroderma renal crisis

A

RNA polymerase III antibody
Tendon friction rubs
Corticosteroids are triggers

19
Q

Describe the pathogenesis of OA

A

Primary articular cartilage disease, but role of whole joint - subchondral bone, synoviium, neuromuscular feedback

20
Q

What genes are implicated in the genetics of OA

A

GDF5, FRZB, DIO2 – affect joint formation, BMD and bone composition

21
Q

What 2 types of OA have the strongest heritability

A

C-spine and L-spine (both 70%)
Hand 65%
Hip 60%
Knee 40%

22
Q

Main joints affected by OA

A

Knee (medial), hips, DIP/PIP, 1st, CMC, C-spine, L-spine, 1st MTP

23
Q

X-ray findings in OA

A 
Joint space narrowing
Osteophytes
Subchondral bone sclerosis
Subchondral cysts
Bone attrition (altered bone contour)
Soft tissue swelling

L: loss of joint space
O: osteophytes
S: subchondral cysts
S: subchondral sclerosis

24
Q

What are bone marrow lesions?

A

Sclerotic but poorly mineralised bone with granulation, oedema, fibrosis and bone necrosis. They predict pain, cartilage damage and loss, and need for TKR.

25
Q

Management of OA entails?

A
  1. Weight management
  2. Allied health/exercise
  3. Simple analgesia - regular paracetamol and/or short term topical NSAIDs/capsaicin.
  4. Trial short term oral NSAID but monitor BP and renal function
26
Q

What factors are taken into account when considering surgery for OA?

A
  • Not only x-ray findings
  • clinical symptoms are primary
  • MAPT score - Multi-Attribute Prioritisation Tool
27
Q

What is the role of Zometa in OA

A

Single infusion of Zometa reduces knee pain and BML size at 6 months, compared to placebo

28
Q

Extra-articular features of RA

A

Eyes: sicca 30%, scleritis/episcleritis 5%

Skin: Rh nodules 25% - only in RhF+

Vasculitis: palpable purpura, ischaemic ulcers, digital gangrene, mononeuritis multiplex (vaso nevorum)

Neurologic: compressive neuropathy

Lymphoma B-cell: 3 x higher risk, a/w long term disease activity

Lung manifestations

Felty’s Syndrome

Large granular lymphocyte syndrome

29
Q

Lung manifestations of RA

A
  • Exudate pleural effusions
  • Lung nodules (a/w subcut nodules)
  • UIP or NSIP
30
Q

Antibody with the highest specificity for RA

A

Anti-CCP (90% spec)
detectable prior to onset of clinical disease and 50% of Anti-CCP+ patients will develop inflammatory arthritis over 5 years, especially if smokers (shared epitope)

31
Q

Poor prognostic markers in RA

A
  • Sustained high levels of synovial and systemic inflammation
  • RhF +/- anti-CCP
  • early erosions on imaging
  • extra-articular features - ILD, nodules
  • smoking
  • poor education and socioeconomic status
  • HLA-DRB1*04 homozygosity (shared epitope)
32
Q

Natural history of palindromic rheumatism

A

-67% develop RA
-15% continue to have PR
-15% remission
Tx: HCQ - may reduce risk of progression to RA

33
Q

Features of remitting, seronegative, symmetric synovitis with pitting oedema (RS3PE)

A
  • Boxing glove like oedema of dorsum of hand
  • Tenosynovitis
  • RhF/Anti-CCP negative
  • No erosions
  • Responds well to steroids
  • Can be paraneoplastic phenomenon
34
Q

Radiographic features of RA

A
  • Periarticular soft tissue swelling - oedema, effusion, tenosynovitis
  • Juxta-articular osteoporosis
  • Marginal erosions
  • Joint space narrowing
  • Symmetric involvement
  • Deformities in advanced disease
35
Q

Predictor of subsequent CV disease related death in RA

A

CRP level at baseline

Risk reduces with good control of inflammatory arthritis

36
Q

ENA associations in SLE:

1) Anti-Ro
2) Anti-La
3) Anti-Sm
4) Anti-U1RNP/RNP
5) Anti-ribosomal p
6) Anti-neuronal
7) Anti-dsDNA

A

1) Neonatal lupus, lymphopenia, photosensitivity, C2 def, subacute cutaneous lupus, Sjogren’s
2) Neonatal lupus, Sjogren’s
3) Renal and CNS disease (highly specific)
4) Myositis, Raynaud’s
5) Psychosis, depression
6) Organic brain syndromes
7) Disease activity, lupus nephritis, TNFi-induced lupus
(highly specific)

37
Q

Biochemical markers of SLE disease activity?

A

ESR > CRP
Increased Anti-dsDNA Ab
Low C3/C4

38
Q

Disease activity measures for SLE

A

SLEDAI, BILAG, SLAM, ECLAM

BPT (6 decks)

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