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Peds-Final > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

rheumatic dz’s are characterized by what/

A

autoimmunity & inflammation

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2
Q

rheumatic dz’s DDx

A

active infxns
post-infectious phenomena
malignancies

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3
Q

rheumatic dz is characterized by what arthritis (synovitis)….

A 
enthesitis
serositis
myositis
vasculitis
activation of reticuloendothelial system
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4
Q

activation of the reticuloendothelial system causes what?

A

lymphadenopathy

in systemic-onset JRA & SLE- HSM

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5
Q

chronic inflammation in rheumatic dz’s can lead to what?

A

growth delay & disability

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6
Q

what is the hallmark of rheumatic dz?

A

chronicity

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7
Q

radiographic joint findings in rheumatic dz’s

A

lag far behind symptoms

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8
Q

what is one of the MC vasculitides of CH?

A

Kawasaki dz

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9
Q

theorized pathology of Kawaski dz

A

abnormal immune response to some super-antigen

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10
Q

epidemiology of Kawasaki dz

A 
peak age 2-3 yr
rare over age 7
peaks bet. Feb & May
highest incidence in Japan
MCC of acquired heart dz in CH
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11
Q

Kawasaki dz diagnostic criteria

A

fever for 5+ days!!!!
presence of 4 of the following:
1. bilateral nonpurulent conjunctivitis w/ perilimbic sparing (MC)
2. changes in the oropharyngeal mucous membranes
3. extremity changes (most often swelling & redness of the hands & ft)
4. rash (>80% of CH)
5. cervical adenopathy (>1.5 cm node, 70% of CH)
illness unexplained by other dz

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12
Q

some of the changes in oropharyngeal membranes you might see in Kawasaki dz

A

injected pharynx
injected lips
dry or cracked lips
strawberry tongue

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13
Q

some changes in the perpheral extremities you might see in Kawasaki dz

A

edema or erythema
desquamation
periungual desquamation

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14
Q

what type of rash might you see in Kawasaki dz?

A

mostly truncal

polymorphic but not vesicular

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15
Q

acute phase of Kawasaki dz

A

affected CH are generally irritable
abdominal pain is common
hydrops of the GB, CSF pleocytosis, arthritis & carditis can manifest in acute phase

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16
Q

subacute phase of Kawasaki dz

A

thrombocytosis
desquamation of groin, hands & feet
coronary aneurysms (up to 20% of untreated CH)- higher risk in boys, CH w/ prolonged fever or inflammatory markers, age under 1 yr

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17
Q

lab work-up in Kawasaki dz

A 
increased WBC
increased ESR, CRP
anemia
mild increase in transaminases
decreased albumin
sterile pyuria
CSF pleocytosis
increased platelets by day 10-14
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18
Q

you can use an echocardiography to search for what cardiovascular complications in Kawsaki dz

A

myocarditis
pericarditis
evidence of coronary vasculitis

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19
Q

viral exanthems

A 
measles
rubella
roseola
adenovirus
EBV
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20
Q

staphylococcal or streptococcal toxin-mediated syndrome

A

staphy scalded skin syndrome
scarlet fever
streptococcal toxic shock

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21
Q

difficulties w/ dx KD

A

no single test is diagnostic
Kawasaki dz remains a dx of exclusion
atypical Kawasaki dz- when illness does not fulfill all criteria; more common 8 yo

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22
Q

KD tx

A
  • admit to monitor cardiac function
  • r/o other causes of similar dz
  • complete cardiac eval (EKG & echocardiogram)
  • IVIG (2mg/kg)- reduces coronary aneurysm rate to s persist or recur
  • aspirin
  • f/u echocardiography in 6-8 wks after IVIG
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23
Q

aspirin tx in KD

A

high-dose for anti-inflammatory effect in first 24-96 hrs

low dose for anti-thrombotic activity pending f/u echocardiography for late-onset coronary aneurysms

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24
Q

SLE

A

deposition of immune complexes in tissue which activates lymphocytes, neutrophils, & complement

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25
Q

SLE is a multisystem inflammatory dz & may include what?

