Ch 3 - Rheumatology: Juvenile RA

Question 1

What is the MC form of childhood arthritis?

Answer 1

Juvenile RA

Question 2

What are the 3 clinical subtypes of Juvenile RA?

Answer 2

Systemic
Polyarticular
Pauciarticular

Question 3

What is the ACR diagnostic criteria for JRA?

Answer 3

• Onset < 16 yo
• Persistent arthritis in 1 or > joints at least 6 wks
• Exclusion of other types of childhood arthritis
• Type of onset of disease during the 1st 6 mo classified as polyarthritis, oligoarthritis, or systemic arthritis with intermittent fever

Question 4

What are causes of childhood arthritis besides JRA?

Answer 4

Rheumatic fever
Infection
SLE
Vasculitis

Question 5

What is Still’s disease?

Answer 5

Systemic JRA

Question 6

What is the onset of Still’s disease?

Answer 6

1 to 6 years old

Male = female

Question 7

Describe fever in Still’s disease.

Answer 7

Persistent intermittent fever daily > 101°F spikes daily or twice daily

Question 8

Describe the rash seen in Still’s disease.

Answer 8

Transient, nonpruritic seen on the trunk

Question 9

Describe multisystemic involvement in Still’s disease.

Answer 9

– Growth delay 
– Osteoporosis, osteopenia 
– Diffuse lymphadenopathy 
– Hepatosplenomegaly 
– Pericarditis 
– Pleuritis 
– Anemia 
– Leukocytosis 
– Acute phase reactants

Question 10

What is Polyarticular JRA?

Answer 10

5 or more joints involved during the first 6 mo

Question 11

What is the onset of Polyarticular JRA?

Answer 11

Females&raquo_space; males

> 8 years old

Question 12

Describe clinical features of Polyarticular JRA.

Answer 12

• Gradual onset of swelling, stiffness involving the cervical spine and hips
• Growth retardation—early closure of the epiphyseal plates
• No extra-articular manifestations of systemic onset disease

Question 13

Describe clinical features of Polyarticular JRA patients with (+) RF.

Answer 13

• 5-10%
• Females > 10 years old
• Erosive and chronic
• Unremitting disease (worst prognosis)
• No Uveitis
• Subcutaneous nodules

Question 14

Describe clinical features of Polyarticular JRA patients with (-) RF.

Answer 14

90-95% of Polyarticular JRA

25% males < 6 years old

Question 15

What is Pauciarticular JRA?

Answer 15

Oligoarticular JRA: 4 or fewer joints affected during the 1st 6 mo

Question 16

What are the types of Pauciarticular JRA?

Answer 16

– Early onset: females,&raquo_space; 5 years old

– Late onset: males

Question 17

What labs are positive in Pauciarticular JRA?

Answer 17

• (+) HLA-B27
• (+) ANA
• RF (–) (Only + in < 2%)

Question 18

What are Pauciarticular JRA patients with + ANA at risk for?

Answer 18

Greater risk of eye involvement

Question 19

What does chronic iridocyclitis in Pauciarticular JRA lead to?

Answer 19

Cataracts, glaucoma, or blindness (often asymptomatic)

Question 20

What is mandatory in Pauciarticular JRA?

Answer 20

• Ophthalmology referral

* Slit lamp exam is required 4x/ year, for 4 to 5 years

Question 21

What are poor prognostic indicators of JRA?

Answer 21

– RF (+)
– Nodules
– Bony erosions on x-ray
– Polyarticular group with unremitting disease

Question 22

What medication should be avoided in treatment of JRA?

Answer 22

Salicylates should be avoided because of risk of Reyes’ syndrome

Question 23

Which JRA types is methotrexate MC used?

Answer 23

Systemic and polyarticular types

Question 24

Which JRA patients are most likely to get chronic iridocyclitis?

Answer 24

< 6 yo in 20-40%

MC in females with ANA (+)