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MSK/Derm > Pediatric Rheumatology > Flashcards

Pediatric Rheumatology Flashcards

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1
Q

What are the hallmarks of inflammation?

A

calor - heat

rubor - redness

tumor - swelling

dolor - pain

functio laesia - loss of function

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2
Q

immunological bases of autoinflammatory disease vs. autoimmune disease

A

autoinflammatory - perturbation of innate immunity

autoimmune disease - perturbation of adaptive immunity

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3
Q

cellular basis of autoinflammatory disease vs. autoimmune disease

A

inflammatory - neutrophils, macrophages

autoimmune - B cells, T cells, T regulatory cells

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4
Q

clinical recognition of autoinflammatory disease vs. autoimmune disease

A

inflammatory - family history, specific symptoms

autoimmune - MHC associations and autoantibodies

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5
Q

conceptual understanding of autoinflammatory disease vs. autoimmune disease

A

inflammatory - tissue-specific danger signals

autoimmune - breaking of self-tolerance

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6
Q

therapy of autoinflammatory disease vs. autoimmune disease

A

inflammatory - block cytokine cascades

autoimmune disease - block lymphocytes or their products

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7
Q

What is the epidemiology of juvenile idiopathic arthritis (sJIA)?

A

5-15% of children with JIA

male:female ratio is about equal

age at onset is any time in childhood

can occur in adults

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8
Q

How is arthritis classified in children?

A

joint swelling or effusion

OR

2 or more of the following:

  • limited range of motion
  • pain on range on motion
  • warmth
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9
Q

juvenile idiopathic arthritis

A

arthritis for 6 weeks

diagnosis of exclusion

onset type defined by type of disease in first 6 months

onset age 16 or younger

prevalence of chronic childhood arthritis is 20-100:100k

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10
Q

systemic JIA

A

arthritis, usually polyarticular:

  • large and small joints, usually symmetric

spiking quotidian (once or twice daily) fever

evanescent rash:

  • salmon colored patches or macules
  • trunk, proximal extremities, axilla, groin
  • appears with fever

hepatosplenomegaly, symmetric and generalized lymphadenopathy, serositis

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11
Q

What is the epidemiology of sJIA?

A

5-15% of children with JIA

any age including adults: stills

male = female

mild to severe

most morbidity and mortality of all JIA subtypes

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12
Q

What is the pathophysiology of sJIA?

A

IL-1, IL-6, and TNFalpha driven inflammation via abnormal cytokine expression - autoinflammatory

no consistent HLA associations

SNP in IL-6 regulatory region

polymorphism in macrophage inhibitory factor gene -> worse outcome

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13
Q

What are the clinical signs of sJIA?

A

symptoms may not all present simultaneously - increases in severity over time

fever can be isolated initially - high spiking to 39 C or above, 1-2x daily with rapid return to normal or below

10% have no arthritis at presentation

wide range of presentation from mild to critically ill

rash, serositis, generalized lymphadenopathy, hepatosplenomegaly

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14
Q

What are the important features of labwork in sJIA?

A

complete blood count signs of inflammation - anemia, leukocytosis, thrombocytosis

elevated ESR, CRP, ferritin, d-dimers

elevated LFTs especially LDH

signs of DIC - prolonged coags, high d-dimers, low fibrinogen

rare to be ANA+

RF+ is exclusion criteria

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15
Q

What is macrophage activation syndrome?

A

complication of sJIA

severe hyperinflammatory state - can be fatal

5-8% of sJIA patients

labs:

  • DIC -> low ESR, low fibrinogen
  • pancytopenia
  • elevated triglycerides, LFTs
  • very high ferritin
  • high D-dimers
  • hemophagocytosis
  • polyclonal elevation of immunoglobulins
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16
Q

What is the treatment for sJIA?

A

nonsteroidal anti-inflammatories

glucocorticoids

methotrexate

biologics - anti IL-1, anti IL-6 > anti-TNF

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17
Q

What is the prognosis of sJIA?

A

variable - monophasic, recurrent, persistent

about half of patients develop chronic, destructive polyarthritis

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18
Q

What is the clinical presentation of Kawasaki Disease?