A 
joints
serous linings 
skin 
kidneys
CNS
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26
Q

epidemiology of SLE

A

female predominant
median age for peds ~12 yo (uncommon before age 4 yo)
Asians>blacks>hispanics>caucasians
health disparity-Caucasians have better survival rates
peds presenting sx’s may be vague

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27
Q

MC findings in SLE

A 
fever
abdominal complaints
malar rash &/or oral ulcers
MS arthritis or arthralgias
hematologic anemia, leukopenia, thrombocytopenia
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28
Q

crash & burn pneumonic for KD

A 
Conjunctivitis
Rash
Adenopathy
Strawberry tongue
Hands & feet

burn (fever)

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29
Q

renal findings in CH w/ SLE

A

hematuria
proteinuria
mild hypertension
diffuse proliferative glomerulonephritis

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30
Q

neurologic findings in CH w/ SLE

A 
HA
deterioration of academic performance
withdrawal
social isolation
depression
*full hx & PE important in teens, screening CBC, ESR reasonable in vague complaints
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31
Q

more dramatic neurologic presentations in SLE

A

stroke
seizures
chorea
coma

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32
Q

hematologic findings in CH w/ SLE

A

thrombocytopenia, hemolytic anemia, leukopenia (together will lead to sending an ANA)
f/u studies when suspecting SLE: antiphospholipid Ab’s, lupus anticoagulant activity

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33
Q

cardiopulmonary findings in CH w/ SLE

A

increased risk of atherosclerosis & CAD
increased risk of pericarditis, endocarditis
pleural effusions

34
Q

diagnostic criteria of SLE

A 
malar rash
discoid rash
photosensitivity
oral ulcers
arthritis
serositis
renal d/o
neruologic d/o
hematologic d/o
immunologic d/o
anti-nuclear Ab
35
Q

malar rash in SLE

A

fixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds

36
Q

discoid rash in SLE

A

erythematous, raised patches w/ adherent keratotic scaling & follicular plugging

37
Q

photosensitivity in SLE

A

skin rash as a result of unusual rxn to sunlight

38
Q

oral ulcers in SLE

A

usually painless

39
Q

arthritis in SLE

A

nonerosive arthritis involving 2+ peripheral joints, characterized by tenderness, swelling, or effusion

40
Q

serositis in SLE

A

pleuritis or pericarditis

41
Q

renal d/o in SLE

A

persistent proteinuria, cellular casts

42
Q

neurologic d/o in SLE

A

seizures or psychosis

43
Q

hematologic d/o SLE

A

hemolytic anemia, leukopenia, lymphopenia, thrombocytopenia

44
Q

immunologic d/o in SLE

A

anti-phospholipid Ab, anti-DNA, anti-Sm, false positive syphilis serology

45
Q

anti-nuclear Ab in SLE

A

abnormal titer of ANA by immunofluorescence or an equivalent assay

46
Q

labs for dx in SLE

A

ANA
CBC w/ leukopenia, anemia
complement level
ESR

47
Q

checking for dz severity in SLE

A

anti-dsDNA Ab more specific (also Abs to Sm nuclear antigen)

antiphospholibid Abs as anti-cardiolipin Ab, lupus anticoagulant or false + RPR or VDRL over 6 months

48
Q

UA & renal function in SLE

A

BUN
Cr
renal dz is a major component of SLE

49
Q

SLE therapy

A

corticosteroids
cyclophosphamide-early therapy improves survival
steroid-sparing agents
non-steroidal anti-inflammatory drugs

50
Q

steroid sparing agents for SLE

A

methotrexate
azathiprine
mycophenolate
mofetil

51
Q

non-steroidal anti-inflammatory drugs for SLE

A

hydroxychloroquine

dapsone

52
Q

challenges in CH w/ SLE

A

poor compliance
renal dz- 30% will go on to renal failure
neurologic complications
intercurrent infxns

53
Q

what is the most prevalent chronic rheumatologic dz of CH

A

juvenile rheumatoid arthritis (JIA- juvenile idiopathic arthritis?)

