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Rheumatology Flashcards

1
Q

difference between CRP and ESR

A

Inflammatory markers
CRP -autoinflammatory - elevates quick and normalizes quick - cytokines (IL-I and IL-VI) drive CRP up ….think liver

ESR (erythrocyte sedimentation Rate) - Elevates slow and normalizes slow (remember this is the one that measures how many erythrocytes fall in an hour….things that make this heavy have higher ESR

things that elevate ESR….
-fibrinogen
-IgG - in Lupus your IgG is elevated which drives up your ESR
-High lipids
-SCD

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2
Q

Viral or Bacterial
High CRP
Low ESR

A

viral

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3
Q

which part of immune system is autoinflammatory

A

innate

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4
Q

which part of immune system is autoimmune

A

adaptive

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5
Q

Periodic fevers
Autoimmune or autoinflammatory

A

autoinflammatory so innate immune system

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6
Q

B cells and T cells
part of innate or adaptive

A

adaptive

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7
Q

Cytokines
part of innate of adaptive

A

innate

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8
Q

Anakinra is a ___ blocker

A

IL-I blocker

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9
Q

ANA positive….pt is at risk for

A

uveitis

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10
Q

+RF or CCP increases risk for

A

destructive arthritis

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11
Q

PFAPA

A

Periodic
Fever - 38.5-41C 2-7 days - can almost be predicted
Aphthus stomatitis - round gray oral ulcers
Adenitis

no cause so must be strep neg

non specific increase in CBC, CRP and ESR during attack. normal in between

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12
Q

Med to treat PFAPA

A

Steroid during flares (prednisolone)
if needed - Colchicine
50% resolve after tonsillectomy

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13
Q

FMF

A

Familial Mediterranean Fever

Fever
Serousitis - think where you have serous fluid
Risk for amyloidosis (renal)

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14
Q

If ESR is elevated, what else can you order

A

Fibrinogen
IgG

If IgG is elevated - Rheum cares about this

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15
Q

Medications for JIA

A

Scheduled NSAIDS- so BID - takes 2-3 weeks to hit peak antiinflammatory properties

then either Methotrexate or Humira

Also intra-articular steroid injections

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16
Q

difference in Methotrexate and Humira for JIA treatment

A

Methotrexate is 1x per week for about 2 years
2-3 months before you can fail therapy
PO or subq injection however the injection is more effective and the po pills is 2.5mg each. Average dosing is 20-25 mg so it is a lot of pills
Also day after you feel tired and n/v. oral. The injection has a smaller side effect profile
folic acid helps with side effects because the med depletes the folic acid

Humira
injection is 1x every 2 weeks
This is a biologic
more effective
less side effects
black box warding for males with IBD there is an increased risk of lymphoma

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17
Q

JIA diagnostic criterion

A

> 6 weeks in 1 joint

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18
Q

systemic JIA is autoimmune or autoinflammatory

A

autoinflammatory

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19
Q

what can you recommend for hair loss in JIA

A

Folic acid and Biotin

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20
Q

CRASH & BURN

A

Kawasaki
Conjunctivitis (without exudate) * Also limbic sparing is a big clue - red eyes with clear halo around pupil

Rash (not vesicular or bullous)

Adenopathy (must be at least one lymph node in the anterior chain at least 1.5)

Strawberry tongue (mucositis - fissuring on lips)

Hand or Feet swelling or erythema

Burn (5 days of fever)

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21
Q

age group not to trust in kawasaki

A

12 mos and younger. Can be an incomplete picture

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22
Q

Thrombocytopenia worsening during kawasaki

A

Not a sign of disease worsening……it is normal progression

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23
Q

What is most concerning in kawasaki

A

Coronary dilation

-increase risk of heart attack at a young age

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24
Q

After giving IVIG for kawasaki, how long do we allow fever for before we treat again

A

36 hours

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25
Q

Treatment for Kawasaki

A

Options
Pulse steroids (30mg/kg, max 1g) - up to 3 days

IVIG
can repeat if still have fever after 36 hours

Infliximab (Remicade)

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26
Q

Conical teeth on exam —think…

A

This is a red flag.
Needs genetic testing

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27
Q

Rheum emergency in Hemophagocytic lymphohistiocytosis (HLH)

