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msk-derm > Pediatric Rheumatology > Flashcards

Pediatric Rheumatology Flashcards

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1
Q

What is the sex ratio of systemic onset of juvenile idiopathic arthritis?

A

About equal (1:1)

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2
Q

What is the age of onset of systemic onset juvenile idiopathic arthritis?

A

Can be any time in childhood, can also occur in adults

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3
Q

What is the pathogenesis of systemic onsite juvenile idiopathic arthritis?

A

It has a multifactorial disease caused by dysregulated innate immune response (autoinflammatory) to environmental stimuli leading to macrophage activation, T cell clonal expansion, and increased levels of IL-6, TNF-alpha, and IL-1

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4
Q

What polymorphisms contribute to systemic onset juvenile idiopathic arthritis?

A

Polymorphisms of IL-6, TNF, and IL-1 genes

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5
Q

What cytokines are increased in systemic onset juvenile idiopathic arthritis?

A

IL-6, TNF-alpha, and IL-1

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6
Q

What are the clinical signs of systemic onset juvenile idiopathic arthritis?

A
  • Arthritis minimal at onset, in any joint, increasing in severity over time
  • High spiking quotidian fever (1-2x daily)
  • Rash
  • Serositis (pericarditis, pleuritis)
  • Generalized lymphadenopathy
  • Hepatosplenomegaly
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7
Q

What inflammatory markers are found in lab results of patients with systemic onset juvenile idiopathic arthritis?

A

Leukocytosis, thrombocytosis, increased erythrocyte sediment rate (ESR), c-reactive protein (CRP), ferritin, d-dimers, polyclonal immunoglobulins

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8
Q

Are autoantibodies common in systemic onset juvenile idiopathic arthritis? Which ones?

A

No they are not common

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9
Q

What are the symptoms of macrophage activation syndrome?

A

Spiking fevers, enlarging liver and spleen, hemmorhage, CNS dysfunction

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10
Q

What lab results are suggestive of macrophage activation syndrome?

A

Dropping cell lines/Hb/platelets/WBCs, increasing TGs, decreasing ESR, hypofibrinogenemia, elevated d-dimers, extremely elevated ferritin

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11
Q

What pediatric disease is associated with macrophage activation syndrome?

A

Systemic onset juvenile idiopathic arthritis

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12
Q

What is the treatment for macrophage activation syndrome?

A

Cyclosporine (first IV, then oral), corticosteroids, anti IL-1

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13
Q

What is the therapy for systemic JIA?

A
  • oral and IV corticosteroids
  • immunosuppressive agents (methotrexate)
  • biologics (IL-1, IL-6 antagonists, CTLA4 antagonists)
  • calcium and vitamin D supplements
  • treat disease complications
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14
Q

What demographics are at highest risk of Kawasaki disease?

A
  • Asians at highest risk
  • Male predominance
  • Usually children under the age of 5
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15
Q

What is the pathogenesis of kawasaki disease?

A

Systemic necrotizing vasculitis with fibrinoid necrosis of medium-sized muscular arteries (especially coronary arteries) with inflammatory infiltrate into vessels, disruption of lamina elastica, and aneurysms

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16
Q

What are the three phases of Kawasaki disease?

A

Acute febrile (1-2 weeks)

Subacute (2-4 weeks)

Convalescent

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17
Q

What is the diagnostic criteria of Kawasaki disease?

A

Fever for more than 5 days plus at least 4 of the following:

  • bilateral bulbar conjunctival injection
  • oropharyngeal mucosal changes (injected and/or fissured lips, strawberry tongue, injected pharynx)
  • changes in peripheral extremities (edema or erythema of hands or feet, periungual desquamation)
  • polymorphous rash, primarily truncal, nonvesicular
  • cervical lymphadenopathy with at least one node > 1.5 cm, usually unilateral
18
Q

What lab results are associated with kawasaki disease?

