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Pediatrics- Noelle > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

What are 3 long term complications of JIA

A 
uveitis
flexion contracture
leg length discrepancy of involved leg
joint erosions
muscle wasting
growth disturbances
delay of motor development
malalignment of joints
TMJ disease
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2
Q

what are the criteria for Kawasaki disease

A

fever >5 days and 4/5 of:
C- conjunctivitis (bilateral, non exudative)
R- rash (polymorphous)
A- adenopathy (>1.5cm)
S- skin changes (fissured lips, strawberry tongue)
H- hands and feet (edema, erythema) and perineal (erythema/peeling)
typical age 3 mon- 5 years

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3
Q

what do we worry about for familial Mediterranean fever? what is the treatment?

A

amyloidosis, especially renal amyloidosis

tx: colchicine

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4
Q

what rash is seen with dermatomyositis? what type of weakness? what nail changes? nodules?

A

heliotrope rash over the eyelids (purple)
gottron’s papules (dorsal surface of the knuckles)
symmetric proximal weakness
can get nailfold changes- drop out, dilatation, tuortuosity
calcinosis- calcium nodules

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5
Q

what 3 investigations would you do for dermatomyositis? Tx?

A

EMG- denervation and myopathy
muscle biopsy- necrosis and inflammation
lab work ( elevated CK, AST, LDH, aldolase)
MRI-can help identify muscle inflammation
Tx: corticosteroids

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6
Q

what are 2 renal complications associated with lupus?

A 
HTN
Proteinuria
edema
renal failure
hematuria
nephrotic syndrome
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7
Q

what are the criteria for rheumatic fever?

A 
2 major or 1 major and 2 minor with evidence of GAS infection (positive throat culture or elevated or rising ASOT)
J- joints (migratory arthritis)
O- carditis
N- nodules (subcutaneous)
E- erythema marginatum
S- syndenham's chorea

Minor: arthralgia, fever, ESR, CRP, prolonged PR

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8
Q

what are the 7 types of JIA

A

Age <16 and persists for > 6 weeks

  1. oligo
  2. polyarticular RF +
  3. polyarticular RF -
  4. systemic JIA
  5. enthesitis related arthritis
  6. psoriatic arthritis
  7. undifferentiated arthritis
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9
Q

what is the definition of arthritis

A

joint swelling/ effusion
OR
limited ROM with joint line tenderness or painful ROM

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10
Q

what is the most common type of JIA

-most frequent joints involved? (4)

A

oligoarthritis

  • most frequent joints involved:
    1. Knees
    2. Ankles
    3. Wrists
    4. Elbows
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11
Q

what are the two types of oligoarthritis

A

persistent oligoarthritis- <4 joints

extended oligoarthritis- >4 joints after first 6 months

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12
Q

what increases risk of uveitis with oligoarticular JIA (4) and what is the treatment for uveitis?

A 
ANA positive
young onset age
girls
oligoarticular JIA
tx: topical steroids
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13
Q

What causes neonatal lupus? why do we worry about neonatal lupus?

A

maternal autoantibodies anti- ro and la

worry about 3rd degree heart block

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14
Q

what is the treatment for Kawasaki disease?

A

acute phase:
IVIG x 2 (if poor response to first dose)
steroids for persistent symptoms unresponsive to IVIG x2, symptomatic myocarditis
regular dose ASA

subacute phase:
low dose ASA
echo surveillance in acute phase and at 6 weeks

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15
Q

uveitis in JIA is most common with what type?

A

oligoarthritis
associated with positive ANA
usually ASYMPTOMATIC

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16
Q

what type of JIA is associated with symptomatic uveitis

A

enthesitis related arthritis

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17
Q

what are 4 complications of uveitis

A

synechiae- irregular pupil
glaucoma
cataract
visual loss

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18
Q

what can be seen on x ray with JIA

A 
acceleration of growth
acceleration of maturation
growth inhibition
osteoporosis
erosions
loss of cartilage
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19
Q

what can be seen on MRI with JIA

A

bone marrow edema
erosions
synovitis
cartilage

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20
Q

what is required for systemic JIA

A 
2 weeks of fever
arthritis and at least one of:
- rash (evanescent, salmon coloured)
- generalized lymphadenopathy
- hepatosplenomegaly
- serositis
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21
Q

what lab findings are associated with MAS? on bone marrow?

