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PPS - From Nelson's > Rheuma > Flashcards

Rheuma Flashcards

1
Q

Differentials fever

A

Systemic JIA, SLE, vasculitis, acute rheumatic fever, sarcoidosis, MCTD

Malignancies, infections and post-infectious syndromes, inflammatory bowel disease, periodic fever (autoinflammatory) syndromes, Kawasaki disease, HSP

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2
Q

Differentials arthralgia

A

JIA, SLE, rheumatic fever, JDM, vasculitis, scleroderma, sarcoidosis

Hypothyroidism, trauma, endocarditis, other infections, pain syndromes, growing pains, malignancies, overuse syndromes

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3
Q

Differentials weakness

A

JDM, myositis secondary to SLE, MCTD, and deep localized scleroderma

Muscular dystrophies, metabolic and other myopathies, hypothyroidism

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4
Q

Differential chest pain

A

Juvenile rheumatoid arthritis, SLE (with associated pericarditis or costochondritis)

Costochondritis (isolated), rib fracture, viral pericarditis, panic attack, hyperventilation

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5
Q

Differentials back pain

A

Enthesitis related arthritis, juvenile ankylosing spondylitis

Vertebral compression fracture, diskitis, intraspinal tumor, spondylolysis, spondylolisthesis, bone marrow–occupying malignancy, pain syndromes, osteomyelitis, muscle spasm, injury

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6
Q

Differentials fatigue

A

SLE, JDM, MCTD, vasculitis, JIA

Pain syndromes, chronic infections, chronic fatigue syndrome, depression

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7
Q

Differentials malar rash

A

SLE, JDM

Sunburn, parvovirus B19 (fifth disease), Kawasaki disease

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8
Q

Differentials oral ulcer

A

SLE, Behçet disease

HSV infection, PFAPA syndrome

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9
Q

Differentials Purpuric rash

A

Vasculitis, e.g., ANCA-associated vasculitis, HSP

Meningococcemia, thrombocytopenia, clotting disorders

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10
Q

Gottron papules

Heliotrope rash, periungual telangiectasias

A

JDM

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11
Q

Differential Arthritis

A

Juvenile idiopathic arthritis, SLE, vasculitis, HSP, MCTD, scleroderma, acute rheumatic fever, reactive arthritis

Postviral arthritis, reactive arthritis, trauma, infection, Lyme disease, Kawasaki disease, malignancy, overuse syndromes

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12
Q

Differential Gottron papules non rheuma

A

Psoriasis, eczema

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13
Q

Moa nsiads

A

Inhibit Cox cyclooxygenase which is critical in production of prostaglandin

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14
Q

Nsaid use skin reaction

Small hypopigmented depressed scars in areas of minor skin trauma

A

Pseudoporphyria

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15
Q

Side effect of hydroxychloroquine

A

Retinal toxicity

Irreversible color blindness or loss of central vision

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16
Q

Serious side effect of rituximab

A

Multifocal leukoencephalopathy

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17
Q

Most common rheumatic problem in kids

A

Jia

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18
Q

Criteria JIA

A

Age at onset: <16 yr
Arthritis (swelling or effusion, or the presence of 2 or more of the following signs: limitation of range of motion, tenderness or pain on motion, increased heat) in ≥1 joint
Duration of disease: ≥6 wk
Onset type defined by type of articular involvement in the 1st 6 mo after onset:
Polyarthritis: ≥5 inflamed joints
Oligoarthritis: ≤4 inflamed joints
Systemic-onset disease: arthritis with rash and a characteristic quotidian fever
Exclusion of other forms of juvenile arthritis

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19
Q

arthritis fever hepatosplenomegaly lymphadenopathy serositis

A

systemic JIA

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20
Q

cutaneous hypersensitivity to trauma in jia

A

koebner phenomenon

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21
Q

rare but fatal complication of systemic jia

A

macrophage activation syndrome
or hemophagocytic syndrome
or hemophagocytic lymphohistiocytosis

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22
Q 
acute profound anemia
thrombocytopenia
leukopenia
high fever
lymphadenopathy
hepatosplenomegaly
prolonged pt and ptt
A

macrophage activation syndrome
or hemophagocytic syndrome
or hemophagocytic lymphohistiocytosis

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23
Q

treatment of macrophage activation syndrome
or hemophagocytic syndrome
or hemophagocytic lymphohistiocytosis

A

mppt
cyclosporine
anikinra

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24
Q

quality of arthritis in ARF

A

exquisite joint pain

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25
Q

hip pain due to idiopathic avascular necrosis of femoral head

A

Legg–Calvé–Perthes disease

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26
Q

ANA + in kids with JIA is associated with

A

chronic uveitis

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27
Q

complications of anterior uveitis in jia

A

posterior synechiae, cataracts, band keratopathy, blindness

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28
Q

predictors of severe disease in jia

A 
young age at onset
RF +
rheumatoid nodules
\+ anti CCP antibodies
large number of affected joints
wrist/hands affected
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29
Q

reactive arthritis occurs after

A

Salmonella sp., Shigella exneri, Yersinia enterocolitica, Campylobacter jejuni, Chlamydia trachomatis

