Rheumatology

Question 1

What effect does allopurinol have on azathioprine?

Answer 1

Enhances the effects of azathioprine and hence the dose of azathioprine should be reduced by 75%

Question 2

In a pt with SLE who is ANA negative, what antibody are the usually + for?

Answer 2

Anti Ro

Question 3

What is the first treatment for oligoarticular JIA

Answer 3

1st NSAIDs + intra articular steroids

Question 4

In osteogenesis imperfecta alk phos levels are usually elevated T/F

Answer 4

F - usually blood tests in OI show no particular abnormality

Question 5

Osteogenesis imperfecta can be associated with hearing impairment T/F

Answer 5

T
Other extra skeletal features: blue/grey sclera, discoloured teeth that break easily (dentinogenesis imprefecta), ligamental laxity, increased skin laxity

Question 6

Bisphosphonate infusions are used in the treatment of osteogenesis imperfecta T/F

Answer 6

T

Question 7

Myasthenia gravis is associated with what antibodies?

Answer 7

Antibodies against acetylcholine receptors

Question 8

Criteria of systemic onset juvenile idiopathic arthritis excluding the characteristic rash (SOJIA)

Answer 8

At least 2 weeks of fever, followed by arthritis in one or more joints PLUS generalised lymphadenopathy or hepato or splenomegaly or serositis

Question 9

5 features of Kawasaki disease in addition to fever

Answer 9

1. Lymphadenopathy in neck at least 15mm
2. Lip or oral cavity erythema or cracked lips
3. Conjunctival injection
4. Rash on trunk
5. Swelling/erythema of hands or feet

Need 4 of these + 5 days of fever for kawasaki diagnosis

Question 10

Criteria for the diagnosis of juvenile dermatomyositis?

Answer 10

Pathognomonic rash + 3 of

1. Proximal muscle weakness
2. Elevated serum levels of muscle enzymes
3. Typical electromyographic changes
4. Histopath changes on muscle biopsy

Note: if option between biopsy and electromyography do electromyography first as it is less invasive

Question 11

In juvenile dermatomyositis calcinosis occurs in the majority of children T/F

Answer 11

F - in 40%

Note: JDM is not associated with malignancy

Question 12

How many joints need to be involved to diagnosis polyarticular JIA?

Answer 12

5 or more joints involved in the first 6 months.

Question 13

JIA is assoc with what eye pathology?

Answer 13

Chronic anterior uveitis

Note: often clinically silent

Question 14

Behcet’s disease is assoc with what eye pathology?

Answer 14

Anterior, posterior or pan uveitis

Note: often severe recurrent attacks, usually presents about 3-4 yrs after oral and genital ulcers

Question 15

What is Still’s disease AKA?

Answer 15

Systemic onset juvenile idiopathic arthritis

Question 16

What would be expected for coags and plt count in HSP?

Answer 16

Normal

Question 17

In a pt with active SLE serum complement levels are invariably reduced T/F

Answer 17

T - this is due to the formation of immune complexes

Question 18

What is the most common sub type of JIA?

Answer 18

Oligoarticular

Note: 50-60% of ALL cases of chronic arthritis of childhood

Question 19

Polyarteritis nodosa is assoc with what antibody and other disease?

Answer 19

ANCA (positive in 50% of cases)

Hepatitis B

Note: medium vessel vasculitis

Question 20

Which ANCA is positive in granulomatosis with polyangitis and microscopic polyangitis?

Answer 20

Granulomatosis = cANCA

Micro polyangitis = pANCA

Note: granulomatosis with polyangitis affects nasal/sinuses but micropolyangitis does not. Both affect lung and kidneys. Micro polyangitis may also have non specific abdominal and joint pain

Question 21

Neonatal lupus may be present at birth but can also develop months later T/F

Answer 21

T

Question 22

Is the facial rash in neonatal lupus a malar distribution?

Answer 22

No, it is not the typical malar distribution that is seen in older children/adults with lupus

Note: rash may be precipitated by phototherapy for hyperbili

Question 23

In a patient with polyarticular JIA what is the next medication after the have failed NSAIDS?

Answer 23

Methotrexate

Question 24

Children with ank spond may experience inflammatory back pain. Common clinical features of this type of pain?

Answer 24

Pain at night with morning stiffness and improvement throughout the day with exercise (No improvement with rest)
Insidious onset
Good response to NSAIDs

Question 25

How does ank spond typically present in children?

Answer 25

Enthesis and peripheral arthritis of lower limbs

Axial disease and inflammatory back pain are less common at disease ONSET than in the adult pop

Question 26

What cardiac abnormality is often present in ank spond?

Answer 26

Aortic regurgitation

Question 27

In patients with SLE who have a non photosensitive rash what antibody is most specific and what antibody tends to be negative

Answer 27

Anti Ro is most specific

ANA tends to be negative

Question 28

What is the difference in eye disease in those with JIA vs enthesitis related arthritis?

Answer 28

JIA - 70% of patients will have bilateral

Enthesitis - more likely unilateral

Question 29

HSP is associated with IgA deposits in the mesangium T/F

Answer 29

T

Note: HSP is a small vessel vasculitis

Question 30

Enteropathic arthritis is associated with positive IgM rheumatoid factor T/F

Answer 30

F - it is a seronegative arthritis

Note: patients with IBD have increased risk of arthritis when disease is active

Question 31

What is the remission rate of polyarticular JIA

Answer 31

About 30%

Question 32

The low dose OCP is CI in those with SLE

Answer 32

False - if they have it if they have low disease activity and a documented negative anti phospholipid ab

Note: avoid OCP with high dose oestrogen

Question 33

In a pt with SLE neutropenia is the most common haem abnormality T/F

Answer 33

F- a mild leukopenia is the most common abnormality usually caused by lymphopenia, neutropenia is rare

Question 34

In a pt with SLE lymphopenia is the most common haem abnormality T/F

Answer 34

T

Question 35

What is the most common form of JIA?

Answer 35

Persistent oligoarthritis

Usually girls, around 3 yrs old