SM_241a: Pediatric Rheumatology Flashcards Preview

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Flashcards in SM_241a: Pediatric Rheumatology Deck (42)
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1

Describe juvenile idiopathic arthritis

Juvenile idiopathic arthritis

  • Arthritis for 6 weeks
  • Diagnosis of exclusion
  • Onset type defined by type of disease in first 6 months
  • Onset 16th birthday or younger
  • Prevalence is 20-100 / 100k

2

Describe epidemiology of sJIA

sJIA epidemiology

  • 5-15% of children with JIA
  • Any age including adults
  • Male = female
  • Greatest morbidity and mortality of all JIA subtypes

3

sJIA can onset at ___ age and is ____ common in males compared to females

sJIA can onset at any age and is equally common in males compared to females

 

(greatest morbidity and mortality of all JIA subtypes)

4

Describe the clinical presentation of sJIA

sJIA clinical presentation

  • Arthritis (often polyarticular): large and small joints involved, usually symmetric
  • Spiking quotidian (1-2x/daily) fever for at least three weeks
  • One or more of evanescent rash, hepatosplenomegaly, lyphadenopathy symmtric and generalized, and serositis (pericardial or pleural effusions)

5

_____ rash is seen in sJIA

Evanescent rash is seen in sJIA

  • Salmon colored patches or macules
  • Trunk, proximal extremities, axilla, and groin
  • Appears with fever

6

Describe clinical signs of sJIA

sJIA clinical signs

  • Symptoms may not all present simultaneously
  • Fever can be isolated initially
  • 10% have no arthritis at presentation
  • Wide range of presentation from mild to critically ill

7

Describe sJIA pathophysiology

sJIA pathophysiology

  • IL-1, IL-6, and IL-18 driven inflammation via abnormal cytokine expression - autoinflammatory
  • No consistent HLA associations
  • T-lymphocyte abnormalities later in course of disease

8

sJIA inflammation is driven by abnormal expression of ____, ____, and ____ cytokines (autoinflammatory)

sJIA inflammation is driven by abnormal expression of IL-1, IL-6, and IL-18 cytokines (autoinflammatory)

 

 

(treated by glucocorticoids and anti IL-1 and anti IL-6 biologics)

9

Describe sJIA labwork

sJIA labwork

  • CBC signs of inflammation: anemia, leukocytosis, thrombocytosis
  • Elevated ESR, CRP, ferritin
  • Elevated LFTs
  • Coagulation changes: prolonged PT/PTT, high d-dimers, and high fibrinogen

10

____ is a severe hyperinflammatory state that is a complication of sJIA

Macrophage activation syndrome is a severe hyperinflammatory state that is a complication of sJIA

11

Describe macrophage activation syndrome

Macrophage activation syndrome

  • Complication of sJIA: severe hyperinflammatory state that can be fatal, 10% of sJIA patients but subclinical in 30-40%
  • Secondary hemophagocytic lymphohistiocytosis
  • Labs: DIC -> low ESR, low fibrinogen, pancytopenia, elevated triglycerides, LFTs especially LDH, sky-high ferritin, hemophagocytosis in BM

12

sJIA therapy includes ____ and ____

sJIA therapy includes glucocorticoids and anti IL-1 and anti IL-6 biologics 

 

(early biologic therapy alters course of disease)

13

Describe Kawasaki disease diagnostic criteria

Kawasaki disease diagnostic criteria

  • Fever for ≥ 5 days
  • Plus at least four of: changes in peripheral extremities (edema / erythema) or perineum, polymorphous rash, bilateral non-exudative conjunctivitis, changes of lips and oral cavity, and cervical lymphadenopathy
  • Arthralgia is uncommon, arthritis is rare

14

____ rash is present in Kawasaki disease

Polymorphous rash is present in Kawasaki disease

 

 

(erythematous macular, papular, annular, morbiliform, no vesicles)

15

Describe Kawasaki disease epidemiology

Kawasaki disease

  • Highest incidence in Japan
  • In US, most common among Asians
  • 85% under age 5, peak at 2-3 years old
  • Slight male predominance

