Rheumatology

Question 1

Markers associated with diffuse cutaneous systemic sclerosis

Answer 1

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

Question 2

Markers associated with limited cutaneous systemic sclerosis

Answer 2

Anti-centromere

Question 3

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

Answer 3

~10% in both

Question 4

Antibodies in systemic sclerosis

Answer 4

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)

Question 5

What is the added significance of RNA Polymerase III in dcSSc

Answer 5

Renal, skin involvement

Malignancy within 2-5 years of diagnosis

Question 6

Which autoantibody in SSc is associated with ILD

Answer 6

Scl-70 (topoisomeraise 1)

Question 7

What disease has the highest mortality amongst autoimmune rheumatic diseases?

Answer 7

Systemic sclerosis

Question 8

What is the leading cause of death in SSc

Answer 8

Cardiopulmonary manifestations

prior to ACE-I, renal crisis was the leading

Question 9

What is the pulmonary feature most common in Systemic Sclerosis

Answer 9

Non specific Interstitial Pneumonitis&raquo_space; Usual interstitial pneumonitis

Question 10

Typical findings on HRCT for NSIP

Answer 10

Ground glass

Fine fibrosis

Question 11

Typical findings on HRCT for UIP

Answer 11

Peripheral reticulo-nodular opacities
Honey-combing
Traction bronchiectasis

Question 12

What is the high risk phenotype for SSc-ILD

Answer 12

1. Early dcSSc and anti-Scl 70

2. Early dcSSc and elevated CRP

Question 13

Pulmonary screening for patients with early dSSc with ILD

Answer 13

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

Question 14

First line therapy for SSc ILD

Answer 14

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

Azathioprine if unable to tolerate MMF

Question 15

Mechanism of action of Nintedanib

Answer 15

Tyrosine kinase to:

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

Question 16

What concurrent issue worsens survival in SSc Pulmonary Arterial Hypertension?

Answer 16

Iron deficiency HR 0.34

Question 17

What is scleroderma renal crisis

Answer 17

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

Question 18

Risk factors for scleroderma renal crisis

Answer 18

RNA polymerase III antibody
Tendon friction rubs
Corticosteroids are triggers

Question 19

Describe the pathogenesis of OA

Answer 19

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

Question 20

What genes are implicated in the genetics of OA

Answer 20

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

Question 21

What 2 types of OA have the strongest heritability

Answer 21

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

Question 22

Main joints affected by OA

Answer 22

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

Question 23

X-ray findings in OA

Answer 23

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

Question 24

What are bone marrow lesions?

Answer 24

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

Question 25

Management of OA entails?

Answer 25

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

Question 26

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

Answer 26

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

Question 27

What is the role of Zometa in OA

Answer 27

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

Question 28

Extra-articular features of RA

Answer 28

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

Question 29

Lung manifestations of RA

Answer 29

- Exudate pleural effusions - Lung nodules (a/w subcut nodules) - UIP or NSIP

Question 30

Antibody with the highest specificity for RA

Answer 30

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)

Question 31

Poor prognostic markers in RA

Answer 31

- 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)

Question 32

Natural history of palindromic rheumatism

Answer 32

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

Question 33

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

Answer 33

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

Question 34

Radiographic features of RA

Answer 34

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

Question 35

Predictor of subsequent CV disease related death in RA

Answer 35

CRP level at baseline | Risk reduces with good control of inflammatory arthritis

Question 36

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

Answer 36

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)

Question 37

Biochemical markers of SLE disease activity?

Answer 37

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

Question 38

Disease activity measures for SLE

Answer 38

SLEDAI, BILAG, SLAM, ECLAM