A

massive release of pro inflammatory cytokines
accumulation of macrophages in liver, spleen, LN and CNS

elevated ferritin
elevated lipids
elevated CRP
decreased ESR
elevated d-dimer
decreased fibrinogen

on bone marrow- hemophagocytosis

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22
Q

what is the treatment for oligo JIA? poly JIA? systemic JIA

A

oligo-NSAIDS, joint injection
poly-NSAIDS then MTX and/or SSZ, Biologic
systemic- NSAIDS then corticosteroids and/or MTX then biologic (often tx now with anti IL-1 or anti IL-6 first)

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23
Q

can live vaccines be given with MTX and biologics??

A

NO!

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24
Q

what antibiotic cannot be given with MTX except in prolyphactic dosing?

A

sulpha antibiotics

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25
Q

what is the treatment for rheumatic fever?

A

penicillin x 10 days
ASA/NSAIDS for arthritis
ASA/steroids for carditis

without carditis: prophylactic penicillin for at least 5 years or until 21 years
with carditis: up to 21 yo or 10 years post attack
residual heart disease: up to age 40 or 10 years post initial attack (essentially life long)

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26
Q

what is the most common vital organ involvement in SLE?

A

kidney- glomerulonephritis

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27
Q

what are the indications for testing for APS

A

thrombosis
SLE yearly
unexplained PTT prolong
clinical features of APS

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28
Q

what is the treatment for HSP?

A

supportive
NSAIDs
systemic steroids- considered if severe abdominal pain, severe renal disease

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29
Q

what are the criteria for HSP

A

palpable purpura with lower limb predominance and without thrombocytopenia or coagulopathy and at least 1 of the following

●Abdominal pain (usually diffuse, with acute onset)- can see intusseception
●Arthritis or arthralgia (acute onset)
●Renal involvement (proteinuria, hematuria)
●Leukocytoclastic vasculitis or proliferative glomerulonephritis, with predominant IgA deposition

recurrences common in approx 30% in the first 1-2 months

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30
Q

what investigation is typically done before treating someone with systemic JIA

A

bone marrow aspirate

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31
Q

what are the characteristics of growing pains

A 
age 3-10 years
bilateral leg pain
occurs at night
no interference with activities
normal physical exam
normal lab tests (if done)
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32
Q

what is the common cause of hip pain age 2-6

A

transient synovitis

- may follow viral infection

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33
Q

what is the common cause of hip pain age 4-10

A

legg calves perthes

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34
Q

what is the common cause of hip pain age 10-14

A

slipped capital femoral epiphysis
= SURGICAL EMERGENCY
No weight bearing until pinned

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35
Q

what is complex regional pain syndrome? treatment?

A

girls» boys
may follow trauma
very painful, tender extremity
autonomic changes (cool, sweaty, swollen, discoloration)
two types: no nerve lesion versus definable nerve lesion
treatment: physio, psychotherapy, pharmacotherapy

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36
Q

peak age for oligo? common joints?

A

peak age 1-3
girls>boys
affects large joints (knee most common- also ankles, wrists, elbows)

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37
Q

How often do we screen for uveitis for oligo

A

age of onset of arthritis <6 and ANA +: every 3 months initially then every 6 months
age of onset >6 and ANA +: every 6 months

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38
Q

what joints are affected with RF-neg polyarticular JIA

A

small and large joints

TMJs

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39
Q

what are the characteristics of RF positive polyarticular JIA

A

teenage girls
symmetrical small and large joint arthritis
rheumatoid nodules over pressure points in 30%
ANA may be positive
worse prognosis with erosive disease

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40
Q

what are the characteristics of enthesitis related arthritis

A 
arthritis and enthesitis
older boys >> girls
lower limb arthritis
sacroilitis
family history
HLA-B27 positive
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41
Q

enthesitis related arthritis may be associated with what 3 things

A

IBD
psoriasis
symptomatic uveitis

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42
Q

dactylitis is associated with what type of arthritis?