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30
Q

arthritis
uveitis
urethritis

A

Reiter syndrome

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31
Q

rubella
hepa B
typically affect __

A

small joints

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32
Q

mumps
varicella
typically affect _

A

large joints

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33
Q

preceded by respiratory infection
severe hip pain
normal ESR, WBC in cbc
on utz noted effusion/widening of joint space

A

transient synovitis

or toxic synovitis

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34
Q

charcs of biopsy of sle lesion

A

hyperkeratosis
follicular plugging
infiltration of mononuclear cells into the dermal/epidermal junction

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35
Q

immunofluorescence of both affected and not affected skin reveal disposition of immune complexes w/in
dermal-epidermal junction

A

lupus band test

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36
Q

drugs associated with drug induced lupus

A

Minocycline, procainamide, hydralazine, isoniazid, penicillamine, diltiazem, interferon-α, methyldopa, chlorpromazine, etanercept, infliximab, adalimumab

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37
Q

how to diagnose sle

A

> or 4 (at least 1 clinical and 1 lab) OR biopsy proven lupus nephritis with positive ANA or anti DNA

clinical criteria:
acute/chronic lupus
oral ulcers
alopecia
arthritis
serositis
renal
neuro
hemolytic anemia
leukopenia
thrombocytopenia
immuno critera:
ANA
anti DNA
anti SM
antiphospholipid antibody
low complement (c3, c4, ch50)
direct coombs
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38
Q

t or f

ana levels correlate with disease severity

A

false

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39
Q

what lab marker correlates with disease severity

A

anti dsDNA levels

particularly anti Smith antibody

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40
Q

most common complications/death

A

infection
complication of glomerulonephritis
neuropsychiatric disorders

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41
Q

side effects of cyclophosphamide

A

hemorrhagic cystitis
premature gonadal failure
malignancy

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42
Q

conduction abnormalities in neonatal lupus detected at. ____ aog

A

16wks aog

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43
Q

which maternal antobodies are transferred to fetus

A 
anti Ro
anti La (SSB)
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44
Q

__% recurrence with congenital heart block in next baby (if prev baby has heart block)

A

15%

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45
Q

proximal muscle weakness

heliotrope rash

A

juvenile dermatomyositis

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46
Q

rash in JDM over neck and chest due to photosensitivity

A

shawl sign

47
Q

thickened erythematous scaly rash on palms
anti Jo1 antibodies
JDM

A

mechanic’s hands

48
Q

proximal muscle weakness symptoms

A 
difficulty climbing
combing hair
getting out of bed
postiive gowers sign
head lag
49
Q

associated with longstanding disease

JDM

A

lipodystrophy

calcinosis

50
Q

what deposited in calcinosis

JDM

A

calcium phosphate
hydroxyapatite
fluoroapatite

51
Q

findings EMG NCV in JDM

A

EMG myopathy and denervation with increased insertional activity, fibrillations, and sharp waves
NCV normal

52
Q

diagnosis of JDM

A

Classic rash: Heliotrope rash of the eyelids Gottron papules

Plus 3 of the following:
Weakness: Symmetric, Proximal
Muscle enzyme elevation (≥1): CK, Aspartate aminotransferase, Lactate dehydrogenase, Aldolase
Electromyographic changes: Short, small polyphasic motor unit potentials, Fibrillation, Positive sharp waves, Insertional irritability, Bizarre, high-frequency repetitive discharges
Muscle biopsy: Necrosis, inflammation

53
Q

what is given to reduce toxicity and side effects from folate inhibition in methotrexate use

A

folic acid

54
Q

most common visceral manifestation of scleroderma

A

pulmonary

55
Q

previously known as CREST syndrom

A

CREST - calcinosis cutis, raynaud, esophageal dysfxn, telangiectasia
now systemic sclerosis

56
Q

order of raynauds in cold/stress

A

blanching -> cyanosis -> erythema

57
Q

how to diagnose juvenile systemic sclerosis

major criterion

A 
MAJOR  CRITERION  (REQUIRED)*
Proximal skin sclerosis/induration of the skin proximal to metacarpophalangeal or metatarsophalangeal joints
58
Q

how to diagnose juvenile systemic sclerosis

minor criterion

A

AT LEAST 2 REQUIRED

Cutaneous: sclerodactyly
Peripheral vascular: Raynaud phenomenon, nailfold capillary abnormalities (telangiectasias), digital tip ulcers

