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ALLERGY/IMMUNOLOGY/Rheum Flashcards

1
Q

low B cells and low Ig

A

brutons agammaglobulinemia

defect in tyrosine kinase in B cells
so failure of B cell maturation = low Ig production

presents with recurrent sinopulmonary and GI ifx

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2
Q

CVID vs Brutons agammaglobulinemia

A

brutons –> low B cells and low Ig levels

CVID –> Ig levels low but B cell is normal

both have sinopulmonary and GI ifx but CVID is less severe after adolecense

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3
Q

triad of digeorge syndrome

A

22q,11.2 deletion

congenital heart dz, t cell deficiency, and hypocalcemia

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4
Q

poisoning with what can cause stocking glove neuropathy?

tx?

A

arsenic (pesticides)

dimercaprol or DMSA

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5
Q

poison ivy/oak..type of rxn?

A

type 4

delayed, t cell and macrophage mediated

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6
Q

rhomboid crystals on synovial fluid analysis

A

pseudogout

calcium pyrophosphate crystal, positive birefringence

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7
Q

age >50, headache with localized tenderness and elevated ESR

dx?
tx?

A

temporal arteritis

glucocorticoids

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8
Q

anaphylactoid reaction versus anaphylaxis

difference in mechanism?
difference in treatment?

A

A rxn is non IGE related (no prior sensitization) versus anaphylaxis has prior sensitization to the antigen

treated the same way: IM epinephrine, antihistamines (diphenhydramine H1 blocker and ranitidine H2 blocker), and glucocorticoids

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9
Q 
angioedema
mechanism?
associated with recent start of what medication?
dx?
tx?
A

deficiency of c1 esterase inhibitor that causes sudden facial swelling and stidor with NO urticaria

dx with decreased C2 and C4 levels in the complement pathway and deficiency of c1 esterase inhibitor
tx
does not respond to glucocorticoids
acute –> airway protection FIRST and then FFP and ecallantide
long term –> androgens like danazol

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10
Q 
CVID
etiology?
presentation?
dx?
tx?
A

normal B cell count, but they dont produce enough Ig = low IgG, IgM, IgA

adults with recurrent sino-pulmonary ifx, GI malabsorption, and anemia

dx: low Ig levels and low response of B cells to antigen stimulation; normal B count; normal lymphoid tissue present
tx: abx for infections and regular IVIG

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11
Q

which immunodeficiency disorder has increased risk for lymphoma?

A

CVID

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12
Q

x-linked (Bruton) agammaglobulinemia

presentation?
dx?
tx?

A

male kids with sinopulmonary ifxs

low B cells, normal T cells + decreased lymphoid tissue (little or absence of tonsils, LNs, and spleen)

regular IVIG and abx for ifxs

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13
Q

SCID
presentation/mechanism?
tx?

A

B and T cells are BOTH deficient
B cell deficiency causes low Ig = sinopulm ifxs starting at 6 mos
T cell deficiency causes OIs like PCP, varicella, and candida

tx: bone marrow transplant (can be curative)

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14
Q

IgA deficiency
presentation?
increased risk of?
tx?

A

recurrent sinopulm ifxs + allergies + anaphylaxis to blood transfusions (normal IgA level) + GI malabsorption

risk of vitiligo, thyroiditis, and RA

tx: blood only from IgA deficient donors or that has been washed
IVIG DOES NOT WORK

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15
Q

HyperIgE syndrome

presentation?

A

recurrent skin staph ifxs

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16
Q

wiskott aldrich syndrome
presentation?

tx?

A

normal B and T cells + thrombocytopenia + eczema

tx: bone marrow transplant

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17
Q 
chronic granulomatous disease
presentation?
common bugs?
dx?
tx?
A

extensive inflammatory reactions = recurrent bacterial and fungal ifxs
granulomas and LNs with purulent material leaking out
often will see aphthous ulcers and nasal inflammation

staph/burkholderia/nocardia/aspergillus

dx: nitroblue tetrazolium testing (decrease in NADPH oxidase)

interferon gamma + bone marrow transplant

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18
Q

how to diagnose chronic granulomatous disease?

