Paed Rheumatology

Question 1

What is the commonest chronic rheumatological disease in children?

Answer 1

JIA

Question 2

What pro inflammatory markers are present in JIA?

Answer 2

TNF

Inerleukin 1 and 2

Question 3

Criteria for JIA

Answer 3

At least 6 weeks 
morning stiffness or swelling 
irritability or refusal to walk in toddlers
school absence or limited ability to participate activity 
Rash / fever 
fatigue
Poor appetite / weight loss
Delayed puberty

Question 4

Differential diagnosis of JIA

Answer 4

Septic arthritis 
Osteomyelitis 
Transient synovitis 
Malignancies e.g. lymphoma, neuroblastoma, bone tumours
Recurrent haemarthrosis 
Vascular abnormalities 
Trauma
Others

Question 5

Signs of JIA

Answer 5

Swelling : periarticular soft tissue oedema / intraarticular effusion / hypertrophy of synovial membrane
Tenosynovitis (swollen tendons)
Pain
Joint held in position of maximum comfort
Range of motion limited at extremes

Question 6

Presentation of systemic JIA

Answer 6

Unwell 
Arthritis 
Intermittent fever > 2 weeks
Salmon pink erythematous rash 
Generalised lymphadenopathy 
serositis
hepatomegaly/splenomegaly 
High inflammatory markers

Question 7

Investigations for JIA

Answer 7

Labs
Plain x ray
USS
MRI with contrast

Question 8

Treatment of JIA

Answer 8

NSAIDs
DMARDs
Biologics 
Intra-articular / oral steroids
Psychological counselling
School life / physical adjustments 
Nutrition particularly to address anaemia or generalised osteoporosis 
Physio 
OT

Question 9

Remission when using intra-articular steroids (IAS)

Answer 9

Remission > 6 months 84%

Question 10

What type of JIA does IAS have must success in?

Answer 10

Oligoarticular

Question 11

What is the DMARD of choice in JIA?

Answer 11

Methotrexate

Question 12

How is methotrexate mostly given in JIA?

Answer 12

Injectable (subcutaneous) form

Question 13

What needs to be done when on methotrexate?

Answer 13

Blood monitoring

Question 14

When are biologics used in JIA?

Answer 14

Failure to respond to DMARD

Question 15

What biological agents are commonly used?

Answer 15

Anti TNF

Question 16

What is uveitis associated with?

Answer 16

JIA

Question 17

What can uveitis progress to if untreated?

Answer 17

Chronic uveitis

Question 18

All children with JIA undergo screening for what?

Answer 18

Uveitits

Question 19

What kind of JIA is uveitis more common in?

Answer 19

ANA positive oligo JIA

Question 20

Presentation of uveitis

Answer 20

Red eyes
headache
reduced vision

Question 21

Complications of uveitis

Answer 21

Cataracts
Glaucoma
Blindness

Question 22

investigations of uveitis of JIA

Answer 22

Slit lamp examination

Question 23

Treatment of uveitis

Answer 23

Initially topical steroids to reduce inflammation
More severe need systemic steroids
Poor response to steroids DMARD and biologics

Question 24

Complications of JIA

Answer 24

Poor growth 
Localised growth disturbances
Micrognathia
contractures
ocular complications

Question 25

Who may be hypotonic on examination?

Answer 25

An acutely ill child

Question 26

What is hypotonia associated with?

Answer 26

Encephalopathy in the newborn period most commonly caused by HIE

Question 27

Central causes of hypotonia

Answer 27

Downs syndrome Prader willi syndrome Hypothyroidism CP (may precede the development of spasticity)

Question 28

Neurological and muscular causes of hypotonia

Answer 28

``` Spinal muscular atrophy Spina bifida Gullian barre syndrome Myasthenia gravis Muscular dystrophy Myotonic dystrophy ```