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
Treatment for Kawasaki
Options Pulse steroids (30mg/kg, max 1g) - up to 3 days IVIG can repeat if still have fever after 36 hours Infliximab (Remicade)
26
Conical teeth on exam ---think...
This is a red flag. Needs genetic testing
27
Rheum emergency in Hemophagocytic lymphohistiocytosis (HLH)
Iron >500
28
What is considered B symptoms
Fevers night sweats Weight loss
29
Causes of JDM (Juvenile dermatomyositis
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
30
Hyperreflexia and hypertonia upper or lower motor neuron?
upper
31
Hyporeflexia and hypotonia upper or lower motor neuorn
lower
32
Juvenile Dermatomyositis buzzwords
Difficulty -going upstairs -Combing hair -Standing up Symmetric intact sensation positive Gower's sign Trendelenburg gait
33
dosing for pulsing steroids
30mg/kg - max 1 g
34
Ash leaf spot think
tubular sclerosis
35
hypomelanic macules are called
ash leaf spots
36
Ash leaf spots (Tubular sclerosis)
37
heliotrope rash seen in Dermatomyositis
38
what happens in Juvenile Dermatomyositis
This is a vasculopathy where immune cells attack vessels
39
Risks in JDM
Bowel perforation in kids, JDM do not have associated cancer risk
40
symptoms of JDM
Proximal weakness> distal symmetric weakness +/- tenderness Limb /girdle muscles Ant neck flexors back and abd muscles can affect voice
41
Gottron Papules seen in Juvenile Dermatomyositis
42
Hypopigmented red papules over knuckles
Gottron papules
43
Dry dark and red dry skin
extensor erythema Juvenile dermatomyositis
44
Inverse Gottron papules Think...
They are on the palms instead of dorsal side of hand Juvenile Dermatomyositis Anti MDA5 type Causes aggressive interstitial lung disease
45
which type of JDM causes bowel perforation
JDM Anti-MJ
46
What type of JDM causes interstitial lung disease
Anti MDA-5
47
tell me about JDM with anti-p155/140 autoantibodies
Most patients are white and have extensive photosensitive skin rashes. also associated with a chronic course of illness and generalized lipodystrophy
48
tell me about Juvenile Dermatomyositis with Anti-MJ autoantibodies
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
Cutaneous signs in Juvenile Dermatomyositis
V-neck distribution Shawl sign Holster sign Cuticles and gums - look for prominent or tortuous capilaries
50
calcinosis in JDM, what should you order
Xray - see if it could be deeper or more
51
what associated problems associated with JDM
insulin resistance HTN increased bMI Metabolic syndrome Macrophage activation syndrome Anemia Osteopenia Osteoporosis
52
what labs test for muscle specific inflammatory markers
AST Aldolase CK LDH
53
which muscle inflammatory markers respond last and which respond first
first AST and CK Last Aldolase and LDH
54
what would warrant hospitalization in JDM
-Loss of ambulation -Inability to raise head against gravity -Bulbar symptoms ---Difficulty chewing ---Difficulty swallowing ---Difficulty talking -Dyspnea -Significant abd pain
55
Treatment for JDM
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
How long do you avoid live vaccines after IVIG and Methotrexate
11 months
57
term for capillaries inflamed in nails
nailfold capilaropathy
58
what rheum disease do you see rash in between knuckles and not on them
lupus
59
Infliximab is what type of medication
TNF inhibitor
60
what test before can start Humira
Quantiferon gold
61
Arthritis in a flair can show an _______ D-Dimer
elevated
62
polyarteritis nodosa (PAN) clinical symptoms
fever malaise weight loss + signs of multiorgan involvement
63
a systemic necrotizing vasculitis with aneurysm formation affects mall and/or med sized vessels
Polyarteritis Nodosa (PAN)
64
pan dx
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
steroids does what to WBC on labs
can drive it up
66
Lupus uses ____ criteria for dx
SLICC
67
what lab is most specific for lupus
Anti-smith
68
antiphospholipids during illness
can be falsely elevated. re-test in 3 months
69
Anti-ds-DNA is specific to
nephritis
70
Complements (C3, C4, CH50) are ______ in kidney disease
low
71
direct combs looks for
hemolytic anemia
72
when is CRP not reliable
with decreased liver fx....CRP is made in the liver
73
what does your body do with ferritin when your sick
stores/hoards iron if body thinks there is infection
74
> 500 ferritin think....
MAS (macrophage activation syndrome) HLH
75
Primary vs secondary HLH is driven by
genetic causes - can't turn off inflammation when there is an infection secondary - trigger by infection (EBV, cancer)
76
what IL involved in fever
IL-I and IL -VI
77
IN HLH, what will CRP and ESR look like
CRP elevated ESR low Big red flag
78
is JIA painful
no
79
HLH/MAS treatment
IVMP pulse 30mg/kg (max 1g) +/- anakinra
80
why is anakinra nice
stays in system for less than 24 hours
81
criteria for Behcett's
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
Rheumatologic differential for retinal vasculitis (+/- chorioretinitis) includes
Behçet's, lupus, polyarteritis nodosa, granulomatous polyangiitis, sarcoidosis, HLA-B27 associated disease, inflammatory bowel disease, relapsing polychondritis, and VKH
83
what does CH50 tell us?
the activity level of the complements
84
lg vessel vasculites (aorta)
Takayasu arteritis giant cell arteritis (adult only)
85
med vessel vasculitis (vessels going into organs)
Kawasaki PAN
86
small vessel vasculites (vessels in organs)
ANCA HSP Pulmonary capillaritis
87
If you have a large vessel vasculitis, can you have a small vessel vasculites? vise versa?