16

Kawasaki disease is most common in ____ and ____ and onsets most often before age ____

 

(treated with aspirin and IVIG in first 10 days)

Kawasaki disease is most common in Asians and men and onsets most often before age 5

17

Describe Kawasaki disease pathogenesis

Kawasaki disease pathogenesis

  • Likely infectious trigger
  • May be superantigen stimulating large numbers of T cells
  • May be genetic predisposition
  • Systemic necrotizing vasculitis with predilection for coronary arteries (small and medium size vessels)

18

Kawasaki disease likely has a(n) ____ trigger and involves ____

Kawasaki disease likely has an infectious trigger and involves systemic necrotizing vasculitis with predilection for coronary arteries (small and medium size vessels)

19

Disease course of Kawasaki disease involves ____, ____, and ____ phase

Disease course of Kawasaki disease involve acute febrile phase, subacute phase, and convalescent phase

 

  • Acute febrile phae (10-14 days): preceding URI or GI symptoms, fever and cutaneous symptoms (criteria), carditis / pericarditis / abdominal pain / irritability
  • Subacute phase (2-4 weeks): desquamation, arthritis, and aneurysms
  • Convalescent phase (months): asymptomatic

20

Describe Kawasaki disease laboratory data

Kawasaki disease laboratory data

  • Increased ESR and CRP
  • Elevated WBC count
  • Anemia
  • Platelets rise by 2nd week
  • Transaminitis, sterile pyuria, low albumin common
  • ECG and echo: carditis and coronary aneurysms
  • Shock and macrophage activation syndrome can occur

21

Kawasaki disease therapy involves ____ and ____

Kawasaki disease therapy involves aspirin and IVIGz

 

  • Reduces risk of coronary involvement if within first 10 days

22

Describe epidemiology of IgA vasculitis

IgA vasculitis epidemiology

  • Most common childhood vasculitis
  • Most frequent between 3-15 yo: rare but very severe in adults
  • Female to mael: 1:1.5
  • More cases in winter months

23

IgA vasculitis is the ____ vasculitis, is predominant in ____, and manifests primarily in ____ months

IgA vasculitis is the most common vasculitis, is predominant in males, and manifests primarily in winter months

24

____ rash is in IgA vasculitis

Palpable purpura rash is in IgA vasculitis

 

  • Dependent distribution
  • Can be preceded by macules and urticaria

25

Describe pathogenesis of IgA vasculitis

IgA vasculitis

  • Infectious trigger: Group A strep, preceded by URI
  • IgA-mediated dysregulated immune response to antigen
  • Alternative complement pathway is triggered
  • Familial clustering: HLA prediposition

26

IgA commonly occurs after a ___, involves ___ and ___, and has a ___ prediposition

IgA commonly occurs after a URI, involves IgA-mediated dysregulated immune response to antigen and triggered alternative complement pathway, and exhibits HLA predisposition

27

Describe clinical signs of IgA vasculitis

IgA vasculitis clinical signs

  • Palpable purpura: dependent distribution, can be preceded by macules / urticaria
  • GI manifestations due to vasculitis: currant jelly stools, colicky abdnominal pain (risk of ileo-ileal intussusception)
  • Kidney involvement (30%) with 3-6 weeks of rash: microscopic hematuria, proteinuria, renal failure
  • Arthralgia, arthritis in 50-80% predominantly lower extremities
  • Subcutaneous edema: often periarticular
  • Scrotal pain, swelling

28

IgA lab data shows ____ and is often ____

IgA lab data shows NO thrombocytopenia and is often unremarkable

 

(elevated IgA, check urinalysis and BP)

29

Juvenile dermatomyositis is ____, has ____, involves progressive, and is ____

Juvenile dermatomyositis is inflammatory myopathy with vasculopathy, has cutaneous findings, involves progressive muscle weakness, and is NOT associated with cancer in children

30

Describe epidemiology of juvenile dermatomyositis

Juvenile dermatomyositis

  • Incidence: 1-3 million children/year
  • Most common in Caucasians
  • Female predominance
  • Average age at onset 6.7 years but 25% are below age 4

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