A

Iron >500

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28
Q

What is considered B symptoms

A

Fevers
night sweats
Weight loss

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29
Q

Causes of JDM (Juvenile dermatomyositis

A

Infections
-viral
-bacterial
-parasitic

Non-infectious
-Muscular dystrophies
-Multiple sclerosis
-Paraneoplastic myopathy
-Metabolic myopathy

Immunologic
-inflammatory
-Lupus
-Guinane barre syndrome
-other connective tissue disease

other
-drug induced
-toxin

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30
Q

Hyperreflexia and hypertonia

upper or lower motor neuron?

A

upper

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31
Q

Hyporeflexia and hypotonia

upper or lower motor neuorn

A

lower

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32
Q

Juvenile Dermatomyositis buzzwords

A

Difficulty
-going upstairs
-Combing hair
-Standing up

Symmetric
intact sensation
positive Gower’s sign
Trendelenburg gait

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33
Q

dosing for pulsing steroids

A

30mg/kg - max 1 g

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34
Q

Ash leaf spot think

A

tubular sclerosis

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35
Q

hypomelanic macules are called

A

ash leaf spots

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36
Q
A

Ash leaf spots (Tubular sclerosis)

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37
Q
A

heliotrope rash
seen in Dermatomyositis

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38
Q

what happens in Juvenile Dermatomyositis

A

This is a vasculopathy where immune cells attack vessels

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39
Q

Risks in JDM

A

Bowel perforation
in kids, JDM do not have associated cancer risk

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40
Q

symptoms of JDM

A

Proximal weakness> distal symmetric weakness
+/- tenderness
Limb /girdle muscles
Ant neck flexors back and abd muscles
can affect voice

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41
Q
A

Gottron Papules
seen in Juvenile Dermatomyositis

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42
Q

Hypopigmented red papules over knuckles

A

Gottron papules

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43
Q

Dry dark and red dry skin

A

extensor erythema
Juvenile dermatomyositis

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44
Q

Inverse Gottron papules
Think…

A

They are on the palms instead of dorsal side of hand
Juvenile Dermatomyositis
Anti MDA5 type

Causes aggressive interstitial lung disease

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45
Q

which type of JDM causes bowel perforation

A

JDM
Anti-MJ

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46
Q

What type of JDM causes interstitial lung disease

A

Anti MDA-5

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47
Q

tell me about JDM with anti-p155/140 autoantibodies

A

Most patients are white and have extensive photosensitive skin rashes.
also associated with a chronic course of illness and generalized lipodystrophy

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48
Q

tell me about Juvenile Dermatomyositis with Anti-MJ autoantibodies

A

Most patients are white. Anti-MJ autoantibodies are associated with muscle cramps, muscle atrophy, joint contractures, dysphonia, and an absence of truncal rashes. Patients with anti-MJ autoantibodies tended to be weaker and have decreased physical function

also gastro perforation

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49
Q

Cutaneous signs in Juvenile Dermatomyositis

A

V-neck distribution
Shawl sign
Holster sign

Cuticles and gums - look for prominent or tortuous capilaries

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50
Q

calcinosis in JDM, what should you order

A

Xray - see if it could be deeper or more

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51
Q

what associated problems associated with JDM

A

insulin resistance
HTN
increased bMI
Metabolic syndrome
Macrophage activation syndrome
Anemia
Osteopenia
Osteoporosis

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52
Q

what labs test for muscle specific inflammatory markers

A

AST
Aldolase
CK
LDH

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53
Q

which muscle inflammatory markers respond last and which respond first

A

first
AST and CK

Last
Aldolase and LDH

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54
Q

what would warrant hospitalization in JDM

A

-Loss of ambulation
-Inability to raise head against gravity
-Bulbar symptoms
—Difficulty chewing
—Difficulty swallowing
—Difficulty talking
-Dyspnea
-Significant abd pain

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55
Q

Treatment for JDM

A

Sunscreen and Sun protection -> Sun flairs disease

1st line
-Methotrexate (muscle)
-steroids
-IVIG
-Hydroxychloroquine (skin)

