Pediatric Rheumatology

Question 1

Question 2

signs and symptoms of arthritis

Answer 2

swelling, warmth and redness, pain, decresed joint space, decreased range of motion, decreased function, poor development of joint

• overall impact on physical, emotional and social growth and maturity

Question 3

ddx of juvenile inflammatory arthritis

Answer 3

1. septic arthtiris
2. osteomyelitis
3. chronic non-infectious osteomyelitis
4. post-infectious arthritis (◦ Rheumatic fever
   ◦ Post-streptococcal arthritis
   ◦ Reactive arthritis
   ◦ TB associated arthritis
   ◦ Lyme disease)
5. arthritis associated with other conditions (malignancy, hemophilia, IBD)
6. mechanical/orthopedic conditions
7. metabolic/genetic disorders

Question 4

juvenile arthritis is not a single disease. what are the subtypes of juvenile idiopathic arthritis?

Answer 4

Question 5

describe the 7 subtypes of juvenile idiopathic arthritis in terms of frequency, age of onset and sex.

(systemic, oligoarticular, rf +, rf-, enthesitis related, psoriatic, undifferentiated)

Answer 5

Question 6

common presentation of oligoarticular arthritis

Answer 6

• female
• elbow, knee, wrist, ankle involvement

 Arthritis in 1-4 Joints in first 6months
 After 6 months of symptoms
 Persistent – 4 or fewer during disease course
 Extended -More than 4 joints cumulatively after 6m

• uveitis/eyeproblems (usually asymptomatic)

Question 7

which gender does oligoarticular arthritis affect more?

Answer 7

3:1 female to male.

Question 8

Answer 8

uveitis/iritis seen in oligoarthritis JIA

 Asymptomatic  Pupillary asymmetry  Cataracts  Band keratopathy  Blindness  Requires routine
screening, frequency
determined by ANA

Question 9

common presentation off rF NEGATIVE JIA

Answer 9

• jaw, bilateral knees, elbows, ankle (more symmetrical than oligoarthritis). arthritis 5> joints within first 6 months.
• has a bimodal peak: 1-3 years and then early adolescnece.
• 3:1 female: male
• affects large and small joints
• if they are ANA positive, there is an increased risk for uveitis

Question 10

what increases the likelihood of someone with JIA having uveitis?

Answer 10

Ana postiive

Question 11

children with poly-JIA RF negative often have painless ___ in whcih the fingers cant lay flat on the bed, some fingers with a 90 degree angle. sometimes they may need surgery or splinting.

Answer 11

contractures.

Question 12

common presentation of rF positive polyarticular JIA

Answer 12

patients are usually a bit older/adolscent

Question 13

common presentation of systemic JIA

Answer 13

the evanescent erythematous rash is usually salmon colored, macular, urticaria-like.

• it is non scarring and can appear with fever spikes.

other systemic JIA features includes pericardidits, pleural effucions, macrophage activating syndrome.

Question 14

common presentation of psoriatic JIA

Answer 14

arthritis and psoriasis

OR

arthritis, dactylitis, nail pitting and onycholysis and psoriasis of a first degree relative.

• exclusion: artheritis in B27 positive after 6th bday, or presence of systemic JIA.

Question 15

typical psoriatic patient (peds)

Answer 15

2 year old patient with swolled 2nd finger, swollen toes, asymmetric joint paint, and irregular nails. parent has a history of red scaly rash on extensor sufaces.

Question 16

common presentation of enthesitis-related JIA

Answer 16

teenage boy

• affects achilles, knees, and pelvis
• more likely to have uveitis

HLAb27+, inflammatory lumbosacral pain, sacroiliac joint tenderness.

• exclusion if rF+, or psoriasis

Question 17

HLAB27 associated diseases

Answer 17

• ankylosing spondylitis
• reactive arthritis (can turn chronic)
• rheumatoid arthritis
• enthesitis related arthritis
• sacroilitius with IBD

Question 18

undifferentiated psoriatic JIA

Answer 18

a JIA that does not fulfill criteria of other categories but meets criteria for more than one subtype

ex/ psoriatic arthritis in HLAB27+ boy, when HLAB27 isn’t really associated with PA as much as it AS (which mainly affects boys)

Question 19

common treatments of JIA

Answer 19

Question 20

juvenile dermatomyositis

Answer 20

derma-skin

myositis= inflammation of the muscle

• symmetrical proximal muscle weakness with skin rash

Median age in pediatric population is 7yr

Question 21

diagnosing dermato myositis

Answer 21

• rash
• systemic proximal muscle weakness
• lab: muscle breakdown products like AST, ALT, CPK/CK, LDH
• muscle biopsy and look for muscle edema
• EMG

Question 22

Answer 22

heliotrope; erythema of the eyelids associated with JDM

Question 23

which joints are most affected by gottrons papules

Answer 23

gottrons papules are papulosquamous-erythema over joints. looks like eczema.

DIP and MCP and extensor surfaces affected

Question 24

Answer 24

nail fold capillary manifestations of JDM

• dilated, tortuous drop-out and hemorrhagic cuticles.

Question 25

goals of treatment for JDM

Answer 25

reduce muscle/skin inflammation

maintain muscle and joint function

prevent long term sequelae

prevent end organ damage

reduce side effects of medication

allow for normal emotional/physical development

Question 26

Juvenile systemic lupus:

15-20% of all lupus cases are diagnosed before 18.

4:1 female: male in 1st decade and 9:1 female:male in third decade.

what are the challenges of making the diagnosis of SLE in children?

Answer 26

many present with initially only the constitutional symptoms

• fever
• fatigue/weakness
• myalgia
• weightloss
• headache
• mood changes/poor school performance.

Question 27

SLE clinical features (remember the acronym SOAP-BRAIN MD)

Answer 27

Question 28

Answer 28

oral ulcers of SLE

Question 29

common treatments in SLE

Answer 29

Question 30

notes: in addition to JIA (7 subtypes) , “common” MSK-related peds issues is JDM and SLE

Answer 30

7 subtypes include

• systemic
• rf +
• rf-
• undifferentiated
• oligoarticular
• enthesitis
• psoriatic