A

fever for more than 5 days

plus at least four of the following:

  • changes in peripheral extremities (edema/erythema) or perineum - edema or erythema of hands or feet, periungual desquamation
  • polymorphous rash: erythematous macular, papular, annular, morbilliform, no vesicles
  • bilateral non-exudative conjunctivitis
  • changes of lips and oral cavity - injected and/or fissured lips, strawberry tongue, injected pharynx
  • cervical lymphadenopathy with at leas one node >1.5cm, usually unilateral
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19
Q

What is the epidemiology of KD?

A

highest incidence in Japan

in US - asian 39/100k > african american > hispanic > white (11/100k)

85% under age of 5, peak 2-3 years old

slight male predominance

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20
Q

What is the pathogenesis of KD?

A

likely an infectious trigger

superantigen stimulating large numbers of T cells

genetic predisposition

systemic necrotizing vasculitis with predilection for coronary arteries (small and medium size vessels

inflammatory infiltrate into vessels -> disruption of lamina elastica -> aneurisms

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21
Q

What is the disease course for Kawasaki Diseaes?

A

acute febrile phase: 10-14 days

subacute phase: 2-4 weeks

convalescent phase: months

22
Q

acute febrile phase of KD

A

10-14 days

preceding URI or GI symptoms

fever and cutaneous symptoms

carditis, pericarditis, abdominal pain

23
Q

subacute phase of KD

A

2-4 weeks

desquamation, arthritis, aneurisms

24
Q

convalescent phase of KD

A

lasts for months

asymptomatic

25
Q

What are the laboratory findings of KD?

A

increased ESR and CRP

elevated white blood cell count

anemia

platelets rise by 2nd week

ECG and echocardiogram - carditis, coronary aneurisms

26
Q

KD therapy

A

goal - control acute inflammation and symptoms

prevent long-term sequelae and aneurismss

high dose aspirin and IVIG within the first 10 days greatly reduces risk of coronary involvement

steroids, biologics if refractory to IVIG

27
Q

What is the prognosis of KD?

A

IVIG reduces incidence of aneurisms by 78%

5% of kids treated with IVIG develop aneurisms

almost always monophasic - very rare to recur

risk of cardiac death due to aneurisms

28
Q

What are the organs affected by Henoch-Schonlein Purpura?

A

purpuric rash in the skin

vasculitis of the kidney - proteinuria

abdominal pain with bloody diarrhea

arthritis

29
Q

What is the epidemiology of HSP?

A

most common childhood vasculitis

most frequent between 3-15 years

mean age of onset is approximately 7 years old

female-male ratio is 1:1.5

more cases in winter months

30
Q

What is the pathogenesis of HSP

A

could be triggered by Group A strep

30-50% preceded by URI

IgA-mediated dysregulated immune response to antigen

alternative complement pathway is triggered

familial clustering - HLA predisposition

31
Q

What are the clinical signs of HSP?

A

palpable purpura - dependent distribution that can be preceded by macules and urticaria

GI manifestations within a week of rash due to vasculitis

currant jelly stools

colicky abdominal pain - risk of intussusception

kidney involvement within 4-6 weeks of rash - microscopic hematuria, proteinuria, and renal failure

arthralgia, arthritis in 50-80% predominantly lower extremities

subcutaneous edema often periarticular

scrotal pain and swelling

32
Q

What are laboratory findings in HSP?

A

NO thrombocytopenia!

basic labs often unremarkable

elevated IgA with deposition in mesangial and GI vasculature

check urinalysis (not dip) with spot protein/creatinine and blood pressure

33
Q

What is the treatemtn for HSP?

A

symptomatic - hydration, bland diet, analgesia, anti-inflammatories

if severe abdominal pain, bloody stools, evaluate for intussusception and consider steroids

corticosteroids do not prevent renal disease but may be helpful when renal involvement is established

34
Q

What is the prognosis of HSP?

A

in 2/3 of cases, HSP runs entire course within 4 weeks (monophasic)

generally prognosis is excellent

<5% progress to renal failure

late kidney involvement rare but possible - monitor urinalysis

35
Q

What is juvenile dermatomyositis?

A

inflammatory myopathy

systemic vasculopathy

cutaneous findings

proximal symmetric myositis

progressive proximal muscle weakness

not associated with cancer in children

36
Q

What is the epidemiology of dermatomyositis?

A

incidence of 3.2/million children/year

73% of cases in the US are caucasian

female:male ratio is 2.3:1

average age at onset is 6.7 years but 25% are below 4

37
Q

What are the diagnostic criteria of juvenile dermatomyositis?