A

psoriatic arthritis

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43
Q

what are the characteristics of systemic JIA

A

fever (x 2 weeks for diagnosis)- must be 3d in a row over the 2 weeks
rash
arthritis- may be delayed*
hepatosplenomegaly
lymphadenopathy
serositis
lab work: anemia, high ESR, CRP, platelets

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44
Q

what type of fever is seen with systemic JIA

A

quotidian, daily, predictable (every night or morning and night)

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45
Q

what type of rash is seen with systemic JIA

A

evanescent- salmon colored rash

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46
Q

what are the clinical features of MAS

A 
persistent fever
hepatosplenomegaly
lymphadenopathy
hermorrhagic skin rash
CNS dysfunction
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47
Q

what lab findings are associated with MAS

A 
cytopenias
hypofibrinogenemia
transaminitis
coagulopathy
hypertriglyceridemia
marked increase ferritin
increase CRP
decreased ESR
low NK cell function
high CD 25
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48
Q

what 3 rheumatic conditions are associated with MAS

A

SLE
systemic JIA
kawasaki disease

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49
Q

what are the treatment options for MAS

A 
high dose corticosteroids
IVIG
cyclosporine
etoposide
ATG
IL-1 inhibition
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50
Q

what side effects are associated with MTX

A

nausea
oral ulcers (typically do not get because they are also on folic acid)
hepatotoxicity
leukopenia
infection
pulmonary toxicity
adolescents- try to avoid EtOH entirely, contraception required if sexually active

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51
Q

what must you rule out before starting anti-TNF agents?

A

TB!

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52
Q

what antibodies are highly specific for SLE

A

anti-ds DNA

anti-sm

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53
Q

what is highly sensitive for lupus?

A

ANA- sensitive

>95% of patients with lupus are ANA +

54
Q

antiphospholipid antibody syndrome is associated with what? causes what 2 things?

A

associated with lupus

causes arterial and venous thrombosis

55
Q

what are the 2 types of lupus nephritis

A

mesagnial lupus nephritis
focal lupus nephritis
diffuse lupus nephritis

56
Q

what do we monitor on labs for lupus

A

complement
ANA, anti-DNA antibodies
ESR- may reflect overall disease activity
CBC: anemia, thrombocytopenia and lymphopenia
* high CRP often suggests infection, serositis or MAS

57
Q

what is the treatment for lupus

A

mild disease:
NSAIDS
plaquenil
low dose steroids

severe disease:
high dose steroids, immunosuppressive therapy (cyclophosphamide, rituximab etc)

58
Q

what drugs can trigger drug-induced lupus?

A

anticonvulsants, minocycline, hydralazine, anti-TNF

tx: stop the drug

59
Q

what are the 3 D’s associated with dermatomyositis affecting smooth muscle

A

dysphagia- difficulty swallowing
dysphonia- nasal speech
dyspnea- respiratory failure with severe weakness

60
Q

what is the treatment of dermatomyositis

A

steroids

  • pulse steroids
  • MTX
  • IVIG for more severe weakness
61
Q

what should be monitored for HSP for 6 months min?

A

BP

urinalysis

62
Q

what lab features are associated with kawasaki disease

A 
neutrophilic leukocytosis
anemia
thrombocytosis (day 7+), occ thrombocytopenia
elevated ESR, CRP
elevated transaminases
hypoalbuminemia*
sterile pyuria
63
Q

what are the two main risk factors associated with developing coronary aneurysms from kawasaki disease

A

age <1*and >9

prolonged fever*

64
Q

what are other manifestations of kawasaki disease

A 
aseptic meningitis
anterior uveitis
oligo-or polyarthritis
hydrops of the gallbladder
meatitis and dysuria
diarrhea in approx 25%
peeling in the perineal area
65
Q

what is the main cardiac complication associated with kawasaki disease?

A 
coronary artery dilation
coronary artery aneurysms
also associated with:
myocardial infarction
myocarditis
pericarditis
valvular dysfunction
arrhythmias
66
Q

what does PFAPA stand for? what is the typical history?

