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Peds-Final > Rheumatology > Flashcards

Rheumatology Flashcards

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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
SLE is a multisystem inflammatory dz & may include what?
``` joints serous linings skin kidneys CNS ```
26
epidemiology of SLE
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
27
MC findings in SLE
``` fever abdominal complaints malar rash &/or oral ulcers MS arthritis or arthralgias hematologic anemia, leukopenia, thrombocytopenia ```
28
crash & burn pneumonic for KD
``` Conjunctivitis Rash Adenopathy Strawberry tongue Hands & feet ``` burn (fever)
29
renal findings in CH w/ SLE
hematuria proteinuria mild hypertension diffuse proliferative glomerulonephritis
30
neurologic findings in CH w/ SLE
``` HA deterioration of academic performance withdrawal social isolation depression *full hx & PE important in teens, screening CBC, ESR reasonable in vague complaints ```
31
more dramatic neurologic presentations in SLE
stroke seizures chorea coma
32
hematologic findings in CH w/ SLE
thrombocytopenia, hemolytic anemia, leukopenia (together will lead to sending an ANA) f/u studies when suspecting SLE: antiphospholipid Ab's, lupus anticoagulant activity
33
cardiopulmonary findings in CH w/ SLE
increased risk of atherosclerosis & CAD increased risk of pericarditis, endocarditis pleural effusions
34
diagnostic criteria of SLE
``` 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
malar rash in SLE
fixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds
36
discoid rash in SLE
erythematous, raised patches w/ adherent keratotic scaling & follicular plugging
37
photosensitivity in SLE
skin rash as a result of unusual rxn to sunlight
38
oral ulcers in SLE
usually painless
39
arthritis in SLE
nonerosive arthritis involving 2+ peripheral joints, characterized by tenderness, swelling, or effusion
40
serositis in SLE
pleuritis or pericarditis
41
renal d/o in SLE
persistent proteinuria, cellular casts
42
neurologic d/o in SLE
seizures or psychosis
43
hematologic d/o SLE
hemolytic anemia, leukopenia, lymphopenia, thrombocytopenia
44
immunologic d/o in SLE
anti-phospholipid Ab, anti-DNA, anti-Sm, false positive syphilis serology
45
anti-nuclear Ab in SLE
abnormal titer of ANA by immunofluorescence or an equivalent assay
46
labs for dx in SLE
ANA CBC w/ leukopenia, anemia complement level ESR
47
checking for dz severity in SLE
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
UA & renal function in SLE
BUN Cr renal dz is a major component of SLE
49
SLE therapy
corticosteroids cyclophosphamide-early therapy improves survival steroid-sparing agents non-steroidal anti-inflammatory drugs
50
steroid sparing agents for SLE
methotrexate azathiprine mycophenolate mofetil
51
non-steroidal anti-inflammatory drugs for SLE
hydroxychloroquine | dapsone
52
challenges in CH w/ SLE
poor compliance renal dz- 30% will go on to renal failure neurologic complications intercurrent infxns
53
what is the most prevalent chronic rheumatologic dz of CH
juvenile rheumatoid arthritis (JIA- juvenile idiopathic arthritis?)
54
what are the 3 types of JIA/JRA
pauciarticular (~50%) /= 5 joints | systemic onset
55
what is JIA/JRA
non-migratory arthropathy w/ a tendency to involve large joints or proximal interphalangeal joints lasting over chronic period of months
56
when is pain usually worse in JIA/JRA
morning | joint swelling w/ diminished mobility
57
JIA/JRA complicated by uveitis
MC treatable cause of blindness in CH | ANA-positive CH are more likely to have chronic uveitis
58
pauciarticular onset JIA/JRA is more common in girls or boys?
girls
59
what are the clinical manifestations of pauciarticular onset JRA
- 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
complications of pauciarticular onset JRA
uveitis untreated may progress toward blindness | inflammation may cause bony changes that distort growth
61
what mild systemic complaints may exist w/ JRA
low grade fever anemia malaise
62
JRA can result in________growth
asymmetric
63
polyarticular onset JRA is more common in girls or boys?
girls | *> 50% have complete remission
64
manifestations of polyarticular onset JRA between ages 2-5 yo
- progressive involvement of joints - symmetrical joint involvement - uveitis - can present w/ systemic sx's
65
symmetrical joint involvement in polyarticular onset JRA
small joints of the hands & feet ankles, wrists, knees may cause fusion of the cervical spine
66
some systemic sx's that may present w/ polyarticular onset JRA
``` malaise fever growth retardation anemia elevated inflammatory markers ```
67
manifestations of polyarticular onset JRA ages 10-14 yo
arthritis of rapid onset small joints of hands & feet typically involved adolescents that are rheumatoid-factor + probably have true adult rheumatoid arthritis
68
systemic onset JRA affects boy or girls more?
affects both sexes equally
69
manifestations of systemic onset JRA
typically present originally without arthritis systemic sx's | dx often made after elimination of other dz's, as sx's become evidently chronic
70
what are some of the systemic sx's of arthritis
fever- often high, regular (daily/twice daily) rashes- salmon pink, macular rash HA, HSM, lyphadenopathy pleural & pericardial effusions
71
arthritis in systemic onset JRA may affect what joints?
may be of any joint wrists, knees, ankles most typical may occur in hands, hips, cervical spine & temporomandibular joints
72
lab findings in systemic onset JRA
``` leukocytosis thrombocytosis anemia elevated ESR AST/ALT elevation hypoalbuminemia elevated globulin level ```
73
complications of systemic onset JRA
macrophage activation syndrome- uncontrolled proliferation of lymphocytes & macrophages prone to sequelae of chronic systemic inflammation- growth retardation, osteoporosis
74
what is dermatomyositis?
inflammatory dz of muscle & skin
75
what are the clinical manifestations of dermatomyositis
``` weakness in proximal m. distribution tenderness of muscles absent DTRs flexion contractures & m. deformities rash ```
76
describe the rash found in dermatomyositis
heliotrope rash of upper eyelids (red-purple color), sun sensitive Gottron's papules
77
what are Gottron's papules
papules found on the extensor surfaces of knuckles, elbow, knees start as erythema, progressing to scales
78
lab findings in dermatomyositis
elevated muscle-source enzymes -CK -aldolase muscle bx
79
tools for diagnosing dermatomyositis
labs electromyography MRI
80
tx of dermatomyositis
PT | meds- steroid anti-inflammatories, methotrexate, refractory cases: IVIG, cyclosporine
81
what can you use meds wise in tx of refractory dermatomyositis?
IVIG | cyclosporine
82
what meds can you use to tx dermatomyositis?
steroid anti-inflammatories | methotrexate

Peds-Final (10 decks)

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