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MSK/Derm > SM 241: Peds Rheumatology > Flashcards

SM 241: Peds Rheumatology Flashcards

1
Q

What is an autoimmune disease?

A

A disease characterized by the adaptive immune system losing tolerance to self antigen, often involving specific organs and antibodies

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2
Q

What is an autoinflammatory disease?

A

A disease characterized innate immune system dysfunction, due to endogenous factors

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3
Q

Which causes sterile inflammation, autoinflammatory disease or autoimmune disease?

A

Autoinflammatory disease is sterile inflammation because it’s driven by the innate immune system

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4
Q

What cells drive autoinflammatory disease?

A

The innate immune system: PMN’s and macrophages

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5
Q

What cells drive autoimmune disease?

A

The adaptive immune system: B-cells, T-cells

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6
Q

What is arthritis?

A

Inflammation of the synovial lining of a joint that leads to joint swelling, pain, limited ROM and warmth

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7
Q

Compare and contrast Arthralgia and Arthritis?

A

Arthralgia is joint pain alone, arthritis in joint pain in the context of inflammation

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8
Q

What defines Juvenile Idiopathic Arthritis?

A

Arthritis for more than 6 weeks in children < 16 y/o, as a diagnosis of exclusion

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9
Q

Why is the 6 week marker important in Juvenile Idiopathic Arthritis?

A

Reactive Arthritis typically resolves prior to 6 weeks

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10
Q

What are the 2 minimum criteria for Systematic Juvenile Idiopathic Arthritis and are they enough?

A

Symmetric polyarticular arthritis + spiking fever for at least 2 weeks; need a 3rd, 1 of 4

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11
Q

What are the potential 3rd criteria for SJIA, in addition to polyarticular arthritis and spiking fever?

A

Any of:

Evanescent Rash
Hepatosplenomegaly
Lymphadenopathy
Serositis = pericardial or pleural effusion

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12
Q

What is an Evanescent Rash?

A

Salmon colored rash that appears with a fever (disappears and reappears with spiking fever in SJIA)

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13
Q

What makes it hard to diagnose SJIA?

A

Not all symptoms present at the same time and the severity of presentation varies widely

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14
Q

What drives SJIA pathophysiology?

A

Abnormal cytokine expression: IL-1, IL-6, IL-18

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15
Q

How does SJIA appear on labs?

A

Anemia, Leuokocytosis
Elevated CRP/ESR/Ferritin
Prolonged PT/PTT

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16
Q

Which disease involves elevated Ferritin, and why is it significant?

A

Elevated Ferritin = SJIA; predicts Macrophage Activation Syndrome

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17
Q

What is Macrophage Activation Syndrome?

A

A severe complication of SJIA representing a hyperinflammatory state that can be fatal, due to cytokine storm

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18
Q

What does Macrophage Activation Syndrome look like on labs?

A

Low ESR
Pancytopenia
Really high Ferritin

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19
Q

How is SJIA treated?

A

Biologics: anti-IL-1 and anti-IL-6

Second line = methotrexate

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20
Q

What is the prognosis of SJIA?

A

Varied severity and may reccur

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21
Q

What are the criteria for Kawasaki Disease?

A

Fever for 5+ days as well as, at least 4 of:

Peripheral erythema/edema
Polymorphous rash
Bilateral non-exudative conjunctivitis
Strawberry tongue
Cervical Lymphadenopathy
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22
Q

Why is the conjuctivitis in Kawasaki Disease unique?

A

Non-exudative and spares the iris

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23
Q

Which demographics are most effected by Kawasaki Disease?

A

Japanese people, males less than 5 y/o

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24
Q

What triggers KD?