A

periodic fever, apthous stomatitis, pharyngitis, adenitis
onset <5years (self limiting within 5 years)
fever every 3-6 weeks, regular recurrence
fever typically lasts 5 days
associated with apthous stomatitis and or pharyngitis (strep swab -ve)
completely well between episodes
no genetic or ethnic associations!!
tx: single dose prednisone

67
Q

what are the clinical features of FMF

A

*fever + serositis!
most common hereditary autoinflammatory disease
AR

fever episodes present age  <20
short duration of fever <72h, irregular
associated with serositis (peritonitis>pleuritis? pericarditis)
erysipeloid erythema
arthralgia>arthritis
mylagia
orchitis (5%)
lack of eye involvement
68
Q

what are the cardinal features of a joint infection

A

redness
fever
swelling
pain and tenderness

69
Q

what is the joint position for joint infection

A

abduction

external rotation

70
Q

what are the most common causes of septic joint

A

staph and strep

also get gram neg bacteria- meningococcus, kingella

71
Q

what are the kocher criteria

A

unable to weight bear
fever >38.5
wbc greater than 12000
elevated inflammatory markers (ESR >40, CRP >20)
helps differentiate transient synovitis from septic arhtritis

72
Q

what rash is seen with lyme disease?

A

erythema migrans

73
Q

what two infections can mimic monoarthrtitis

A 
TB
Lyme disease (borrelia burgdorferi)- tx: amoxicillin or doxycycline if >10 yrs or ceftriaxone for disseminated
74
Q

boy with slapped cheek rash and arthritis

A

parvovirus arthritis

75
Q

Ddx erythema nodosum (6)

A 
Group A strep
TB
Sarcoidosis
IBD
OCP
SLE
76
Q

what type of anemia is seen with lupus?

A

autoimmune hemolytic anemia

77
Q

anti ro/la is associated with what 3 conditions

A

neonatal lupus
sjogren
SLE

78
Q

c ANCA

A

wegener granulomatosis

79
Q

p ANCA

A 
microscopic polyangitis
UC
PSC
SLE
Churg-Strauss syndrome  (EGPA)
80
Q

Associations with HLAB27 (5)

A 
ankylosing spondylitis
JIA enthesitis related, psoriatic
IBD
reactive arthritis
isolated acute anterior uveitis
81
Q

general definition of JIA

A

arthritis of unknown ethology that begins before 16th birthday and lasts more than 6 weeks

82
Q

definition of oligoarthrtitis

A

arthritis affecting 1-4 joints during the first 6 months

83
Q

what is the major complication of systemic JIA

A

Macrophage activation syndrome

84
Q

what is the criteria for enthesitis related arthritis

A

Arthritis and enthesitis
Or, arthritis or enthesitis with at least 2 of the following:
- Presence or history of sacroiliac joint tenderness and/or inflammatory back pain
- Presence of HLA B27 antigen
- Onset of arthritis in a male over 6 years of age
- Acute (symptomatic) anterior uveitis
- History of ankylosing spondylitis, enthesitis related arthritis, sacroiliitis with inflammatory bowel disease, or acute anterior uveitis in a first-degree relative

85
Q

what is the hallmark of psoriatic arthritis

A

dactylitis “sausage digit”

86
Q

what is the criteria for psoriatic arthritis

A 
Arthritis and psoriasis
Or, arthritis and at least 2 of the following:
- Dactylitis
- Nail-pitting or onycholysis
- Psoriasis in a first-degree relative
87
Q

what are characteristics of Raynaud phenomenon? triggers?

tx?

A

vascular spasm leading to triphasic color sequence (white, blue, red)
well demarcated areas of color change
usually fingers and toes
triggers= cold, stress, caffeine, smoking
Treatment:
-avoid precipitants
-may need nifedipine (peripheral vasodilator)

88
Q

what organisms cause reactive arthritis

A 
salmonella
shigella
yersinia
campylobacter
chlamydia
ureaplasma
89
Q

Criteria for Lupus

  • what do you see in complement level in SLE?
  • what lab markers are used to monitor disease activity (2)?
A

SOAP BRAIN MD
4/11 criteria
Serositis – Pleurisy, pericarditis
Oral ulcers- usually painless
Arthritis- non erosive, 2 or more joints
Photosensitivity
Blood disorders (hemolytic anemia, leukopenia, thrombocytopenia)
Renal involvement- proteinuria, cellular casts
Antinuclear antibodies
Immunologic phenomena (dsDNA or anti-sm; anticardiolipin, lupus anticoagulant, false positive syphilis >6mo)
Neurologic disorder- seizures OR psychosis

Malar rash
Discoid rash

  • **Complement is LOW in SLE
  • C3 and C4 are used to monitor disease activity = levels fall during a flare and return to normal after appropriate treatment
90
Q

criteria for APS

A

Vascular thrombosis: ≥1 clinical episode of arterial, venous, or small vessel thrombosis in any tissue or organ confirmed objectively by validated criteria

91
Q

What is the differential diagnosis for monoarthritis?

