Paediatric Rheumatology

Question 1

Which antibodies infer poorer prognosis in JDM?

Answer 1

• Anti-Jo-1 and anti-Ro antibodies

- long-term prognosis is poor with high risk of early death

Question 2

What are the usual lung sequelae in JDM?

Answer 2

• rare to get lung involvement overall (~15%)
• interstitial lung disease, restrictive lung disease which often is not visible on CXR
• aggressive immunosuppression may help prevent this
• Disease-mediated pleuritis is very rare in JDM

Question 3

What antibodies are associated with neonatal lupus?

Answer 3

Anti Ro/SS-A and Anti-La/SS-B - maternal lupus antibodies cross the placenta

Question 4

What autoimmune conditions are associated with erythema nodosum?

Answer 4

• Behcet’s
• Sarcoidosis
• IBD

Question 5

Heel spurs are associated with which rheumatological condition?

Answer 5

• Ankylosing spondylitis

- often HLAB27+

Question 6

Non-response or poor response to codeine is likely due to…

Answer 6

• reduced activity of cytochrome P450 2D6

Question 7

ANA is associated with…

Answer 7

• JIA

- SLE

Question 8

Which autoimmune conditions are associated with Reynaud?

Answer 8

• SLE

- Scleroderma

Question 9

How does ankylosing spondylitis usually present?

Answer 9

Seronegative for rheumatoid factor
Enthesitis- around foot and knee
Arthritis - often oligoarthritis- Legs> arms, esp hips

• SI / axial skeleton affected later in the course of the disease.

Question 10

What factor attracts polymorphs into the synovial fluid?

Answer 10

Complement C5a.

Question 11

What is the most SENSITIVE and SPECIFIC antibody for SLE?

Answer 11

ANA sensitive

anti-Sm (subtype of ANA) and anti-dsDNA specific

Question 12

Rituximab is a monoclonal antibody to…

Answer 12

• CD20

- Depletes B cells

Question 13

What investigations do you do for sarcoidosis?

Answer 13

• Blood markers: amyloid A, serum ACE, soluble
  interleukin 2 receptor
• CT may demonstrate hilar adenopathy, interstitial
  changes
• USS liver- granulomatous changes
• Eye exam – uveitis
• Muscle biopsy – non-caseating epithelioid granuloma which is highly sensitive/specific for diagnosis!!
• Node biopsy – to exclude TB and haematologic
  malignancy (however can do IGRA and Mantoux

Question 14

Where is the rash in JDM?

Answer 14

• Photosensitive red-purple rash
• Heliotropic eyelid rash
• Gottron papules on hands/fingers PIPJ and DIPJ
• Extensors

Question 15

What is the typical systemic JIA rash?

Answer 15

A spotty pale red or pinkish salmon-colored rash. Rarely occurs on the face; typically appears on chest, upper arms and upper thighs, although can be found on other parts of the body. Usually doesn’t recur in the same location. Generally flat, but can emerge in raised, small patches. Rarely itches.

Question 16

What is the typical lupus rash?

Answer 16

Butterfly-shaped rash that spans the cheeks and bridge of the nose; resembles a sunburn. May also cause scaly, disk-shaped rash on the face, neck, ears, scalp and/or chest.