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Flashcards in Pediatric Rheumatic Disease Deck (64):
1

Which gender is more affected with JIA?

Girls

2

What is the pathology that occurs with JIA?

hyperplasia of the synovial lining, causing edema, and hyperemia

3

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

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

4

What are some of the systemic signs of JIA?

-HSM
-LAD
-Fever
-Irritability
-Rash

5

What are the two major kinds of JIA

Polyarthritis
Oligoarthritis

6

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

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

7

What is the oligoarthritis form of JIA?

1-4 joints affected

8

What percent of systemic JIA will remit within 1 year?

50%

9

What is the systemic form of JIA?

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

10

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

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

11

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

Polyarthritis = 5%
Oligoarthritis = 40%
Systemic = rare

12

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

Polyarthritis = 10%
Oligoarthritis = rare
Systemic = rare

13

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

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

14

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

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

15

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

Polyarthritis = moderate
Oligoarthritis = rare
Systemic = Severe

16

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

Uveitis, and requires an ophthalmology exam

17

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

Damage the uvea, leading to poor eyesight

18

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

16 years old

19

What is the duration needed to diagnose JIA

6+ weeks

20

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

-Edema
-RROM
-TTP
-Heat

21

What are the characteristics of the rash seen with JIA?

Diffuse macular rashes that is salmon colored

22

What are the CBC findings in a kid with JIA?

Leukocytosis
Anemia
Thrombocytosis

23

What happens to ESR and CRP levels with JIA?

Elevated

24

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

false--not needed

25

What is the pharmacotherapy for JIA? (4)

-NSAIDs
-DMARDs
-Steroids
-Joint injections

26

What are the only disease modifying treatment available for JIA?

DMARDs

27

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

Infrequent exacerbations, with long periods of remissions

Worse if Rh factor +

If severe, leads to joint destruction

28

What is psoriatic arthritis?

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

29

What is the pharmacotherapy for psoriatic arthritis?

DMARDs

30

What is enthesitis?

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

31

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

Males older than 8

32

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

HLA-B27

33

What might enthesitis-related JIA progress to?

Spondyloarthropathies

34

What is neonatal lupus?

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

35

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

Thrombocytopenia

36

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

Complete heart block

37

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

Hepatomegaly or hepatitis

38

What is the treatment / prognosis for neonatal lupus?

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

39

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

False--not uncommon

40

what, generally, is Henoch-Schonlein purpura?

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

41

What type of purpura are had with HSP?

Non-thrombocytopenic

42

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

2-10 years

43

What are the areas affected with HSP? (4)

-Skin
-Joints
-GI tract
-Kidneys

44

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

Dependent areas (buttock + LE)

symmetric

45

What are the GI symptoms of HSP?

Intermittent, colicy abdominal pain

46

What is a severe GI sequelae of HSP?

intussusception

47

What is the diagnostic study needed to diagnose intussusception?

Ba contrast enema

48

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

-Arthralgias
-Tenderness with ROM
-Stiffness
-Limp

(NOT erythema, edema)

49

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

Nephritis

50

What will and CBC show with HSP?

Normal platelets

51

What will a PT/PTT show with HSP?

Normal

52

What infectious disease should be r/o with HSP?

Strep

53

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

BUN/Cr and UA

54

What is the treatment for HSP?

Supportive
Steroids for abdominal pain

55

What is the usual course for HSP?

Self limiting, but may have recurrences

56

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

Steroids

57

What is the classic triad with HUS?

-Microangiopathic hemolytic anemia
-Thrombocytopenia
-Renal insufficiency

58

What is the infectious agent that causes HUS?

Shiga toxin producing strains of E.coli O157:H7

59

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

HUS has thrombocytopenia, whilst HSP does not

60

What is the usual presentation of HUS?

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

61

What is the role of antimotility agents in kids?

Not used

62

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

Not used-may precipitate HUS

63

What type of transfusions are used in HUS?

pRBCs, but avoid platelet transfusions

64

How do you prevent e.coli diarrhea?

Cook food well