A 
Trauma/Mechnical/Orthopedic 
Infection (Acute-Septic/OM, Chronic- TB/Lyme)
Inflammatory (JIA, FMF) 
Tumor 
Hemarthrosis
92
Q

What is the differential diagnosis for polyarthritis

A

Inflammatory (JIA/SLE/Vasculitis)
Infection
Malignancy
Mechnical Pain syndrome

93
Q

What is rheumatoid factor?

A

IgM autoantibody reacts to Fc portion of IgG antibodies

94
Q

Top common causes of septic arthritis? (3)
In neonates?
In sickle cell?
In teenagers?

A
  1. Staph aureus
  2. Strep pneumonia
  3. Non Group A strep
  4. Kingella Kingae

In neonates: GBS and gram negatives

Sickle cell: Salmonella

Teenagers: gonorrhea

95
Q

Antibiotic choice for osteomyelitis/septic arthritis:

  • neonate
  • 1-3 month old
  • child
  • teenager
  • sickle cell
A
  • Neonate: Cloxacillin and Gentamicin
  • 1-3 month old: Cefuroxime (because of more gram negatives)
  • Child: Cefazolin
  • Teenager: ceftriaxone OR cefixime + azithromycin
  • Sickle cell: cefotaxime (salmonella coverage)
96
Q

3 circumstances where diagnosis of ARF can be made without Jones criteria:

A
  1. Chorea (may occur as the only manifestation)
  2. Indolent carditis
  3. Recurrent episodes
97
Q

What is the timing of development of acute rheumatic fever in relation to when the GAS infection occurred?

A

2-4 wks after GAS pharyngitis

  • 1/3 of patients with ARF have no history of antecedent pharyngitis
  • only 10-20% of throat culture or rapid strep antigen tests will be positive at the time of presentation of ARF; this is why ASOT is needed!
98
Q

What is the best confirmatory test for juvenile dermatomyositis?
-other tests?

A

EMG = denervation & myopathy

  • other tests:
    1. Muscle biopsy = perifascicular atrophy
    2. CK can be normal or high
    3. ANA: 80% can be positive
99
Q

What are 3 ways that maternal SLE can affect neonate?

A
  1. Rash
  2. Congenital heart block
  3. Thrombocytopenia
100
Q

What are physical exam findings of ankylosing spondylitis? (4)

A
  1. Loss of normal lumbar lordosis
  2. Positive Schober test (decreased forward flexion)
  3. Anterior uveitis
  4. Enthesitis (ie. patellar tendon and achilles tendon)
101
Q

What is the pathophysiology behind acute phase reactants?

  • which lab markers increase in acute phase response? (11)
  • which lab markers decrease in acute phase response? (3)
A

Acute phase reactants: plasma proteins produced by the liver that change production during acute phase of inflammation (mediated by cytokines)

  • increased lab markers:
    1. CRP
    2. ESR
    3. Complement proteins
    4. Fibrinogen
    5. Ferritin
    6. Ceruloplasmin
    7. Haptoglobin
    8. G-CSF
    9. IL-1 receptor antagonist
    10. Serum amyloid
    11. Coag factors
  • decreased lab markers:
    1. Albumin
    2. Transferrin
    3. IGF-1
102
Q

A patient with JIA has a positive ANA. What 3 things does this put them at risk for?

A
  1. Earlier disease onset
  2. Asymmetric arthritis
  3. Uveitis
103
Q

What level is considered a positive ANA?

-when should specific antibodies (ie. anti-dsDNA) be ordered?

A

> 1:160

-specific antibodies should only be ordered if ANA is positive AND a disease other than JIA is suspected (ie. SLE)

104
Q

What 4 antibodies are SPECIFIC for SLE?

-what antibody is SENSITIVE for SLE?