A
  • increased acute phase reactants (ESR, CRP)
  • leukocytosis and anemia
  • EKG and echocardiogram findings
19
Q

What is the therapy for kawasaki disease?

A

IVIG and high dose aspirin as soon as possible

20
Q

What is the prognosis of kawasaki disease?

A

Rare recurrence, low mortality, risk depends on level of coronary involvement

21
Q

What populations are most susceptible to IgA vasculitis?

A

Mostly in children aged 3-15, slight male predominance, slight increase in cases in winter months

22
Q

What is the pathogenesis of IgA vasculitis/henoch-schonlein purpura?

A

30-50% preceded by URI, IgA mediated dysregulation of immune response, may involve alternative complement pathway

23
Q

What are the clinical signs of IgA vasculitis?

A
  • Nonthrombocytopenic palpable purpura (dependent distribution, range in size, changes color)
  • Subcutaneous edema
  • GI manifestations
  • Renal involvement (microscopic hematuria to mild proteinuria to renal failure)
  • Scrotal pain and swelling
  • Rarely CNS inflammation, pulmonary hemorrhage
24
Q

What are the lab findings associated with IgA vasculitis?

A

Elevated IgA with mesangial or GI vasculature deposition, proteinuria, HTN, or renal crescents

25
Q

What is the therapy for IgA vasculitis?

A

Hydration, bland diet, analgesia, reduction of intussusception, corticosteroids for GI/renal symptoms

26
Q

What is the prognosis of IgA vasculitis?

A

Very good, fewer than 5% progress to renal failure

27
Q

What demographics are most affectedby juvenile dermatomyositis?

A

Median age of onset is 7 years, but often presents lower. Female predominance (2:1). Usually preceding history of infection.

28
Q

What are the diagnostic criteria for juvenile dermatomyositis?

A

Must have gottron papules or heliotrope rash plus multiple of the following:

  • symmetric proximal muscle weakness
  • elevated muscle enzymes
  • characteristic electromyogram changes
  • characteristic muscle biopsy findings (necrosis, perifascicular atrophy, perivascular mononuclear infiltrate)
29
Q

What is the pathogenesis of juvenile dermatomyositis?

A

Frequently brought on by preceding illness, but can also be through UV light or other environmental factors

30
Q

What HLAs are associated withjuvenile dermatomyositis?

A

HLA-B*08, DRB1*Q301,DQA1*0301

31
Q

What are the clinical signs of juvenile dermatomyositis?

A

Rashes, weakness, pain, fever, dysphagia, difficulty speaking, abdominal pain, arthritis, calcifications, GI bleed

32
Q

What muscle enzymes are high in juvenile dermatomyositis?

A

CK, AST, ALT, aldolase, and LDH

33
Q

What lab findings besides muscle enzyme abnormalities are associated with juvenile dermatomyositis?

A

Nalifold capillary abnormalities, markers of immune and endothelial activation (neopterin, vWF antigen)

34
Q

What are complications of juvenile dermatomyositis?

A

Decreased bone density (increased fractures), calcifications, partial lipodystrophy (associated with insulin resistant diabetes)

35
Q

What is the therapy for juvenile dermatomyositis?

A

IV corticosteroid pulses, methotrexate, IV Igs, hydroxychloroquine, mixed response to biologics

36
Q

What is Kawasaki disease?

A

Systemic necrotizing medium vessel vasculitis with coronary artery predilection, prominent fevers, and polymorphous rash

37
Q

What is IgA vasculitis?

A

Self-limiting small vessel leukocytoclastic vasculitis characterized by palpable purpura and joint/GI/kidney disease

38
Q

What is systemic JIA?

A

Systemic autoinflammatory disease characterized by arthritis, fever, and rash often with organ involvement

39
Q

What is juvenile dermatomyositis?

A

Inflammatory proximal myositis with vasculopathy and characteristic gottronpapules and heliotrope rash

40
Q
A

msk-derm (41 decks)

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