Gastrointestinal: dysphagia, gastroesophageal reflux

Cardiac: Arrhythmias, heart failure

Renal: Renal crisis, new-onset arterial hypertension

Respiratory: pulmonary fibrosis (high-resolution computed
tomography/radiography), decreased diffusing capacity for carbon monoxide, pulmonary arterial hypertension

Neurologic: neuropathy, carpal tunnel syndrome

Musculoskeletal: tendon friction rubs, arthritis, myositis

Serologic: antinuclear antibodies—SSc-selective autoantibodies (anticentromere, antitopoisomerase I [Scl-70], anti brillarin, anti-PM/Scl, anti brillin or anti-RNA polymerase I or III

59
Q

history of recurrent painful shallow that don’t scar (if buccal, lips, tonge) or scar (genitals)
uveitis
erythema nodosum/pseudofolliculitis

A

behcet

60
Q

autosomal recessive
bried acute fever episodes
polyserositis
assoc with AA amyloidosis

A

familial mediterranean fever

61
Q

progressive shrinkage and degeneration of the tissues beneath the skin, usually on only one side of the face (hemifacial atrophy)
form of localized scleroderma

A

Parry–Romberg syndrome

62
Q 
autosomal recessive
severe mental retardation
ataxia
myopathy
cataracts
failure to thrive
decreased mevalonate kinase
recurrent fever lasting 3-7days
ab pain
diarrhea nausea vomiting
arthalgia
ulcer
elevated igD
A

dutch fever

or hyperimmunoglobulinemia D syndrome

63
Q 
autosomal dominant
periodic fever 4-6 days
severe ab pain nausea vomiting
arthritis
myalgia
rash conjunctivitis
unilateral periorbital edema
TNF receptor
AA amyloidosis develops in 25%
A

TRAPS

tumor necrosis factor receptor associated periodic syndrome

64
Q 
autosomal dominant
CIAS1 gene -> cryopyrin
periodic fever
skin rash
on biopsy noted perivascular infiltrates of PMN leukocytes
polyarthralgia
triggered by cold exposure
A

familial cold autoinflammatory syndrome

65
Q 
periodic fever
aphthous stomatitis
pharyngititis
adenitis
2-5 yrs old
ab pain
arthralgia
A

marshall syndrome

66
Q

autosomal dominant

granulomatous deposition in skin, eye,joint

A

blau syndrome

67
Q

cardinal feature of sarcoidosis

A

noncaseating epitheloid granulomatous lesions

68
Q

what are located in center of granulomatous lesions in sarcoidosis

A

macrophage
epitheliod
multinucleated giant cell
cd4 t lymphocye

69
Q

associated lab finding in sarcoidosis

A

hypercalcemia

vit D excess 1,25 OH VitD

70
Q

other name for kawasaki

A

mucocutaneous lymph node syndrome

infantile polyarteritis nodosa

71
Q

leading cause of acquired heart disease in developed countries

A

kawasaki

72
Q

kawasaki affect __ sized arteries

A

medium

73
Q

histopath exam of kawasaki

A

edema of endothelial and smooth muscle cells with intense inflammatory infiltration of the vascular wall
initially by pmn then by macrophages, lymphocytes and plasma cells

74
Q

how to diagnose kawasaki

A 
FEVER PLUS 4:
bilateral non exudative conjunctival injection with limbal sparing
strawberry tongue (erythema of mucosa)
dry cracked lips
edema and erythema of hands and feet
rash
nonsuppurative LAD unilateral >1.5cm
75
Q

giant coronary aneurysm __mm internal diameter

A

8mm

greatest risk of rupture

76
Q

3 clinical phases of kawasaki

A

acute febrile. 1-2 weeks
subacute 2 wk
convalescent 6-8

77
Q

which phase of kawaski has highest risk of death due to development of aneurysms

A

subacute

78
Q

classification of aneurysm in kawasaki

A

small <5mm
medium 508mm
giant >8mm

79
Q

when is 2d echo done in kawasaki

A

at diagnosis
again after 2-3 weeks of illness
1 yr later (if normal 2decho)
then f/u every 5 yr

80
Q

MMR vaccine must be deferred __ mo after IVIG

A

11 months

81
Q

most common vasculitis in kids

A

HSP

82
Q

characteristic of HSP

A

leukocytoclastic vasculitis and igA deposition in the small vessels of skin, joints, gi, kidney

83
Q

skin biopsy of HSP

A

vasculitis of the dermal capillaries and post capillary venules

84
Q

diagnosis of HSP (american)

A

Two of the following criteria must be present:
• Palpable purpura
• Age at onset ≤20 yr
• Bowel angina (postprandial abdominal pain, bloody diarrhea)
• Biopsy demonstrating intramural granulocytes in small arterioles and/or venules

85
Q

diagnosis HSP (european)