2nd line
-Mycophenalate mofetil
-Rituximab
-Tacro
-Cyclosporine

Severe disease
-Cyclosporine

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56
Q

How long do you avoid live vaccines after IVIG and Methotrexate

A

11 months

57
Q

term for capillaries inflamed in nails

A

nailfold capilaropathy

58
Q

what rheum disease do you see rash in between knuckles and not on them

A

lupus

59
Q

Infliximab is what type of medication

A

TNF inhibitor

60
Q

what test before can start Humira

A

Quantiferon gold

61
Q

Arthritis in a flair can show an _______ D-Dimer

A

elevated

62
Q

polyarteritis nodosa (PAN) clinical symptoms

A

fever
malaise
weight loss
+ signs of multiorgan involvement

63
Q

a systemic necrotizing vasculitis with aneurysm formation affects mall and/or med sized vessels

A

Polyarteritis Nodosa (PAN)

64
Q

pan dx

A

must have abnormalities in sm/md A. (necrotizing vasculitis, aneurysm, stenosis or occlusions)
+ 1/5 systemic
1) skin involvement
2) myalgias
3) HTN
4) Peripheral neuropathy
5) Kidney involvement

65
Q

steroids does what to WBC on labs

A

can drive it up

66
Q

Lupus uses ____ criteria for dx

A

SLICC

67
Q

what lab is most specific for lupus

A

Anti-smith

68
Q

antiphospholipids during illness

A

can be falsely elevated. re-test in 3 months

69
Q

Anti-ds-DNA is specific to

A

nephritis

70
Q

Complements (C3, C4, CH50) are ______ in kidney disease

A

low

71
Q

direct combs looks for

A

hemolytic anemia

72
Q

when is CRP not reliable

A

with decreased liver fx….CRP is made in the liver

73
Q

what does your body do with ferritin when your sick

A

stores/hoards iron if body thinks there is infection

74
Q

> 500 ferritin think….

A

MAS (macrophage activation syndrome)

HLH

75
Q

Primary vs secondary HLH is driven by

A

genetic causes - can’t turn off inflammation when there is an infection
secondary - trigger by infection (EBV, cancer)

76
Q

what IL involved in fever

A

IL-I and IL -VI

77
Q

IN HLH, what will CRP and ESR look like

A

CRP elevated
ESR low

Big red flag

78
Q

is JIA painful

A

no

79
Q

HLH/MAS treatment

A

IVMP pulse 30mg/kg (max 1g)
+/- anakinra

80
Q

why is anakinra nice

A

stays in system for less than 24 hours

81
Q

criteria for Behcett’s

A

patients with recurrent oral aphthae (at least three times in one year) plus two of the following clinical features:
●Recurrent genital aphthae (aphthous ulceration or scarring).
●Eye lesions (including anterior or posterior uveitis, cells in vitreous on slit lamp examination, or retinal vasculitis observed by an ophthalmologist).
●Skin lesions (including erythema nodosum, pseudofolliculitis, papulopustular lesions, or acneiform nodules consistent with Behçet syndrome).
●A positive pathergy test. Pathergy is defined by a papule 2 mm or more in size developing 24 to 48 hours after oblique insertion of a 20-gauge needle 5 mm into the skin, generally performed on the forearm.

82
Q

Rheumatologic differential for retinal vasculitis (+/- chorioretinitis) includes

A

Behçet’s, lupus, polyarteritis nodosa, granulomatous polyangiitis, sarcoidosis, HLA-B27 associated disease, inflammatory bowel disease, relapsing polychondritis, and VKH

83
Q

what does CH50 tell us?

A

the activity level of the complements

84
Q

lg vessel vasculites
(aorta)

A

Takayasu arteritis
giant cell arteritis (adult only)

85
Q

med vessel vasculitis
(vessels going into organs)

A

Kawasaki
PAN

86
Q

small vessel vasculites
(vessels in organs)

A

ANCA
HSP
Pulmonary capillaritis

87
Q

If you have a large vessel vasculitis, can you have a small vessel vasculites? vise versa?

A

If you have large, you can have any of them. If you have small, you would not also have large.

