Rheumatology Flashcards Preview

Pediatrics (Pre-Test, OnlineMedEd) > Rheumatology > Flashcards

Flashcards in Rheumatology Deck (37):
1

Systemic IgA-mediated vasculitis involving the joints, GI tract, and kidneys

Henoch-Schönlein Purpura

2

Who does HSP typically affect?

 

Children younger than 10 y/o (median age of onset = 5 years)

 

Males more likely affected (M-F ratio = 2:1)

3

What are the clinical features of HSP?

  1. Viral syndrome or URI precedes HSP in most patients (20% of pts have concomitant or prior group A beta-hemolytic streptococcal infection)
  2. Skin, GI, joint, renal manifestations follow prodrome
    1. Skin: 
      1. Urticarial or erythematous maculopapular lesions --> petechiae and palpable purpuric lesions concentrated on butt and lower extremities
      2. Edema of hands, feet, scrotum, scalp
    2. Joint:
      1. 80% of patients: Arthralgia or arthritis (knees and ankles most commonly involved)
    3. GI:
      1. 67% of patients: colicky abdominal pain, GI bleed
      2. Inc. risk of intussusception
    4. Renal:
      1. Wide range of presentation - mild hematuria to gross hematuria, nephrotic syndrome, chronic renal insufficiency, ESRD (1% of cases)
      2. Renal manifestations may not ecome clinically apparent for up to 3 mo after initial presentation in 25% of pts who develop nephritis

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4

How to dx HSP?

  1. Inc. serum IgA levels present in 50% pts
  2. Circulating IgA immune complexes in serum and IgA deposition in skin and glomeruli
  3. Platelet counts are normal despite presence of petechiae and purpura (i.e., the skin rash is a nonthrombocytopenic purpura)

5

Mgmt/prognosis of HSP?

Treatment based on relief of symptoms (pain control/hydration)

  • Steroids may be effective for relief of abdominal pain/arthritis

 

Prognosis:

  1. Most pts recover within 4 weeks
  2. HSP recurs at least once in 50% of pts
  3. Long-term morbidity is dependent on severity of nephritis

6

Acute febrile vasculitis of childhood, of unknown origin involving multiple organ systems, including the heart, skin, mucous membranes, GI tract, CNS, joints, and peripheral vascular bed

Kawasaki Disease (mucocutaneous lymph node syndrome)

7

Diagnostic Criteria of Kawasaki Disease (5)

Dx of exclusion... must r/o infectious causes, rheumatologic conditions (JRA), drug rxns

 

Then:

  1. Fever > 102 lasting more at least 5 days
  2. 4 of the 5 criteria must be met
    1. Conjunctivitis-b/l (w/o exudate)
    2. Rash (primarily on trunk... erythematous maculopapular)
    3. Adenopathy (cervical) 
    4. Strawberry tongue (oropharyngeal changes: pharyngitis, red, cracked, swollen lips) 
    5. Hands and feet (edema early... peeling around nail beds late)

8

Most serious complication of Kawasaki disease

Coronary artery aneurysms (occur in 20% of untreated Kawasaki usually around days 7-14)

9

What is the time course and associated laboratory findings of Kawasaki Disease?

Triphasic:

  1. Phase I: acute phase (1-2 weeks)
    1. ESR, CRP
  2. Phase II: subacute phase (weeks to months)
    1. Platelet count, decreasing ESR and CRP
  3. Phase III: convalescent phase (weeks to yrs)
    1. Lab values usually normalize around 6-8 weeks

10

Kawasaki Disease: mgmt

IVIG & high dose aspirin

11

Distinguishing Features of Juvenile Rheumatoid Arthritis Subtypes

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12

Rare type of inflammatory arthritis that is similar to RA; shares characteristics of juvenile idiopathic arthritis but it begins in adulthood

Systemic-onset JRA (Still's Disease)

13

Symptoms of Still's Disease

  1. High spiking fevers (temperatures > 102.2) occur most commonly in the late afternoon or evening and subsequenlty return to baseline or subnormal levels
    1. Typically included in the differential dx of "fever of unknown origin"
  2. Transient salmon-colored rash most commonly found on trunk & proximal extremities, especially during febrile episodes
    1. Rash = evanescent (occurs with fever spikes and then fades) and nonpruritic 
  3. Hepatosplenomegaly
  4. Lymphadenopathy
  5. Other features:
    1. Fatigue, anorexia, weight loss, FTT
    2. Serositis
    3. CNS involvement (meningitis, encephalopathy)
    4. Myositis, tenosynovitis

14

Diagnostic Criteria for SLE*

 

*4 of 11 criteria provide sensitivity and specificity of 96%

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15

This inflammatory condition of muscle results in progressive muscle weakness with characteristic skin findings.

Dermatomyositis

16

Characteristic cutaneous findings

 

Dermatomyositis

  • Periorbital violaceous heliotrope rash
  • Gottron's papules

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17

Main treatment for dermatomyositis

Corticosteroids

18

Association with malignancy in pediatric dermatomyositis?

No

 

(In adult disease, malignancy develops in 25% of pts)

19

Major risk factor for Group A beta-hemolytic strep?

Pharyngitis

 

(Streptococcal strains that cause skin infection i.e., impetigo, do NOT cause rheumatic fever)

20

Laboratory findings in JIA

  • Elevated inflammatory markers (ESR, CRP)
  • Hyperferritinemia
  • Hypergammaglobulinemia
  • Thrombocytosis
  • Anemia (due to inc. production of hepcidin which inhibits iron absorption across the small intestine and iron release from macrophages and reticuloendothelial system of the liver)

21

How to confirm dx of B burgdorferi?

ELISA & Western Blot

22

ANA

Sensitive Lupus

23

Histone

Specific Drug-Induced Lupus

24

ds-DNA

Specific Lupus + Renal Involvement

25

Smooth Muscle

AI Hepatitis

26

Centromere

Scleroderma (CREST)

27

RO+LA

Sjogren's

28

CCP

Rheumatoid Arthritis

29

RF

Rheumatoid Arthritis

30

Jo

Polymyositis

31

Topoisomerase (Scl-70)

Systemic Scleroderma

32

Rheumatoid Arthritis + Neutropenia + Splenomegaly

Felty's Syndrome

33

What type of x-ray would you want to get a pt with RA?

Cervical spine to examine C1 and C2

34

RA: Treatment

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35

Urethritis + Arthritis + Uveitis

Reiter's Syndrome

 

Treat: Doxycycline + NSAIDs

36

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