A

Unknown, probably an infectious trigger

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25
What is Kawasaki Disease?
Systemic necrotizing vasculitis with a predilection for coronary arteries
26
What is a vasculitis?
Inflammation of blood vessels
27
What are the phases of Kawasaki Disease?
Acute febrile Subacute Convalescent
28
What occurs during Acute phase KD?
URI/GI symptoms + carditis/pericarditis
29
What occurs during Subacute phase KD?
Desquamation rash and aneurysms
30
What occurs during Convalescent phase KD?
Mostly asymptomatic, but risk for aneurysms
31
What kills people in KD?
Aneurysms - need constant screening and early treatment to prevent
32
How does KD present on labs?
Sterile pyuria Low Albumin Elevated Liver enzymes and ESR/CRP EKG and ECHO show carditis
33
What is the treatment for KD?
High dose aspirin and IVIG within the first 10 days to reduce risk of aneurysm
34
What is IgA Vasculitis?
Also called HSP, most common childhood vasculitis
35
Who does IgA Vasculitis effect?
3 - 15 y/o males, slight male preference
36
When during the year do most IgA Vasculitis cases develop?
Winter months
37
What is the pathogenesis of IgA Vasculitis?
Infectious trigger from Group A Strep (Strep pyogenes) and an IgA mediated response to an antigen
38
Which complement pathway does IgA vasculitis use?
Alternative complement pathway is triggered in IgA Vasculitis
39
How does IgA Vasculitis present?
Palpable purpura in a dependent distribution on the legs/lower back GI manifestations Kidney involvement = hematuria/proteinuria
40
What is a dependent distribution?
Distribution due to gravity and body position
41
What are the GI manifestations of IgA Vasculitis?
Currant jelly stools Colicklly abdominal pain Due to vasculitis
42
What presents with "currant jelly stools"?
IgA Vasculitis
43
What does IgA Vasculitis carry a risk for?
Ileo-ileal intussusception, a medical emergency where the intestine telescopes inside itself
44
How does IgA Vasculitis affect the kidney?
Microscopic hematuria and proteinuria
45
Should you consider IgA Vascultiis if you see purpura + thrombocytopenia?
Nope - IgA Vasculitis does not cause thrombocytopenia Consider DIC or Macrophage Activation Syndrome
46
Why should you monitor blood pressure in IgA Vasculitis?
Kidney involvement + IgA antibodies can lead to IgA nephritis and hypertension
47
How should IgA vasculitis be treated?
Symptomatic treatment with hydration and anti-inflammatories Severe pain for steroids
48
What drug can reverse the purpura rash in IgA Vasculitis?
There isn't one - it goes away on it's own
49
What is the prognosis of IgA Vasculitis?
Lasts 4-6 weeks with some recurrence and a risk of renal failure, but prognosis is excellent
50
What is Juvenile Dermatomyositis?
Inflammatory myopathy with vasculopathy that presents with muscle weakness and cutaneous rash
51
What is the association between Juvenile Dermatomyositis and cancer in children?
There isn't one in kids There is one in adults tho
52
Who does Juvenile Dermatomyositis effect?
Female predominant, age 7 y/o, mostly caucasians
53
What are the diagnositc criteria for Juvenile Dermatomyositis?
Rash and 3 of 4: Symmetric proximal muscle weakness Elevation of skeletal muscle enzymes EMG or MRI findings of myositis Muscle biopsy findings consistent with JMD
54
What causes Juvenile Dermatomyositis?
Infection + genetic susceptibility = myositits specific antibodies
55
What makes the antibodies in Juvenile Dermatomyositis unique?
They are specific to myositis, a hallmark of Juvenile Dermatomyositis
56
Describe the pathogenesis of Juvenile Dermatomyositis?
Antigen-immune complexes activate complement which damages vWF, occluding vessels and leading to infarction/ischemia of local tissues
57
What findings on muscle biopsy are consistent with Juvenile Demratomyositis?
Perifascicular atrophy and Perivascular Infiltrate
58
What is the cutaneous presentation of Juvenile Dermatomyositis and what causes it?
Vasculopathy due to immune complexes occluding vessels leads to vascular inflammation and edema on the extensor surfaces of hands - very characteristic finding
59
How rapidly does Juvenile Dermatomyositis present?
Usually insidious with nonspecific symptoms such as fatigue and low grade fever
60
What can worsen the rash in Juvenile Dermatomyositis?
Sun exposure, leading to rash on sun-exposed extensor surfaces
61
What are Gottron's Papules?
Hypertrophyic papular and scaly areas over the extensor surfaces of hands that are pathopneumoic for Juvenile Dermatomyositis
62
What is a Helotrope rash?
A periorbital rash that crosses the nasal bridge, found in Juvenile Dermatomyositis
63
What skin signs can be found in diffuse vasculopathy in Juvenile Dermatomyositis?
Diffuse vasculopathy correlates with severe disease, and causes nail bed telangiectasisa and infracts of oral/GI/skin
64
What muscle signs can be seen in Juvenile Dermatomyositis?
Proximal muscle weakness with insidious onset, that manifests as difficutly climbing stairs, Gower's sign, perofm a sit up or raise head off of bed
65
What is Gower's sign?
Difficulty rising from the floor due to muscle weakness, seen in Juvenile Dermatomyositis
66
What is Calcinosis cutis and what disease is it associated with?
Muscle and fat atrophy in untreated juvenile dermatomyositis due to extensive vasculitis and insufficient treatment of inflammation Looks like calficifed nodules on skin, extremely painful
67
What enzymes are elevated in Juvenile Dermatomyositis?
Elevated muscle enzymes reflecting muscle damage: CK Aldolase AST/ALT Lactate DH
68
What studies should be considered in Juvenile Dermatomyositis?
MRI for muscle damage, as well as muscle biopsy
69
What is the treatment for Juvenile Dermatomyositis?
Oral and IV corticosteroids with Methotrexate for inflammation Hydroxychloroquine for skin disease
70
What is the prognosis of Juvenile Dermatomyositis?
If treated, excellent prognosis with deaths arising primarily due to lung disease from vasculitis
71
What is essential in the treatment of Juvenile Dermatomyositis?
Aggressive early treatments to ensure remission and good prognosis
72
How do KD, IgA Vasculitis, SJIA, and Juv Dermatomyositis all present?
Fever, Arthritis, Rash

MSK/Derm (46 decks)

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