88
Q

what affects arteries and veins?
lupus
behcets

A

Behcets

89
Q

when a patient presents with bleeding and a prolonged activated partial thromboplastin and prothrombin time, in combination with a lupus anticoagulant

A

Lupus anticoagulant-hypoprothrombinemia syndrome (LA-HPS)

90
Q

vv ecmo
vs
VA ECMO

A

VV - bypass only lungs
VA - bypass heart and lungs

91
Q

nailbed capillaritis can also be called

A

periungal errythema

92
Q

buzz words on MRI for arthritis

A

Synovial enhancement
or uptake

93
Q

_______ can present with monoarticular effusion

A

lyme

it can also be polyarticular

94
Q

IVIG can cause fever for

A

36 hours

95
Q

acute rheumatic fever diagnosis

A

either 2 major or 1major with 2 minor

major
carditits
arthritis (poly only)
chorea
erythema marginatum
subcutaneous nodules

minor
polyarthralgia
fever >=38.5
ESR >=60 in the first hour and or CRP>= 3.0
prolonged PR interval

evidence of GAS infection for either

96
Q

MPO AB

A

Autoantibodies to MPO (MPO antineutrophil cytoplasmic antibodies: ANCA) occur in several diseases and may be involved in the pathogenesis of vascular inflammation in patients with microscopic polyangiitis (MPA).(1,2) Patients with MPA often develop MPO ANCA and may present with azotemia secondary to glomerulonephritis (pauci-immune necrotizing glomerulonephritis). MPO ANCA are not specific for MPA, and also may be detected in patients with systemic lupus erythematosus with or without lupus nephritis, Goodpasture syndrome and Churg-Strauss syndrome.

97
Q

PR3AB

A

Evaluating patients suspected of having Wegener granulomatosis (WG) now called C-ANCA

Distinguishing between WG and other forms of vasculitis, in conjunction with:

-MPO / Myeloperoxidase Antibodies, IgG, Serum

-ANCA / Cytoplasmic Neutrophil Antibodies, Serum (may be obtained as VASC / Antineutrophil Cytoplasmic Antibodies Vasculitis Panel, Serum)

98
Q

ANCA

A

Evaluating patients suspected of having antineutrophil cytoplasmic antibody-associated vasculitis (granulomatosis with polyangiitis and microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis)

Antineutrophil cytoplasmic antibodies (ANCA) can occur in patients with small blood vessel vasculitis, including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), or eosinophilic granulomatosis with polyangiitis (EGPA), collectively referred to as ANCA-associated vasculitis (AAV).(2)

99
Q

c3

A

Assessing disease activity in systemic lupus erythematosus (SLE)

Decreased C3 may be associated with acute glomerulonephritis, membranoproliferative glomerulonephritis, immune complex disease, active systemic lupus erythematosus, septic shock, and end-stage liver disease.

100
Q

decreased C3 means

A

Decreased C3 may be associated with acute glomerulonephritis, membranoproliferative glomerulonephritis, immune complex disease, active systemic lupus erythematosus, septic shock, and end-stage liver disease.

101
Q

c4

A

Assessing disease activity in systemic lupus erythematosus, proliferative glomerulonephritis, rheumatoid arthritis, and autoimmune hemolytic anemia

102
Q

decreased C4

A

C4 may be decreased in systemic lupus erythematosus, early glomerulonephritis, immune complex disease, cryoglobulinemia, hereditary angioedema, and congenital C4 deficiency.

103
Q

positive ASO can last how long after strep

A

1 year

104
Q

anti-DNase B titer

A

Demonstration of acute or recent streptococcal infection using anti-DNase B titer

105
Q

titer to detect a more recent strep infection

A

ASO titer

106
Q

If pt with suspected HSP is >10yrs old. What else should you be suspicious of

A

ANCA
IBS

107
Q

dosing for Plaquenil

A

3-5mk/kg/day
higher doses associated with retinal injury

108
Q

If you pull too much fluid off of a pt it will increase or decrease their creatinine

A

creatinine bump up

109
Q

IVIG can cause headaches. what can you do to help

A

hydrate…have them drink lots of water to help prevent

110
Q

Increased aldolase is an indicator of

A

myositis

111
Q

scoring scale to grade their flare in lupus

A

SLEDEI

112
Q

what enzyme looks at metabolizer ….lab should be ran before starting azathioprine

