Pediatric Rheumatic Disease

Question 1

Which gender is more affected with JIA?

Answer 1

Girls

Question 2

What is the pathology that occurs with JIA?

Answer 2

hyperplasia of the synovial lining, causing edema, and hyperemia

Question 3

What are the s/sx that are specific to JIA? (5)

Answer 3

• morning stiffness
• Rheumatoid nodules
• Limp / refusal to bear weight
• Deformity
• joint edema, erythema, and warmth

Question 4

What are some of the systemic signs of JIA?

Answer 4

• HSM
• LAD
• Fever
• Irritability
• Rash

Question 5

What are the two major kinds of JIA

Answer 5

Polyarthritis

Oligoarthritis

Question 6

What is the polyarthritis form of JIA? How does RF positivity / negativity correlate with symptoms?

Answer 6

-5+ joints
RF + = like adult RA
RF - = 10% have destructive joint dz

Question 7

What is the oligoarthritis form of JIA?

Answer 7

1-4 joints affected

Question 8

What percent of systemic JIA will remit within 1 year?

Answer 8

50%

Question 9

What is the systemic form of JIA?

Answer 9

characterized by arthritis, fever, which typically is higher than the low-grade fever associated with polyarticular and a salmon pink rash.

Question 10

What percent of JIA cases are polyarthritis? Oligoarthritis? Systemic?

Answer 10

Polyarthritis = 40%
Oligoarthritis = 50%
Systemic = 10%

Question 11

What percent of cases of polyarthritis, oligoarthritis, and systemic arthritis have uveitis?

Answer 11

Polyarthritis = 5%
Oligoarthritis = 40%
Systemic = rare

Question 12

What percent of cases of polyarthritis, oligoarthritis, and systemic arthritis are Rh factor +?

Answer 12

Polyarthritis = 10%
Oligoarthritis = rare
Systemic = rare

Question 13

What percent of cases of polyarthritis, oligoarthritis, and systemic arthritis are ANA +?

Answer 13

Polyarthritis =50%
Oligoarthritis = 80%
Systemic = 10%

Question 14

What are the classic joints involved in polyarthritis, oligoarthritis, and systemic arthritis respectively?

Answer 14

Polyarthritis = all large joints
Oligoarthritis = knees and ankles
Systemic = any joint

Question 15

What is the level of systemic involvement in polyarthritis, oligoarthritis, and systemic arthritis respectively?

Answer 15

Polyarthritis = moderate
Oligoarthritis = rare
Systemic = Severe

Question 16

If a JIA patient has a positive ANA, what must they be screened for, and by whom?

Answer 16

Uveitis, and requires an ophthalmology exam

Question 17

What are the long term effects of uveitis 2/2 untreated JIA?

Answer 17

Damage the uvea, leading to poor eyesight

Question 18

JIA can only be diagnosed in kids less than what age?

Answer 18

16 years old

Question 19

What is the duration needed to diagnose JIA

Answer 19

6+ weeks

Question 20

What are the major s/sx of arthritis in JIA pts?

Answer 20

• Edema
• RROM
• TTP
• Heat

Question 21

What are the characteristics of the rash seen with JIA?

Answer 21

Diffuse macular rashes that is salmon colored

Question 22

What are the CBC findings in a kid with JIA?

Answer 22

Leukocytosis
Anemia
Thrombocytosis

Question 23

What happens to ESR and CRP levels with JIA?

Answer 23

Elevated

Question 24

True or false: an ANA is needed to diagnose JIA in most cases

Answer 24

false–not needed

Question 25

What is the pharmacotherapy for JIA? (4)

Answer 25

- NSAIDs - DMARDs - Steroids - Joint injections

Question 26

What are the only disease modifying treatment available for JIA?

Answer 26

DMARDs

Question 27

What is the prognosis for JIA? What if it's Rh factor +?

Answer 27

Infrequent exacerbations, with long periods of remissions Worse if Rh factor + If severe, leads to joint destruction

Question 28

What is psoriatic arthritis?

Answer 28

Form of JIA, that consists of chronic arthritis and psoriasis OR Arthritis with dactylitis, nail pitting, or onycholysis

Question 29

What is the pharmacotherapy for psoriatic arthritis?