If you have large, you can have any of them. If you have small, you would not also have large.
88
what affects arteries and veins? lupus behcets
Behcets
89
when a patient presents with bleeding and a prolonged activated partial thromboplastin and prothrombin time, in combination with a lupus anticoagulant
Lupus anticoagulant-hypoprothrombinemia syndrome (LA-HPS)
90
vv ecmo vs VA ECMO
VV - bypass only lungs VA - bypass heart and lungs
91
nailbed capillaritis can also be called
periungal errythema
92
buzz words on MRI for arthritis
Synovial enhancement or uptake
93
_______ can present with monoarticular effusion
lyme it can also be polyarticular
94
IVIG can cause fever for
36 hours
95
acute rheumatic fever diagnosis
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
MPO AB
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
PR3AB
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
ANCA
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
c3
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
decreased C3 means
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
c4
Assessing disease activity in systemic lupus erythematosus, proliferative glomerulonephritis, rheumatoid arthritis, and autoimmune hemolytic anemia
102
decreased C4
C4 may be decreased in systemic lupus erythematosus, early glomerulonephritis, immune complex disease, cryoglobulinemia, hereditary angioedema, and congenital C4 deficiency.
103
positive ASO can last how long after strep
1 year
104
anti-DNase B titer
Demonstration of acute or recent streptococcal infection using anti-DNase B titer
105
titer to detect a more recent strep infection
ASO titer
106
If pt with suspected HSP is >10yrs old. What else should you be suspicious of
ANCA IBS
107
dosing for Plaquenil
3-5mk/kg/day higher doses associated with retinal injury
108
If you pull too much fluid off of a pt it will increase or decrease their creatinine
creatinine bump up
109
IVIG can cause headaches. what can you do to help
hydrate...have them drink lots of water to help prevent
110
Increased aldolase is an indicator of
myositis
111
scoring scale to grade their flare in lupus
SLEDEI
112
what enzyme looks at metabolizer ....lab should be ran before starting azathioprine
TPMT enzyme
113
Lupus with + _____ antibiody is at higher risk for vascular changes
antiphospholibid antibody
114
Lupus with + _____ antibiody is at higher risk for vascular changes
antiphospholipid antibody
115
HLH triggered by EBV,,,,give what med
Ritux bc knocks out B cells
116
after IVIG...vaccines
no live vaccines for 11 months
117
normal age for HSP? older kids check what
<10yrs ANCA and IBS
118
Photosensitivity occurs in ___% of patients with JDM
50% Sun exposure has been associated with exacerbation
119
Name at least 3 inflammatory myopathies resulting from infectious etilogies mimicking myositis of JDM
Viral: enterovirus, influenza, coxsackie, echovirus, parvovirus, Hep B, Human T lymphotropic virus 1 Bacterial: staph, strep, Lyme Parasitic: trichinosis and toxoplasmosis
120
what is the cause of Trendelenburg sign? Weakness of what muscles
weakness of the hip abductors
121
At what age is a Gower sign developmentally appropriate
Up to age 3
122
Rash on outside of thigh....erythematous papules on lateral aspect of the thighs
Holster sign
123
what type of PT should be initiated at the time of diagnosis (JDM)
Passive ROM 2-3 times per day to prevent loss of ROM using gentle stretching to regain lost range of motion
124
Death in JDM occurs in ___% of children
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
Provide at lease 3 examples that would classify with severe disability as related to JDM
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
what biopsy findings are associated with poor prognosis in JDM
extensive myopathic changes muscle infarction/necrosis lymphocytic infiltrates with follicle like structures including follicular dendritic cells high endothelial venules
127
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)
3 months
128
what sex is most commonly afflicted by JDM
Females >males
129
Aside from pathognomonic classic skin changes, how many other criteria by Bohan and Peter are needed to make a probable diagnosis of JDM
2
130
Provide most effective dose and method of providing methotrexate for JDM
up to 15mg/m2 dose per week as a subcutaneous injection
131
What findings/organ involvement would be active necessitating the use of cyclophosphamide therapy for JDM
Severe ulcerative skin disease cardiomyopathy ILD Severe GI involvement with vasculitis
132
The presence of TNF alpha 308A allele a risk factor for which 2 disease associations and JDM
Calcinosis poor course of disease
133
Mechanic hands associated with what AB in JDM
Anti-Jo AB
134
most common age distribution in children with JDM Median age of presentation
5-14 7
135
non infectious exposures that have been implicated in etiology and pathogenesis of JDM
Meds immunizations stressful life events unusual sun exposure animal contact weight training
136
Types of lipodystrophy associated with JDM
Generalized partial focal
137
metabolic features associated with acquired lipodystrohy in jDM
hypertryglyceride insulin resistance abnormal gtt/dm hypertension NASH generalized lipodystrophy -hypertrichosis -hyperpigmentation -enlarged cliteris
138
Normal liver synthetic function indicated by what labs
normal albumin, glucose, PT/INR