A

nitroblue tetrazolium testing

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19
Q

ice pack test

A

used to dx myasthenia gravis

applied over eyelids improves ptosis! (inhibits ach breakdown at NMJ)

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20
Q

ppsv23 vs pcv13 vaccines

A

ages 19-64 ppsv23 alone annually for those with chronic diseases (pcv13 for high risk pt)

ages 65+ 1 dose of pcv13 followed by ppsv23 at a later time

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21
Q

drug currently approved to treat ALS?

A

riluzole

doesnt stop ALS but may prolong survival

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22
Q

what can give a false positive VDRL in women?

A

antiphospholipid antibody syndrome

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23
Q

Osteoarthritis

A

DIPs
crepitus
stiffness <15 min
dip enlargement (heberden) and pip enlargement (bouchard )

normal labs

xray shows: joint space narrowing, osteophytes

tx: weightloss, exercise, acetaminophen –> nsaids, capsaicin, and intraarticular steroids

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24
Q

mc joint for gout?

A

mtp of the big toe

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25
most accurate test for gout?
aspiration of joint showing needle-shaped crystals with negative birefringence
26
tx for acute gout?
NSAIDS are BETTER than colchicine use injected corticosteroids when there is no response to nsaids or nsaids are contraindicated
27
side effects of colchicine?
diarrhea and bone marrow suppression
28
do not start ____ during acute gout attack, but can continue it if already on the med
allopurinol
29
pseudogout risk factors? presentation? dx?tx?
ca pyrophosphate deposition associated with hemochromatosis and hyperparathyroidism often in knee and wrist uric acid levels are NOrMAL --> arthrocentesis will show positive birefringent rhomboid crystals NSAIDS --> steroids (triamcinolone) colchicine as ppx
30
low back pain, what is it? hx of cancer, sudden onset of focal neuro deficit and point vertebral tenderness + hyperreflexia
cord compression
31
low back pain, what is it? positive straight leg raise (pain into butt and LE), loss of LE reflexes
sciatica = disk herniation
32
sx of L4 disk herniation? L5? S1?
L4 deficits = foot dorsiflexion, knee reflex, inner calf sensory L5 deficits = toe dorsiflexion, no reflex, inner forefoot sensory S1 - eversion of foot, ankle reflex, outer foot sensory
33
low back pain, what is it? younger, pain worsens with rest and improves with activity, decreased chest mobility
ankylosing spondylitis
34
low back pain, what is it? bowel and bladder incontinence, erectile dysfunction, bilateral leg weakness and saddle anesthesia
cauda equina
35
tx for disk herniation
nsaids and continuation of normal activity (NOT bed rest)
36
when there is obvious cord compression, next step is...
steroids BEFORE imaging to decrease pressure on cord
37
back pain, what is it? pain that radiates to butt and thighs bilaterally, worse when walking downhill (leaning back), better when sitting or leaning forward, normal ABI best test?
spinal stenosis MRI
38
best treatment for fibromyalgia?
amitriptyline
39
what dx test for carpel tunnel?
NOT MRI do electromyography or nerve conduction testing
40
dupuytren contracture mech? tx?
hyperplasia of the palmar fascia leading to contracture of the 4th and 5th digits triamcinolone, lidocaine or collagenase --> surgical release when function is impaired
41
most accurate test for rotator cuff tear?
MRI
42
does plantar fasciitis improve or get worse with use?
improves! worst in AM
43
patellofemoral syndrome
pain anterior to knee or under patella especially with stairs worse when just starting to walk after prolonges sitting, improves after walking crepitus, joint locking and instability tx: pt and strength training
44
what is the more specific test for RA?
anti-CCP >>RF
45
___ is spared in RA
DIP
46
what syndrome = RA + splenomegaly + neutropenia
felty syndrome
47
what syndrome = RA + penumoconniosis + lung nodules
caplan syndrome
48
mc cause of death in RA?
CAD`
49
before surgery, an RA patient needs ____
cervical spine xray many RA patient have C1/C2 subluxation which could be problematic with intubation
