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MSK/Derm > SM 241: Peds Rheumatology > Flashcards

SM 241: Peds Rheumatology Flashcards

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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
Q

What is Kawasaki Disease?

A

Systemic necrotizing vasculitis with a predilection for coronary arteries

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26
Q

What is a vasculitis?

A

Inflammation of blood vessels

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27
Q

What are the phases of Kawasaki Disease?

A

Acute febrile
Subacute
Convalescent

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28
Q

What occurs during Acute phase KD?

A

URI/GI symptoms + carditis/pericarditis

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29
Q

What occurs during Subacute phase KD?

A

Desquamation rash and aneurysms

30
Q

What occurs during Convalescent phase KD?

A

Mostly asymptomatic, but risk for aneurysms

31
Q

What kills people in KD?

A

Aneurysms - need constant screening and early treatment to prevent

32
Q

How does KD present on labs?

A

Sterile pyuria
Low Albumin
Elevated Liver enzymes and ESR/CRP
EKG and ECHO show carditis

33
Q

What is the treatment for KD?

A

High dose aspirin and IVIG within the first 10 days to reduce risk of aneurysm

34
Q

What is IgA Vasculitis?

A

Also called HSP, most common childhood vasculitis

35
Q

Who does IgA Vasculitis effect?

A

3 - 15 y/o males, slight male preference

36
Q

When during the year do most IgA Vasculitis cases develop?

A

Winter months

37
Q

What is the pathogenesis of IgA Vasculitis?

A

Infectious trigger from Group A Strep (Strep pyogenes) and an IgA mediated response to an antigen

38
Q

Which complement pathway does IgA vasculitis use?

A

Alternative complement pathway is triggered in IgA Vasculitis

39
Q

How does IgA Vasculitis present?

A

Palpable purpura in a dependent distribution on the legs/lower back
GI manifestations
Kidney involvement = hematuria/proteinuria

40
Q

What is a dependent distribution?

A

Distribution due to gravity and body position

41
Q

What are the GI manifestations of IgA Vasculitis?

A

Currant jelly stools
Colicklly abdominal pain

Due to vasculitis

42
Q

What presents with “currant jelly stools”?

A

IgA Vasculitis

43
Q

What does IgA Vasculitis carry a risk for?

A

Ileo-ileal intussusception, a medical emergency where the intestine telescopes inside itself

44
Q

How does IgA Vasculitis affect the kidney?

A

Microscopic hematuria and proteinuria

45
Q

Should you consider IgA Vascultiis if you see purpura + thrombocytopenia?

A

Nope - IgA Vasculitis does not cause thrombocytopenia

Consider DIC or Macrophage Activation Syndrome

46
Q

Why should you monitor blood pressure in IgA Vasculitis?

A

Kidney involvement + IgA antibodies can lead to IgA nephritis and hypertension

47
Q

How should IgA vasculitis be treated?

A

Symptomatic treatment with hydration and anti-inflammatories

Severe pain for steroids

48
Q

What drug can reverse the purpura rash in IgA Vasculitis?

A

There isn’t one - it goes away on it’s own

49
Q

What is the prognosis of IgA Vasculitis?

A

Lasts 4-6 weeks with some recurrence and a risk of renal failure, but prognosis is excellent

50
Q

What is Juvenile Dermatomyositis?

A

Inflammatory myopathy with vasculopathy that presents with muscle weakness and cutaneous rash

51
Q

What is the association between Juvenile Dermatomyositis and cancer in children?

A

There isn’t one in kids

There is one in adults tho

52
Q

Who does Juvenile Dermatomyositis effect?

A

Female predominant, age 7 y/o, mostly caucasians

53
Q

What are the diagnositc criteria for Juvenile Dermatomyositis?

A

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
Q

What causes Juvenile Dermatomyositis?

A

Infection + genetic susceptibility = myositits specific antibodies

55
Q

What makes the antibodies in Juvenile Dermatomyositis unique?

A

They are specific to myositis, a hallmark of Juvenile Dermatomyositis

56
Q

Describe the pathogenesis of Juvenile Dermatomyositis?

A

Antigen-immune complexes activate complement which damages vWF, occluding vessels and leading to infarction/ischemia of local tissues

57
Q

What findings on muscle biopsy are consistent with Juvenile Demratomyositis?

A

Perifascicular atrophy and Perivascular Infiltrate

58
Q

What is the cutaneous presentation of Juvenile Dermatomyositis and what causes it?

A

Vasculopathy due to immune complexes occluding vessels leads to vascular inflammation and edema on the extensor surfaces of hands - very characteristic finding

59
Q

How rapidly does Juvenile Dermatomyositis present?

A

Usually insidious with nonspecific symptoms such as fatigue and low grade fever

60
Q

What can worsen the rash in Juvenile Dermatomyositis?

A

Sun exposure, leading to rash on sun-exposed extensor surfaces

61
Q

What are Gottron’s Papules?

A

Hypertrophyic papular and scaly areas over the extensor surfaces of hands that are pathopneumoic for Juvenile Dermatomyositis

62
Q

What is a Helotrope rash?

A

A periorbital rash that crosses the nasal bridge, found in Juvenile Dermatomyositis

63
Q

What skin signs can be found in diffuse vasculopathy in Juvenile Dermatomyositis?

A

Diffuse vasculopathy correlates with severe disease, and causes nail bed telangiectasisa and infracts of oral/GI/skin

64
Q

What muscle signs can be seen in Juvenile Dermatomyositis?

A

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
Q

What is Gower’s sign?

A

Difficulty rising from the floor due to muscle weakness, seen in Juvenile Dermatomyositis

66
Q

What is Calcinosis cutis and what disease is it associated with?

A

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
Q

What enzymes are elevated in Juvenile Dermatomyositis?

A

Elevated muscle enzymes reflecting muscle damage:

CK
Aldolase
AST/ALT
Lactate DH

68
Q

What studies should be considered in Juvenile Dermatomyositis?

A

MRI for muscle damage, as well as muscle biopsy

69
Q

What is the treatment for Juvenile Dermatomyositis?

A

Oral and IV corticosteroids with Methotrexate for inflammation

Hydroxychloroquine for skin disease

70
Q

What is the prognosis of Juvenile Dermatomyositis?

A

If treated, excellent prognosis with deaths arising primarily due to lung disease from vasculitis

71
Q

What is essential in the treatment of Juvenile Dermatomyositis?

A

Aggressive early treatments to ensure remission and good prognosis

72
Q

How do KD, IgA Vasculitis, SJIA, and Juv Dermatomyositis all present?

A

Fever, Arthritis, Rash

MSK/Derm (46 decks)

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