54
Q

what are the 3 types of JIA/JRA

A

pauciarticular (~50%) /= 5 joints

systemic onset

55
Q

what is JIA/JRA

A

non-migratory arthropathy w/ a tendency to involve large joints or proximal interphalangeal joints lasting over chronic period of months

56
Q

when is pain usually worse in JIA/JRA

A

morning

joint swelling w/ diminished mobility

57
Q

JIA/JRA complicated by uveitis

A

MC treatable cause of blindness in CH

ANA-positive CH are more likely to have chronic uveitis

58
Q

pauciarticular onset JIA/JRA is more common in girls or boys?

A

girls

59
Q

what are the clinical manifestations of pauciarticular onset JRA

A
  • arthritis of large joints (knee>ankle>wrist)
  • uveitis is only systemic manifestation
  • involves < 5 joints
  • ANA often +
  • rheumatoid factor often neg
  • may have no fever/abnormalities of inflammatory markers (WBC, ESR, CRP)
  • 90% have complete remission
60
Q

complications of pauciarticular onset JRA

A

uveitis untreated may progress toward blindness

inflammation may cause bony changes that distort growth

61
Q

what mild systemic complaints may exist w/ JRA

A

low grade fever
anemia
malaise

62
Q

JRA can result in________growth

A

asymmetric

63
Q

polyarticular onset JRA is more common in girls or boys?

A

girls

*> 50% have complete remission

64
Q

manifestations of polyarticular onset JRA between ages 2-5 yo

A
  • progressive involvement of joints
  • symmetrical joint involvement
  • uveitis
  • can present w/ systemic sx’s
65
Q

symmetrical joint involvement in polyarticular onset JRA

A

small joints of the hands & feet
ankles, wrists, knees
may cause fusion of the cervical spine

66
Q

some systemic sx’s that may present w/ polyarticular onset JRA

A 
malaise
fever
growth retardation
anemia
elevated inflammatory markers
67
Q

manifestations of polyarticular onset JRA ages 10-14 yo

A

arthritis of rapid onset
small joints of hands & feet typically involved
adolescents that are rheumatoid-factor + probably have true adult rheumatoid arthritis

68
Q

systemic onset JRA affects boy or girls more?

A

affects both sexes equally

69
Q

manifestations of systemic onset JRA

A

typically present originally without arthritis systemic sx’s

dx often made after elimination of other dz’s, as sx’s become evidently chronic

70
Q

what are some of the systemic sx’s of arthritis

A

fever- often high, regular (daily/twice daily)
rashes- salmon pink, macular rash
HA, HSM, lyphadenopathy
pleural & pericardial effusions

71
Q

arthritis in systemic onset JRA may affect what joints?

A

may be of any joint
wrists, knees, ankles most typical
may occur in hands, hips, cervical spine & temporomandibular joints

72
Q

lab findings in systemic onset JRA

A 
leukocytosis
thrombocytosis
anemia
elevated ESR
AST/ALT elevation
hypoalbuminemia
elevated globulin level
73
Q

complications of systemic onset JRA

A

macrophage activation syndrome- uncontrolled proliferation of lymphocytes & macrophages
prone to sequelae of chronic systemic inflammation- growth retardation, osteoporosis

74
Q

what is dermatomyositis?

A

inflammatory dz of muscle & skin

75
Q

what are the clinical manifestations of dermatomyositis

A 
weakness in proximal m. distribution
tenderness of muscles
absent DTRs
flexion contractures & m. deformities
rash
76
Q

describe the rash found in dermatomyositis

A

heliotrope rash of upper eyelids (red-purple color), sun sensitive
Gottron’s papules

77
Q

what are Gottron’s papules

A

papules found on the extensor surfaces of knuckles, elbow, knees
start as erythema, progressing to scales

78
Q

lab findings in dermatomyositis

A

elevated muscle-source enzymes
-CK
-aldolase
muscle bx

79
Q

tools for diagnosing dermatomyositis

A

labs
electromyography
MRI

80
Q

tx of dermatomyositis

A

PT

meds- steroid anti-inflammatories, methotrexate, refractory cases: IVIG, cyclosporine

81
Q

what can you use meds wise in tx of refractory dermatomyositis?

A

IVIG

cyclosporine

82
Q

what meds can you use to tx dermatomyositis?

A

steroid anti-inflammatories

methotrexate

Peds-Final (10 decks)

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