50
``` adverse effects of RA medications: 1 anti TNF 2 hydroxychloroquine 3 sulfasalazine 4 rituximab 5 gold salts 6 methotrexate ```
``` 1 TB reactivation 2 retinal toxicity 3 rash, g6pd hemolysis 4 infection 5 nephrotic syndrome 6 liver, lung, marrow toxicity ```
51
Juvenile rheumatoid arthritis presentation? labs? tx?
high spiking fever in a young person with a salmon colored rash on chest/abdomen +/- splenomegaly, mild joint pain and pericardial effusion ferritin is elevated (acute phase reactant) aspirin or nsaids --> steroids if resistant
52
testing for SLE
ANA anti-ds DNA anti Smith
53
anti-SSA antiSSB
sjogrens syndrome | Ro and La
54
in an acute SLE flare, what labs change?
complement drops | anti-dsDNA rises
55
``` antiphospholipid syndrome presentation? best initial test? most accurate test? tx? ```
lupus anticoagulant or anticardiolipin ab thrombosis in both arteries and veins and spontaneous recurrent abortions ass with: normal PT and INR, and elevated aPTT false positive VDRL/RPR best initial test = mixing study most apecific = russel viper venom tx recurrent thrombosis with lifelong heparin to warfarin (inr 2-3)
56
in APL, to prevent recurrent abortion what is the tx?
heparin and aspirin | warfarin is contraindicated in pregnancy!
57
limited scleroderma | aka?
CREST calcinosis, raynauds, esophageal dysmotility, sclerodectyly, telangiectasia anticentromere ab (no lung, kidney or heart involvement)
58
SCLERODERMA
CREST + HEART/LUNG/KIDNEY FIBROSIS scl70 (anti-topoisomerase)
59
dermatomyositis
proximal muscle weakness (getting up from seat or stairs) ``` shawl sign (erythema) heliotrope rash gottrom papules (scaly patches on back of hands) ``` ass with cancer 25% best initial test: cpk and aldolase most accurate = muscle biopsy tx: steroids
60
sjogrens syndrome
``` keratoconjunctivitis sicca (dryness of eyes "sand"), dry mouth/dental caries dyspareunia ``` ass with: lymphoma best initial: shirmer eye test or SSa/SSB in blood most accurate: lip or parotid gland biopsy tx: water the mouth + fluoride + artifical tears + pilocarpine to stimulate Ach (saliva production)
61
what organ is spared in polyarteritis nodosa?
lungs
62
pain in pelvis and shoulders...hard to get up from chair and or brush hair in >50yo normal labs + elevated ESR
polymyalgia rheumatica
63
giant cell (temporal) arteritis
visual sx, jaw claudication when chewing, scalp tenderness, ha, bruits esr and crp are elevated dx: biopsy of temporal artery tx: start prednisone BEFORE BIOPSY RESULTS GET BACK (blindness is not reversible)
64
wegener granulomatosis
upper and lowery respiratory tract findings plus renal issues c-anca + best test is lung biopsy tx: prednisone and cyclophosphamide
65
what 2 diseases are + for p-anca?
churg strauss and microscopic polyangiitis
66
churg strauss presentation? most accurate test? tx?
lung and renal issues + eosinophilia and asthma biopsy prednisone and cyclophosphamide
67
HSP
GI pain or bleeding, skin purpura, arthralgias, and hematuria in KIDS biopsy is best dx (leukoclastic vasculitis) tx: steroids
68
hx of hep C and nonblanching purpura + glomerulonephritis think... tx?
cryoglobulinemia treat underlying hep c with interferon and ribavirin (steroids dont work!)
69
best initial test for ankylosing spondylitis? | most accurate?
SI joint xray (bamboo spine = fusion of vertebral joints) | MRI
70
pencil in cup on xray sausage digits best tx?
psoriatic arthritis NSAIDS --> methotrexate if resistant
71
reiter syndrome triad
joint pain uveitis/conjunctivitis urethritis/genital
72
rare side effects of bisphosphonates?
osteonecrosis of the jaw
73
osteopenia vs osteoporosis in scores
osteopenia is bone density between 1 and 2.5 standard devliations below normal osteoporosis >2.5 below normal
74
osteoporosis tx
vitamin d and calcium --> bisphosphonates --> estrogen or raloxifene for postmeno women
75
meds that cause drug induced lupus?
hydralazine procainamide methyldopa
76
lupus nephritis ab? tx?
ds-DNA dx with biopsy and then tx w iv cyclophosphamide --> oral mycophenylate motefil
77
pannus formation in joints?
RA
78
periarticular osteopenia?
RA
79
tx for scleroderma renal crisis?
DO NOT GIVE STEROIDS give ace inhibitor

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