Pediatric Rheumatology

Question 1

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

Answer 1

About equal (1:1)

Question 2

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

Answer 2

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

Question 3

What is the pathogenesis of systemic onsite juvenile idiopathic arthritis?

Answer 3

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

Question 4

What polymorphisms contribute to systemic onset juvenile idiopathic arthritis?

Answer 4

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

Question 5

What cytokines are increased in systemic onset juvenile idiopathic arthritis?

Answer 5

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

Question 6

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

Answer 6

• 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

Question 7

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

Answer 7

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

Question 8

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

Answer 8

No they are not common

Question 9

What are the symptoms of macrophage activation syndrome?

Answer 9

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

Question 10

What lab results are suggestive of macrophage activation syndrome?

Answer 10

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

Question 11

What pediatric disease is associated with macrophage activation syndrome?

Answer 11

Systemic onset juvenile idiopathic arthritis

Question 12

What is the treatment for macrophage activation syndrome?

Answer 12

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

Question 13

What is the therapy for systemic JIA?

Answer 13

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

Question 14

What demographics are at highest risk of Kawasaki disease?

Answer 14

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

Question 15

What is the pathogenesis of kawasaki disease?

Answer 15

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

Question 16

What are the three phases of Kawasaki disease?

Answer 16

Acute febrile (1-2 weeks)

Subacute (2-4 weeks)

Convalescent

Question 17

What is the diagnostic criteria of Kawasaki disease?

Answer 17

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

Question 18

What lab results are associated with kawasaki disease?

Answer 18

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

Question 19

What is the therapy for kawasaki disease?

Answer 19

IVIG and high dose aspirin as soon as possible

Question 20

What is the prognosis of kawasaki disease?

Answer 20

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

Question 21

What populations are most susceptible to IgA vasculitis?

Answer 21

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

Question 22

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

Answer 22

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

Question 23

What are the clinical signs of IgA vasculitis?

Answer 23

• 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

Question 24

What are the lab findings associated with IgA vasculitis?

Answer 24

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

Question 25

What is the therapy for IgA vasculitis?

Answer 25

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

Question 26

What is the prognosis of IgA vasculitis?

Answer 26

Very good, fewer than 5% progress to renal failure

Question 27

What demographics are most affectedby juvenile dermatomyositis?

Answer 27

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

Question 28

What are the diagnostic criteria for juvenile dermatomyositis?

Answer 28

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)

Question 29

What is the pathogenesis of juvenile dermatomyositis?

Answer 29

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

Question 30

What HLAs are associated withjuvenile dermatomyositis?

Answer 30

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

Question 31

What are the clinical signs of juvenile dermatomyositis?

Answer 31

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

Question 32

What muscle enzymes are high in juvenile dermatomyositis?

Answer 32

CK, AST, ALT, aldolase, and LDH

Question 33

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

Answer 33

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

Question 34

What are complications of juvenile dermatomyositis?

Answer 34

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

Question 35

What is the therapy for juvenile dermatomyositis?

Answer 35

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

Question 36

What is Kawasaki disease?

Answer 36

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

Question 37

What is IgA vasculitis?

Answer 37

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

Question 38

What is systemic JIA?

Answer 38

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

Question 39

What is juvenile dermatomyositis?

Answer 39

Inflammatory proximal myositis with vasculopathy and characteristic gottronpapules and heliotrope rash