A

definite diagnosis reqires rash plus 3 or 4 of the following:

symmetrical proximal muscle weakness

elevation of skeletal muscle enzymes in serum

electromyographic findings typical of myositis

muscle biopsy findings typical of JDM

38
Q

What is the etiology of juvenile dermatomyositis?

A

UV exposure

antibodies - myositis specific and associated

genetic susceptibility - 80% have DQQA1*0501

cytokine polymorphisms: TNF-alpha

39
Q

What is the pathogenesis of juvenile dermatomyositis?

A

putative antigen and/or immune complexes activate endothelial cells

endothelium is damaged by complement activation - release of von Willebrand factor antigen

occlusion of capillaries and arterioles results in infarction of local tissues

cutaneous manifestations caused by capillary and arteriolar vasculopathy and occlusion

dermis - vascular inflammation and edema

thinned epidermis

40
Q

What are the systemic clinical signs of dermatomyositis?

A

disease onset is usually insidious

nonspecific symptoms - fatigue, low grade fever, weight loss, irritability, arthralgia, and abdominal pain

can be acute with high fever and profound clinical symptoms

41
Q

What are the clinical signs of juvenile dermatomyositis in the skin?

A

rash + weakness in 50% of cases

rash occurs first in 25% of cases

rash is prominent on sun-exposed surfaces

  • torso (shawl or V distribution)
  • extensor surfaces of extremities
  • scalp with hair loss due to chronic inflammation
42
Q

Gottron’s papules

A

a finding of juvenile dermatomyositis

hypertrophic, reddish pink areas of skin with are papular and scaly

occur over the MCP and interphalangeal joints as well as elbows and knees

heal as atrophic colorless bands

43
Q

heliotrope rash

A

a finding in juvenile dermatomyositis

periorbital violaceous erythema

may cross nasal bridge

44
Q

What does diffuse vasculopathy signify in juvenile dermatomyositis?

A

nail bed telangiectasia

infarction of oral epithelium and skin-folds

digital, skin and gastrointestinal ulceration

45
Q

What are the clinical signs in juvenile dermatomyositis pertaining to muscle?

A

proximal muscle weakness

often insidiuous

cmon signs include difficulty climbing stairs, getting on school bus, combing hair, arising from bed or chair

Gower’s sign - difficulty rising from floor

inability to raise head off bed (neck flexors)

inability to perform a sit-up (abdominals)

46
Q

What are other clinical signs of juvenile dermatomyositis?

A

hoarse voice, nasal speech

dysphagia - poor prognostic sign requiring aggressive mdial management

shortness of breath - interstitial lung diseae

constipation, hematochezia from weakness, gut vasculopathy

47
Q

What are the late clinical signs of juvenile dermatomyositis?

A

muscle and fat atrophy

calcinosis cutis - in subcutaneous tissue and fascia

  • 20% of patients
  • consequenece of delayed/insufficient suppression of inlammation
  • potential source of ulceration, infection
48
Q

What are the common laboratory findings in juvenile dermatomyositis?

A

elevated muscle enzymes reflecting leaky muscle membranes - can be subtle

- CK

- aldolase

- AST, ALT

- lactate dehydrogenase

elevated von Willebrand factor antigen reflecting disrupted endothelium

CRP/ESR usually normal

anemia is possible

RF is negative

kidney function tests are normal

myositis specific antibodies predict findings, prognosis

49
Q

What are the complications of juvenile dermatomyositis?

A

decreased bone density - increased fractures with increase disease duration

calcifications - associatated with TNF-alpha-308A, untreated long disease, major contributor to mortality and morbidity

partial lipodystrophy - associated with insulin resistant diabetes

50
Q

What is the treatment for juvenile dermatomyositis?

A

oral and IV corticosteroids

methotrexate

other steroids sparing immunosuppressants - cyclosporine, mycophenolate mofetil

IVIG for skin disease

hydroxycloroquine for skin disease

calcium and vitamin D

sunscreen at least SPF 45

51
Q

What is the prognosis of juvenile dermatomyositis?

A

current morality is 1%

long term outcome secondary to systemic vasculopathy is unclear - cardiovascular morbidity likely

long duration of untreated disease predicts poor outcome

aggressive treatment strategies decreases time to remission

MSK/Derm (54 decks)

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