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MSK-Derm by Minnie AH Version > 242: Peds Rheumatology > Flashcards

242: Peds Rheumatology Flashcards

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1
Q

What is the prognosis of JDM?

A

Much better now that steroid treatment is available

Must be treated early to prevent aggressive calcinosis, long-term complications

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2
Q

Which of the following accurately describes the fever pattern seen in systemic JIA?

  1. Daily fever for 5 days or more without a quotidian pattern
  2. Daily fever for 2 weeks or more which is persistently elevated for at least 3 days
  3. Daily fever for 2 weeks with at least 3 days of quotidian pattern
A

c. Daily fever for 2 weeks with at least 3 days of quotidian pattern

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3
Q

True or False?

Distal muscle weakness is characteristic of juvenile dermatomyositis.

A

False

Muscle weakness in JDA is proximal

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4
Q

True or False?

Ultraviolet radiation plays a role in the pathogenesis of juvenile dermatomyositis.

A

True

Look for rash in sun-exposed ares on presentation

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5
Q

True or False?

Edema of the hands and feet can be seen in both IgA vasculitis and Kawasaki disease.

A

True

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6
Q

What is the treatment for JDM?

A

Oral and IV corticosteroids + Methotrexate

  • Can also use:
    • Cyclosporine, other steroid-sparing immunosuppresants
    • IVIG for skin disease
    • Hydroxychloroquine for skin disease
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7
Q

True or false?

Kawasaki disease is the leading cause of acquired heart disease in children in the U.S.

A

True

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8
Q

What are the clinical signs of IgA Vasculitis?

A
  • Palpable purpura
  • GI manifestations
    • Currant jelly stools
    • Colicky abdominal pain
  • Kidney
    • Microscopic hematuria, proteinuria, renal failure
  • Arthralgia, arthritis
  • Subcutaneous edema
  • Scrotal pain, swelling
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9
Q

What defins JIA?

A

Arthritis for 6+ weeks

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10
Q

True or false:

Joint pain on range of motion is sufficient to diagnose arthritis.

A

False

If no swelling or effusion, need 2 or more of the following:

  • Limited range of motion
  • Tenderness
  • Pain on range of motion
  • Warmth
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11
Q

True or False?

Normal muscle enzyme measurements at the time of presentation rule out the diagnosis of juvenile dermatomyositis

A

False

May have gone up and down by the time the patient presents, or skin disease may be much more prominent than muscle

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12
Q

What is the leading cause of coronary artery disease in children?

A

Kawasaki disease

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13
Q

True or False

The pathogenesis of systemic JIA is due to defects in adaptive immunity and anti-inflammatory pathways.

A

False

Pathogenesis is driven by dysfunction in innate immunity

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14
Q

What laboratory findings are consistent with a diagnosis of JDM?

A

Elevated muscle enzymes

  • Creatine kinase
  • Aldolase
  • AST, ALT
  • Lactate DH
  • CRP, ESR normal
  • RF negative
  • Kidney function norml
  • Myositis specific antibodies predict findings, prognosis
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15
Q

What are the general differences between autoimmune diseases and autoinflammatory diseases?

A
  • Autoimmune
    • Loss of tolernce to self
    • Adaptive immune system is dysfunctional
      • Often autontibody mediated
    • Thereapeutic goal is to block lymphocytes or products
  • Autoinflammatory
    • Innate immune system is dysfunctional
      • Often mediated by endogenous factors
      • -> elevated acute phase reactants
    • Therapeutic goal is to block cytokine cascade
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16
Q

Which childhood rheumatic disease is this characteristic of?

Spiking, quotidian fever

A

Systemic JIA

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17
Q

What is calcinosis cutis?

What pediatric rheumatic syndrome is it associated with?

A

Juvinile dermatomyositis

  • Occurs late in the course
  • Usually only appears if diagnosis is missed or treatment is incomplete
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18
Q

True or False?

To treate Kawasaki disesae, intravenous immunoglobulin (IVIG) should be given before the 5th day of fever onset to reduce the risk of coronary involvement.