A

TPMT enzyme

113
Q

Lupus with + _____ antibiody is at higher risk for vascular changes

A

antiphospholibid antibody

114
Q

Lupus with + _____ antibiody is at higher risk for vascular changes

A

antiphospholipid antibody

115
Q

HLH triggered by EBV,,,,give what med

A

Ritux bc knocks out B cells

116
Q

after IVIG…vaccines

A

no live vaccines for 11 months

117
Q

normal age for HSP?
older kids check what

A

<10yrs
ANCA and IBS

118
Q

Photosensitivity occurs in ___% of patients with JDM

A

50%
Sun exposure has been associated with exacerbation

119
Q

Name at least 3 inflammatory myopathies resulting from infectious etilogies mimicking myositis of JDM

A

Viral: enterovirus, influenza, coxsackie, echovirus, parvovirus, Hep B, Human T lymphotropic virus 1
Bacterial: staph, strep, Lyme
Parasitic: trichinosis and toxoplasmosis

120
Q

what is the cause of Trendelenburg sign? Weakness of what muscles

A

weakness of the hip abductors

121
Q

At what age is a Gower sign developmentally appropriate

A

Up to age 3

122
Q

Rash on outside of thigh….erythematous papules on lateral aspect of the thighs

A

Holster sign

123
Q

what type of PT should be initiated at the time of diagnosis (JDM)

A

Passive ROM 2-3 times per day to prevent loss of ROM using gentle stretching to regain lost range of motion

124
Q

Death in JDM occurs in ___% of children

A

1-2%: most often within 2 years of disease onset and associated with progressive skin and muscle disease that is unresponsive to tx

Death often resulting from resp insufficiency or ILD, myocarditis, acute GI ulceration w/perforation or bleeding

125
Q

Provide at lease 3 examples that would classify with severe disability as related to JDM

A

Inability to get out of bed
CMAS score of 15 or MMT8 score of 30
Parenchymal lung disease
gut vasculitis
other autoimmune or mimicking disease
need for ICU management
Age <1 yr
Aspiration or dysphagia

126
Q

what biopsy findings are associated with poor prognosis in JDM

A

extensive myopathic changes
muscle infarction/necrosis
lymphocytic infiltrates with follicle like structures including follicular dendritic cells
high endothelial venules

127
Q

After how many months of initiating hydroxychloroquine is it expected to improve skin rash associated with JDM according to brief study by olson and lindsley (only 9 children)

A

3 months

128
Q

what sex is most commonly afflicted by JDM

A

Females >males

129
Q

Aside from pathognomonic classic skin changes, how many other criteria by Bohan and Peter are needed to make a probable diagnosis of JDM

A

2

130
Q

Provide most effective dose and method of providing methotrexate for JDM

A

up to 15mg/m2 dose per week as a subcutaneous injection

131
Q

What findings/organ involvement would be active necessitating the use of cyclophosphamide therapy for JDM

A

Severe ulcerative skin disease
cardiomyopathy
ILD
Severe GI involvement with vasculitis

132
Q

The presence of TNF alpha 308A allele a risk factor for which 2 disease associations and JDM

A

Calcinosis
poor course of disease

133
Q

Mechanic hands associated with what AB in JDM

A

Anti-Jo AB

134
Q

most common age distribution in children with JDM
Median age of presentation

A

5-14
7

135
Q

non infectious exposures that have been implicated in etiology and pathogenesis of JDM

A

Meds
immunizations
stressful life events
unusual sun exposure
animal contact
weight training

136
Q

Types of lipodystrophy associated with JDM

A

Generalized
partial
focal

137
Q

metabolic features associated with acquired lipodystrohy in jDM

A

hypertryglyceride
insulin resistance
abnormal gtt/dm
hypertension
NASH

generalized lipodystrophy
-hypertrichosis
-hyperpigmentation
-enlarged cliteris

138
Q

Normal liver synthetic function indicated by what labs

A

normal albumin, glucose, PT/INR

clinicals (8 decks)

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