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Specialty Medicine > Pediatric Rheumatic Disease > Flashcards

Pediatric Rheumatic Disease Flashcards

1
Q

Which gender is more affected with JIA?

A

Girls

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2
Q

What is the pathology that occurs with JIA?

A

hyperplasia of the synovial lining, causing edema, and hyperemia

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3
Q

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

A
  • morning stiffness
  • Rheumatoid nodules
  • Limp / refusal to bear weight
  • Deformity
  • joint edema, erythema, and warmth
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4
Q

What are some of the systemic signs of JIA?

A
  • HSM
  • LAD
  • Fever
  • Irritability
  • Rash
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5
Q

What are the two major kinds of JIA

A

Polyarthritis

Oligoarthritis

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6
Q

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

A

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

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7
Q

What is the oligoarthritis form of JIA?

A

1-4 joints affected

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8
Q

What percent of systemic JIA will remit within 1 year?

A

50%

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9
Q

What is the systemic form of JIA?

A

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

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10
Q

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

A 
Polyarthritis = 40%
Oligoarthritis = 50%
Systemic = 10%
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11
Q

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

A 
Polyarthritis = 5%
Oligoarthritis = 40%
Systemic = rare
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12
Q

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

A 
Polyarthritis = 10%
Oligoarthritis = rare
Systemic = rare
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13
Q

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

A

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

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14
Q

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

A 
Polyarthritis = all large joints
Oligoarthritis = knees and ankles
Systemic = any joint
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15
Q

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

A 
Polyarthritis = moderate
Oligoarthritis = rare
Systemic = Severe
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16
Q

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

A

Uveitis, and requires an ophthalmology exam

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17
Q

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

A

Damage the uvea, leading to poor eyesight

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18
Q

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

A

16 years old

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19
Q

What is the duration needed to diagnose JIA

A

6+ weeks

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20
Q

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

A
  • Edema
  • RROM
  • TTP
  • Heat
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21
Q

What are the characteristics of the rash seen with JIA?

A

Diffuse macular rashes that is salmon colored

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22
Q

What are the CBC findings in a kid with JIA?

A

Leukocytosis
Anemia
Thrombocytosis

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23
Q

What happens to ESR and CRP levels with JIA?

A

Elevated

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24
Q

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

A

false–not needed

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25
Q

What is the pharmacotherapy for JIA? (4)

A
  • NSAIDs
  • DMARDs
  • Steroids
  • Joint injections
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26
Q

What are the only disease modifying treatment available for JIA?

27
Q

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

A

Infrequent exacerbations, with long periods of remissions

Worse if Rh factor +

If severe, leads to joint destruction

28
Q

What is psoriatic arthritis?

A

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

29
Q

What is the pharmacotherapy for psoriatic arthritis?

30
Q

What is enthesitis?

A

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

31
Q

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

A

Males older than 8

32
Q

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

33
Q

What might enthesitis-related JIA progress to?

A

Spondyloarthropathies

34
Q

What is neonatal lupus?

A

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

35
Q

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

A

Thrombocytopenia

36
Q

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

A

Complete heart block

37
Q

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

A

Hepatomegaly or hepatitis

38
Q

What is the treatment / prognosis for neonatal lupus?

A

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

39
Q

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

A

False–not uncommon

40
Q

what, generally, is Henoch-Schonlein purpura?

A

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

41
Q

What type of purpura are had with HSP?

A

Non-thrombocytopenic

42
Q

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

A

2-10 years

43
Q

What are the areas affected with HSP? (4)

A
  • Skin
  • Joints
  • GI tract
  • Kidneys
44
Q

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

A

Dependent areas (buttock + LE)

symmetric

45
Q

What are the GI symptoms of HSP?

A

Intermittent, colicy abdominal pain

46
Q

What is a severe GI sequelae of HSP?

A

intussusception

47
Q

What is the diagnostic study needed to diagnose intussusception?

A

Ba contrast enema

48
Q

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

A
  • Arthralgias
  • Tenderness with ROM
  • Stiffness
  • Limp

(NOT erythema, edema)

49
Q

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

50
Q

What will and CBC show with HSP?

A

Normal platelets

51
Q

What will a PT/PTT show with HSP?

52
Q

What infectious disease should be r/o with HSP?

53
Q

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

A

BUN/Cr and UA

54
Q

What is the treatment for HSP?

A

Supportive

Steroids for abdominal pain

55
Q

What is the usual course for HSP?

A

Self limiting, but may have recurrences

56
Q

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

57
Q

What is the classic triad with HUS?

A
  • Microangiopathic hemolytic anemia
  • Thrombocytopenia
  • Renal insufficiency
58
Q

What is the infectious agent that causes HUS?

A

Shiga toxin producing strains of E.coli O157:H7

59
Q

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

A

HUS has thrombocytopenia, whilst HSP does not

60
Q

What is the usual presentation of HUS?

A

Abdominal pain + Bloody diarrhea, followed by:

  • Petechiae
  • oliguria
  • pallor (2/2 anemia)
  • Edema/HTN
  • Sz / coma
61
Q

What is the role of antimotility agents in kids?

62
Q

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

A

Not used-may precipitate HUS

63
Q

What type of transfusions are used in HUS?

A

pRBCs, but avoid platelet transfusions

64
Q

How do you prevent e.coli diarrhea?

A

Cook food well

Specialty Medicine (36 decks)

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