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

34
Q

Ash leaf spot think

A

tubular sclerosis

35
Q

hypomelanic macules are called

A

ash leaf spots

36
Q
A

Ash leaf spots (Tubular sclerosis)

37
Q
A

heliotrope rash
seen in Dermatomyositis

38
Q

what happens in Juvenile Dermatomyositis

A

This is a vasculopathy where immune cells attack vessels

39
Q

Risks in JDM

A

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

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

41
Q
A

Gottron Papules
seen in Juvenile Dermatomyositis

42
Q

Hypopigmented red papules over knuckles

A

Gottron papules

43
Q

Dry dark and red dry skin

A

extensor erythema
Juvenile dermatomyositis

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

45
Q

which type of JDM causes bowel perforation

A

JDM
Anti-MJ

46
Q

What type of JDM causes interstitial lung disease

A

Anti MDA-5

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

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

49
Q

Cutaneous signs in Juvenile Dermatomyositis

A

V-neck distribution
Shawl sign
Holster sign

Cuticles and gums - look for prominent or tortuous capilaries

50
Q

calcinosis in JDM, what should you order

A

Xray - see if it could be deeper or more

51
Q

what associated problems associated with JDM

A

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

52
Q

what labs test for muscle specific inflammatory markers

A

AST
Aldolase
CK
LDH

53
Q

which muscle inflammatory markers respond last and which respond first

A

first
AST and CK

Last
Aldolase and LDH

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

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

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