A

False

Must be given within 10 days of fever onset

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19
Q

What are the mainstays of Kawasaki disease treatment?

A

Aspirin + IVIG

Must be given within the first 10 days to reduce risk fo coronary involvement

  • Steroids, biologics if refractory to IVIG
    • Give steroids up front if known CAA, high risk demographic factors
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20
Q

What lab results are consistent with sJIA?

A
  • CBC
    • Anemia
    • Leukocytosis
    • Thrombocytosis (high platelets)
  • Elevated ESR, CRP, Ferritin
  • Elevated LFT
  • Coagulation changes
    • Prolonged PT/PTT
    • High D-dimer
    • High fibrinogen
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21
Q

True or False?

Patients with IgA vasculitis have purpura with thrombocytopenia

A

False

No thrombocytopenia!

22
Q

What is the prognosis of IgA vasculitis?

A

Usually prognosis is good!

Usually runs its course in 4-6 weeks, recurrence is not common and usually less severe

Rash does not scar

23
Q

What is the most common childhood vasculitis?

A

IgA vasculitis

24
Q

What are the diagnostic criteria for juvenile dermatomyositis?

A

Rash plus 3/4 of the following:

  • Symmetric proximal muscle weakness
  • Elevation of skeletal muscle enzymes
  • Elextromyographic findings typical of myositis
  • Muscle biopsy findings typical of JDM
25
Q

What is the difference between arthralgia and arthritis?

A

Arthralgia = joint pain without inflammatory features

Arthritis = has signs of inflammation

26
Q

What laboratory data is consistent with IgA Vasculitis

A
  • No thrombocytopenia!
  • Basic labs are usually unremarkable
  • Elevated IgA with deposition in mesangial and GI vasculature
  • Check urinalysis
    • Hematuria if kidney involvement
27
Q

What are the major findings of JDM on muscle biopsy?

A

Perivascular infiltrate

Atrophy of myofibrils

28
Q

The diagnosis of systemic JIA requires arthritis in how many joints?

A

At least 1

29
Q

How is sJIA treated?

A
  • 1st line
    • Glucocorticoids
    • Cytokine-directed biologic therapy
      • Anti IL-1, IL-6
  • 2nd line
    • DMARDs
      • Methotrexate
      • Leflunomide
    • Other biologics
30
Q

What kind of rash is characteristic of IgA vasculitis?

A

Palpable purpura

Can be preceded by macules, urticaria

31
Q

Which childhood rheumatic disease is this characteristic of?

Palpable purpura

A

IGA vasculitis

32
Q

What pro-inflammatory cytokines drive the inflammation in sJIA?

A

IL-1, IL-6, IL-18

33
Q

Which juvenile rheumatic disorder is characterized by insidious proximal muscle weakness?

A

Juvinile dermatomyositis

34
Q

Describe the pathogenesis of IgA vasculitis

A
  • Infectious trigger: Group A Strep or URI
    • IgA-mediated dysregulated immune response to the antigen
  • Alternative complement pathway activated
  • HLA genetic predisposition
35
Q

What is macrophage activation syndrome?

It is a complication of which pediatric rheumatic syndrome?

A

Systemic JIA

  • Severe hyperinflammatory state
  • Low ESR, low fibrinogen, very high ferritin
36
Q

These findings, plus a fever for ≥5 days, would increase your suspicion for which pediatric disorder?

A

Kawasaki disease

  • Desquamative rash
  • Changes of lips and oral cavity
  • Non-exudative conjunctivitis (bilateral)
  • Edema in the extremities
37
Q

What laboratory findings are consistent with Kawasaki disease?

A
  • ESR and CRP elevated
  • WBC count elevated
  • Anemia
  • Thrombocytosis by the 2nd week
  • Transaminitis, sterile pyuria, low albumin

Labs useful when patient presents with something that looks like Kawasaki but maybe doesn’t have all 4 of the necessary criteria

38
Q

What are the late clinical signs of JDM?