A
  • most sensitive antibody: ANA (if negative, very unlikely to have SLE
  • most specific:
    1. anti-dsDNA
    2. anti-Ro
    3. anti-La
    4. anti-smith
105
Q

What specific auto-antibodies (3) can often be seen with juvenile dermatomyositis?

A
  1. Anti-Jo1
  2. Anti-SRP
  3. Anti-Mi-2
106
Q

What specific group of auto-antibodies are associated with increased risk of thrombosis?
-two examples of these?

A

Antiphospholipid antibodies

  • this is why people with antiphospholipid antibody syndrome are at increased risk of clots and pregnancy loss secondary to clots
  • ex. lupus anticoagulant, anticardiolipin
107
Q

A patient presents to you with dactylitis. What is your differential diagnosis? (3)

A
  1. Sickle cell anemia
  2. JIA (ie. enthesitis related, psoriatic arthritis)
  3. Trauma
108
Q

What is the diagnostic criteria for RF-negative-polyarthritis?
-what about RF-positive polyarthritis?

A

RF-negative polyarthritis:

  1. Arthritis affecting 5 or more joints during first 6 months of disease
  2. RF testing negative

RF-positive polyarthritis:

  1. Arthritis affecting 5 or more joints during first 6 months of disease
  2. 2 or more positive tests for RF at least 3 months apart during first 6 months of disease
109
Q

Which population is usually affected by RF positive polyarthritis?

A

Adolescent girls = this is essentially adult-type rheumatoid arthritis presenting in a child or adolescent
-usually involve the PIP and MCP joints

110
Q

What is the triad of Felty’s syndrome?

A
  1. Rheumatoid arthritis
  2. Neutropenia
  3. Splenomegaly
111
Q

What are possible long term complications of SLE?

A
  1. Death from infection, renal, CNS, cardiac or pulmonary disease
  2. Early onset coronary artery disease from vasculitis
  3. Bone disease –> osteopenia, avascular necrosis from steroid use
  4. Malignancy from use of immunosuppressants/biologics
112
Q

What are the clinical features of NLE?

A
  1. Cardiac: complete congenital heart block (associated with non-immune hydrops)
  2. Annular, erythematous papulosquamous rash with fine scale and central clearing
    - usually photosensitive
    - can be present at birth or develop within first few weeks of life
    - predilection for face and scalp
    - self-resolving
  3. Hematologic: thrombocytopenia (most common), neutropenia and anemia less common
  4. Hepatic involvement: cholestatic hepatitis with elevated liver enzymes, hepatomegaly
  5. Neurologic: macrocephaly, hydrocephalus, spastic paraparesis, vasculopathy
113
Q

What is the treatment for NLE

A

If fetal bradycardia is found during pregnancy, need fetal ECHO and may require treatment with Dex +/- sympathomimetics
-neonates with complete heart block may need pacemaker

114
Q

What are types of:

  • large vessel vasculitis (2)
  • medium vessel vasculitis (2)
  • small vessel vasculitis (4)
A

Large vessel vasculitis:

  1. giant cell arteritis (older adults)
  2. takayasu arteritis

Medium vessel vasculitis:

  1. Kawasaki
  2. Polyarteritis nodosa

Small vessel vasculitis:

  1. Wegener’s granulomatosis
  2. Churg-Strauss
  3. Microscopic polyangitis
  4. HSP
115
Q

What are the clinical features of takayasu arteritis?

  • diagnostic criteria?
  • treatment?
A

Large vessel vasculitis involving the aorta and its branches (thoracic, abdominal, carotid)
-initially presents as non-specific inflammatory illness with fever, then evolves into chronic, fibrotic phase with signs and symptoms of chronic vascular insufficiency (pulse deficit, claudication, BP discrepancy, bruits)
***Diagnostic criteria:
Needs:
1. Angiographic abnormalities (MRI) of aorta or its main branches (aneurysm/diltation, narrowing or thickened arterial wall)
AND
1 of the following:
1. Pulse deficit or claudication
2. Discrepancy of 4 limb BP > 10 mm Hg
3. Bruits
4. Hypertension
5. Acute phase reactants elevated (ESR or CRP)
-treatment: corticosteroids plus second line agent: cyclophosphamide, methotrexate, etc.