Answer 29

DMARDs

Question 30

What is enthesitis?

Answer 30

Inflammation of the enthesis (site of attachment of muscle or ligament to bone)

Question 31

Which gender is more affected with enthesitis-related JIA? What is the age affected?

Answer 31

Males older than 8

Question 32

What is the HLA haplotype that is associated with enthesitis-related JIA??

Answer 32

HLA-B27

Question 33

What might enthesitis-related JIA progress to?

Answer 33

Spondyloarthropathies

Question 34

What is neonatal lupus?

Answer 34

When the mother transfers antibodies to the fetus, causing thrombocytopenia, cutaneous rash, and complete heart block

Question 35

What is the hematologic abnormality that can arise from neonatal lupus?

Answer 35

Thrombocytopenia

Question 36

What is the cardiac abnormality that can arise from neonatal lupus?

Answer 36

Complete heart block

Question 37

What is the liver abnormality that can arise from neonatal lupus?

Answer 37

Hepatomegaly or hepatitis

Question 38

What is the treatment / prognosis for neonatal lupus?

Answer 38

Most symptoms will resolve w/in 6 months, but complete heart block will require permanent pacemaker

Question 39

True or false: mothers are rarely asymptomatic with SLE when a child develops neonatal SLE

Answer 39

False--not uncommon

Question 40

what, generally, is Henoch-Schonlein purpura?

Answer 40

Idiopathic IgA mediated vasculitis that causes non-thrombocytopenic purpura and vasculitis

Question 41

What type of purpura are had with HSP?

Answer 41

Non-thrombocytopenic

Question 42

What is the age range that is usually affected with HSP?

Answer 42

2-10 years

Question 43

What are the areas affected with HSP? (4)

Answer 43

- Skin - Joints - GI tract - Kidneys

Question 44

Where on the skin are the purpura found with HSP? Is it usually symmetric or asymmetric?

Answer 44

Dependent areas (buttock + LE) symmetric

Question 45

What are the GI symptoms of HSP?

Answer 45

Intermittent, colicy abdominal pain

Question 46

What is a severe GI sequelae of HSP?

Answer 46

intussusception

Question 47

What is the diagnostic study needed to diagnose intussusception?

Answer 47

Ba contrast enema

Question 48

What are the joint symptoms of HSP? (What are not?)

Answer 48

- Arthralgias - Tenderness with ROM - Stiffness - Limp (NOT erythema, edema)

Question 49

What type of kidney pathology does HSP cause: nephrotic or nephritic syndrome?

Answer 49

Nephritis

Question 50

What will and CBC show with HSP?

Answer 50

Normal platelets

Question 51

What will a PT/PTT show with HSP?

Answer 51

Normal

Question 52

What infectious disease should be r/o with HSP?

Answer 52

Strep

Question 53

What are the kidney labs that should be monitored with HSP?

Answer 53

BUN/Cr and UA

Question 54

What is the treatment for HSP?

Answer 54

Supportive | Steroids for abdominal pain

Question 55

What is the usual course for HSP?

Answer 55

Self limiting, but may have recurrences

Question 56

What is the treatment for severe abdominal pain 2/2 HSP?

Answer 56

Steroids

Question 57

What is the classic triad with HUS?

Answer 57

- Microangiopathic hemolytic anemia - Thrombocytopenia - Renal insufficiency

Question 58

What is the infectious agent that causes HUS?

Answer 58

Shiga toxin producing strains of E.coli O157:H7

Question 59

What is the major difference in the hematologic abnormalities between HSP and HUS?

Answer 59

HUS has thrombocytopenia, whilst HSP does not

Question 60

What is the usual presentation of HUS?

Answer 60

Abdominal pain + Bloody diarrhea, followed by: - Petechiae - oliguria - pallor (2/2 anemia) - Edema/HTN - Sz / coma

Question 61

What is the role of antimotility agents in kids?

Answer 61

Not used

Question 62

What is the role of abx in the treatment of E.coli dysentery?

Answer 62

Not used-may precipitate HUS

Question 63

What type of transfusions are used in HUS?

Answer 63

pRBCs, but avoid platelet transfusions

Question 64

How do you prevent e.coli diarrhea?

Answer 64

Cook food well