A

Usually only seen if the diagnosis is missed, or treatment is insufficient - less common now

  • Muscle and fat atrophy
  • Calcinosis cutis
39
Q

What are the diagnostic criteria for Kawasaki disease?

A

Fever ≥ 5 days

Plus at least 4 of the following:

  • Edema/erythema in peripheral extremities or perineum
  • Polymorphous rash
    • Erythematous, macular, papular, annular, morbilliform, no vesicles
    • Bascially any rash that is not vesicular
  • Bilateral, non-exudative conjunctivitis
  • Changes of lips and oral cavity
  • Cervical lymphadenopathy

Pt may have joint pain, but arthritis is rare

40
Q

Mucocutaneous manifestations of Kawasaki disease at onset include all of the following except:

  1. Erythema and cracking of the lips
  2. Vesicular, polymorphous rash on the trunk
  3. A red tongue with prominent fungiform papillae
  4. Desquamation of the hands
  5. Edema of the hands and feet
A

b. Vesicular, polymorphous rash on the trunk

Rash is not vesicular; can be basically any rash that is not vesicular

41
Q

Which childhood rheumatic disease is this characteristic of?

Treat early with aspirin and IVIG to prevent aneurysm

A

Kawasaki disease

42
Q

What is the treatment for IgA vasculitis?

A
  • If symptomatic
    • Hydration
    • Bland diet
    • Analgesia
    • Anti-inflammatories
    • Steroids for severe abdominal pain, arthritis
      • Will not help the rash
43
Q

True or False?

Colicky abdominal pain is a hallmark feature of IgA vasculitis

A

True

44
Q

Describe the clinical signs of JDM

A

Rash + proximal muscle weakness

  • Nonspecific, insidious onset
  • Rash on sun-exposed surfaces
    • Gottron’s papules
    • Heliotrope rash of the face
  • Proximal muscle weakness
    • Insidious onset - trouble climing stairs, getting on the bus, combing hair
  • Diffuse vasculopathy of the skin
    • Correlates with severe disease
    • Nail bed telangiectasia, infarction of roal epithelium and skin folds
    • Digital, skin, GI ulceration
45
Q

When in the course of Kawaski disease are patients at highest risk for aneurysm?

A

Subactue phase

2-4 weeks after symptoms begin

Caused by weaknesses in vascular tissue following

46
Q

What is the diagnostic criteria for systemic juvenile idiopathic arthritis?

A

Arthritis + Fever + one or more of the following:

  • Evanescent rash
    • Salmon colored patches or macules, appears with fever
  • Hepatosplenomegaly
  • Lymphadenopathy
  • Serositis
    • Pericardial or plerual effusions
47
Q

Which childhood rheumatic disease is this characteristic of?

Muscle weakness, heliotrope rash

A

Juvenile dermatomyositis

48
Q

Describe the “typical” Kawasaki patient

A
  • Japanese descent
  • <5 years old
  • Male (slight predominance)
49
Q

Describe the course of Kawasaki disease

A
  • Acute, Febrile Phase
    • 10-14 days
    • Preceding URI or GI symptoms
    • Carditis, pericarditis, abdominal pain, irritability
  • Subacute phase
    • 2-4 weeks
    • Desquamation, arthritis, aneurysm
  • Convalescent phase
    • Months
    • Asymptomtic
50
Q

Which of the following does not describe the rash seen in IgA vasculitis?

  1. Palpable purpura
  2. Dependent distribution
  3. Can start as maculopapular lesions
  4. Erythema of the perineum
  5. Rashes appear and start to fade, then another episode of rash occurs
A

e. Rashes appear and start to fade, then another episode of rash occurs

(Pretty sure this is right, but no answer on the slide)

51
Q

If a patient with sJIA has an unexplained drop in ESR, what syndrome would you worry about?

What other findings would be consistent with this diagnosis?

A

Macrophage activation sydrome

  • Very high ferritin
  • Pancytopenia
  • Elevated triglycerides

MSK-Derm by Minnie AH Version (47 decks)

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