116
Q

What is the diagnostic criteria for:

  • incomplete KD
  • atypical KD
  • alternate way to make diagnosis for KD aside from the 5 or more days of fever + 4/5 features
A

Incomplete KD: if 5 or more days of fever with 2-3 features instead of 4
-commonly seen in infants who are at higher risk of coronary artery involvement

Atypical KD: if KD with unusual manifestation (eg. renal failure)

Alternate way to diagnose KD: in presence of fever AND coronary artery involvement on echo

117
Q

What is the usual age group for KD?

-what is the pathophysiology?

A

3 mo-5 yo

-small vessel vasculitis with predilection for coronary arteries; thought to be triggered by infectious agent

118
Q

What is the diagnostic criteria for Wegener Granulomatosis?

A

***Overall, think small vessel vasculitis primarily affecting upper airway, lungs, and kidneys!!!!
Need 3/6:
1. Histopathology showing granulomatous inflammation within wall of artery
2. Upper airway involvement: chronic purulent or bloody nasal discharge, recurrent epistaxis, nasal septum perforation, saddle nose deformity, chronic sinus inflammation
3. Laryngo-tracheo-bronchial involvement: subglottic, tracheal or bronchial stenosis
4. Pulmonary involvement: nodules, cavities or pulmonary infiltrates (SOB, chronic cough, hemoptysis/alveolar hemorrhage)
5. ANCA positive
6. Renal involvement: proteinuria, elevated Cr, hematuria

119
Q

What is the treatment for Wegener granulomatosis? (2)

A

Initial therapy: Corticosteroids and cyclophosphamide

Maintenance therapy: methotrexate, azathioprin, mycophenolate and low dose corticosteroids

120
Q

What is the most common vasculitis in children?

A

HSP

121
Q

What does the prognosis of HSP depend on?

A

Depends on severity of nephritis (worse prognosis with nephrotic syndrome)
-end stage renal disease occurs in 1-3% of patients

122
Q

What is CREST syndrome?

-most important complication?

A

Aka Limited systemic sclerosis

  1. Calcinosis
  2. Raynaud phenomenon
  3. Esophageal dysmotility
  4. Sclerodactyly
  5. Telangiectasias
    - most important complication: future development of pulmonary hypertension
123
Q

A patient presents to you with isolated Raynaud phenomenon. What are the 2 best predictive factors for future development of autoimmune diseases?

A
  1. Positive ANA

2. Abnormal nail fold vasculature

124
Q

What is the most common form of localized scleroderma in the pediatric population?

A

Linear scleroderma: characterized by one or more linear streaks (following dermatomal distribution) extending over upper or lower extremity

  • can involve face or scalp (en coup de sabre), usually forehead
  • Parry-Romberg syndrome: progressive hemi-facial atrophy, disfiguring
125
Q

What is the minimum duration of fever to be called fever of unknown origin?

A

Fever needs to last > 14 days with standard investigations not resulting in a clear diagnosis

126
Q

What are the clinical features of TRAPS?

-treatment?

A

TNF-receptor associated periodic syndrome

  1. Long duration of fevers lasting 3-4 weeks and occur at irregular intervals
  2. Migrating erythematous, maculopapular rash
  3. Severe migratory myalgias with rash, arthralgias
  4. Conjunctivitis, periorbital edema
  5. Severe abdo pain
    - treatment: unproven therapy; steroids provide symptomatic relief but do not change frequency
127
Q

What are examples of DMARDs? (7)

A

DMARDs = disease-modifying anti-rheumatic drug

  1. Azathioprine
  2. Cyclophosphamide
  3. Cyclosporine
  4. Hydroxychloroquine
  5. Methotrexate
  6. Mycophenolate mofetil
  7. Sulfasalazine
128
Q

What monitoring needs to occur when patients are on hydroxychloroquine (plaquenil)?

A

Eye exams q6mo to assess for retinal deposits (retinal toxicity)

129
Q

Name 2 classes of medications that can trigger drug-induced lupus.

A

antibiotics (ex: tetracyclines)

anticonvulsants

130
Q

Name 1 non-medication cause that can exacerbate lupus (2)

A
  1. sun exposure

2. pregnancy

Pediatrics- Noelle (28 decks)

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