A

Palpable purpura (in absence of coagulopathy or thrombocytopenia) and
1 MORE:
• Abdominal pain (acute, diffuse, colicky pain)
• Arthritis or arthralgia
• Biopsy of affected tissue demonstrating predominant
immunoglobulin A deposition
• Renal involvement (proteinuria >3 grams/24 hr), hematuria or red cell casts

86
Q

age of onset takayasu

A

10 to 40yrs old

87
Q

pulseless disease

A

takayasu arteritis

88
Q

most commonly involed vessels in takayasu arteritis

A

subclaian
renal
carotid

89
Q

proposed classification of pedia takayasu arteritis

A

Angiographic abnormalities (conventional, CT, or magnetic resonance angiography) of the aorta or its main branches and ONE OF FF:
• Decreased peripheral artery pulse(s) and/or claudication of extremities
• Blood pressure difference between arms or legs of >10 mm Hg
• Bruits over the aorta and/or its major branches
• Hypertension (de ned by childhood normative data)
• Elevated acute phase reactant (erythrocyte sedimentation rate or C-reactive protein)

90
Q

gold standard in diagnosis of takayasu

A

arteriography of aorta and major branches: carotid, subclavina, pulmo, renal, mesenteric

91
Q

biopsy result in PAN

A

necrotizing vasculitis with granulocytes and monocytes infiltrating

92
Q

gold standard imaging for PAN

finding?

A

arteriography

beads on a string

93
Q

three forms of ANCA (antineutrophil circulating antibodies) asoociated vasculitis

A

wegener granulomatosis
microscopic polyangiitis
churg strauss syndrome

94
Q

mean age diagnosis wegener

male or female predominance?

A

14 yrs

female

95
Q

biopsy in wegener granulomatosis

A

necrotizing vasculitis
kidney: cresentic GN with little to no immune complex deposition
granulomatous inflammation

96
Q 
nasal ulceration -> saddle nose deformity
epistaxis
pulmo hemorrhage
hematuria, proteinuria, hypertension
conjunctivitis, uveitis, optic neuritis
A

wegener granulomatosis

97
Q

treatment of ANCA associated vasculitis

A

corticosteroids
oral cyclophosphamide (if kidney)
methotrexate (if pulmo only)

98
Q

peak age of growing pains

A

4-8 yrs old

99
Q

diffuse musculoskeletal pain in 3 different areas in 3 months

A

juvenile primary fibromyalgia syndrome

100
Q

ongoing burning limb that is subsequent to an injury, immobilization

A

complex regional pain syndrome

or previously Reflex sympathetic dystrophy

101
Q

key features of complex regional pain syndrome

A

allodynia
hyperalgesia
autonomic dysfxn (cyanosis, mottling, hyperhidrosis)

102
Q

age of onset complex regional pain syndrome

predominance

A

9-15yr

females 6:1

103
Q

treatment for complex regional pain syndrome

A

amytriptylline

104
Q

episodic cartilage inflammation causing destruction

A

relapsing polychondritis

105
Q

diagnosis psoriatic arthritis

A 
arthritis and psoriasis or arthritis and AT LEAST TWO
of following: 
(1) dactylitis, 
(2) nail pitting or onycholysis, 
(3) psoriasis in a 1st-degree relative
106
Q

diagnosis of Juvenile Ankylosing Spondylitis

A

xray evidence of sacroiliitis (sacroiliitis of grade 2 or greater bilaterally or at least grade 3 unilaterally)

PLUS ONE of FF:

inflammatory back pain
limitation of motion in the lumbar spine
limitation of chest expansion

107
Q

erythema nodosum, pyoderma gangrenosum, fever, weight loss, or anorexia in a child with chronic arthritis

A

inflammatory Bowel Disease

108
Q

Sclerosis typically starts on the __ side

A

iliac

109
Q

Squaring of the corners of the vertebral bodies and syndesmophyte formation

A

bamboo spine

Ankylosing Spondylitis

110
Q

AA amyloidosis has also been associated with

A

granulomatous dis- eases such as sarcoidosis, cystic brosis, Crohn disease, malignancies such as mesothelioma and Hodgkin diseases, intravenous drug abuse, and other infections, such as bronchiectasis and HIV.

111
Q

hereditary amyloidosis genes involved

A

transthyretin and apolipoprotein A

112
Q

fevers, myalgias, arthralgias, urticarial-like rash, and progressive sensorineural hearing loss

A

Muckle-Wells syndrome

113
Q

homogeneous eosinophilic material that stains with Congo red dye and demonstrates the pathognomonic “apple-green birefringence” in polarized light

A

amyloid bodies

114
Q

___ is the underlying cause of death in 40-60% of patients with amyloidosis

A

End-stage renal failure

PPS - From Nelson's (24 decks)

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