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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

triad of digeorge syndrome

A

22q,11.2 deletion

congenital heart dz, t cell deficiency, and hypocalcemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

poisoning with what can cause stocking glove neuropathy?

tx?

A

arsenic (pesticides)

dimercaprol or DMSA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

poison ivy/oak..type of rxn?

A

type 4

delayed, t cell and macrophage mediated

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

rhomboid crystals on synovial fluid analysis

A

pseudogout

calcium pyrophosphate crystal, positive birefringence

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

age >50, headache with localized tenderness and elevated ESR

dx?
tx?

A

temporal arteritis

glucocorticoids

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

which immunodeficiency disorder has increased risk for lymphoma?

A

CVID

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

HyperIgE syndrome

presentation?

A

recurrent skin staph ifxs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

wiskott aldrich syndrome
presentation?

tx?

A

normal B and T cells + thrombocytopenia + eczema

tx: bone marrow transplant

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

how to diagnose chronic granulomatous disease?

A

nitroblue tetrazolium testing

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

ice pack test

A

used to dx myasthenia gravis

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

drug currently approved to treat ALS?

A

riluzole

doesnt stop ALS but may prolong survival

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

what can give a false positive VDRL in women?

A

antiphospholipid antibody syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

mc joint for gout?

A

mtp of the big toe

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

most accurate test for gout?

A

aspiration of joint showing needle-shaped crystals with negative birefringence

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

tx for acute gout?

A

NSAIDS are BETTER than colchicine

use injected corticosteroids when there is no response to nsaids or nsaids are contraindicated

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

side effects of colchicine?

A

diarrhea and bone marrow suppression

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

do not start ____ during acute gout attack, but can continue it if already on the med

A

allopurinol

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

pseudogout
risk factors?
presentation?
dx?tx?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

low back pain, what is it?

hx of cancer, sudden onset of focal neuro deficit and point vertebral tenderness + hyperreflexia

A

cord compression

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

low back pain, what is it?

positive straight leg raise (pain into butt and LE), loss of LE reflexes

A

sciatica = disk herniation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

sx of L4 disk herniation? L5? S1?

A

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
Q

low back pain, what is it?

younger, pain worsens with rest and improves with activity, decreased chest mobility

A

ankylosing spondylitis

34
Q

low back pain, what is it?

bowel and bladder incontinence, erectile dysfunction, bilateral leg weakness and saddle anesthesia

A

cauda equina

35
Q

tx for disk herniation

A

nsaids and continuation of normal activity (NOT bed rest)

36
Q

when there is obvious cord compression, next step is…

A

steroids BEFORE imaging to decrease pressure on cord

37
Q

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?

A

spinal stenosis

MRI

38
Q

best treatment for fibromyalgia?

A

amitriptyline

39
Q

what dx test for carpel tunnel?

A

NOT MRI

do electromyography or nerve conduction testing

40
Q

dupuytren contracture
mech?
tx?

A

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
Q

most accurate test for rotator cuff tear?

A

MRI

42
Q

does plantar fasciitis improve or get worse with use?

A

improves! worst in AM

43
Q

patellofemoral syndrome

A

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
Q

what is the more specific test for RA?

A

anti-CCP&raquo_space;RF

45
Q

___ is spared in RA

A

DIP

46
Q

what syndrome = RA + splenomegaly + neutropenia

A

felty syndrome

47
Q

what syndrome = RA + penumoconniosis + lung nodules

A

caplan syndrome

48
Q

mc cause of death in RA?

A

CAD`

49
Q

before surgery, an RA patient needs ____

A

cervical spine xray

many RA patient have C1/C2 subluxation which could be problematic with intubation

50
Q
adverse effects of RA medications:
1 anti TNF
2 hydroxychloroquine
3 sulfasalazine
4 rituximab
5 gold salts
6 methotrexate
A
1 TB reactivation
2 retinal toxicity
3 rash, g6pd hemolysis
4 infection
5 nephrotic syndrome
6 liver, lung, marrow toxicity
51
Q

Juvenile rheumatoid arthritis
presentation?
labs?
tx?

A

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
Q

testing for SLE

A

ANA
anti-ds DNA
anti Smith

53
Q

anti-SSA antiSSB

A

sjogrens syndrome

Ro and La

54
Q

in an acute SLE flare, what labs change?

A

complement drops

anti-dsDNA rises

55
Q
antiphospholipid syndrome
presentation?
best initial test?
most accurate test?
tx?
A

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
Q

in APL, to prevent recurrent abortion what is the tx?

A

heparin and aspirin

warfarin is contraindicated in pregnancy!

57
Q

limited scleroderma

aka?

A

CREST
calcinosis, raynauds, esophageal dysmotility, sclerodectyly, telangiectasia

anticentromere ab

(no lung, kidney or heart involvement)

58
Q

SCLERODERMA

A

CREST + HEART/LUNG/KIDNEY FIBROSIS

scl70 (anti-topoisomerase)

59
Q

dermatomyositis

A

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
Q

sjogrens syndrome

A
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
Q

what organ is spared in polyarteritis nodosa?

A

lungs

62
Q

pain in pelvis and shoulders…hard to get up from chair and or brush hair in >50yo
normal labs + elevated ESR

A

polymyalgia rheumatica

63
Q

giant cell (temporal) arteritis

A

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
Q

wegener granulomatosis

A

upper and lowery respiratory tract findings plus renal issues
c-anca +

best test is lung biopsy

tx: prednisone and cyclophosphamide

65
Q

what 2 diseases are + for p-anca?

A

churg strauss and microscopic polyangiitis

66
Q

churg strauss
presentation?
most accurate test?
tx?

A

lung and renal issues + eosinophilia and asthma

biopsy

prednisone and cyclophosphamide

67
Q

HSP

A

GI pain or bleeding, skin purpura, arthralgias, and hematuria in KIDS

biopsy is best dx (leukoclastic vasculitis)

tx: steroids

68
Q

hx of hep C and nonblanching purpura + glomerulonephritis think…

tx?

A

cryoglobulinemia

treat underlying hep c with interferon and ribavirin (steroids dont work!)

69
Q

best initial test for ankylosing spondylitis?

most accurate?

A

SI joint xray (bamboo spine = fusion of vertebral joints)

MRI

70
Q

pencil in cup on xray
sausage digits

best tx?

A

psoriatic arthritis

NSAIDS –> methotrexate if resistant

71
Q

reiter syndrome triad

A

joint pain
uveitis/conjunctivitis
urethritis/genital

72
Q

rare side effects of bisphosphonates?

A

osteonecrosis of the jaw

73
Q

osteopenia vs osteoporosis in scores

A

osteopenia is bone density between 1 and 2.5 standard devliations below normal

osteoporosis >2.5 below normal

74
Q

osteoporosis tx

A

vitamin d and calcium –> bisphosphonates –> estrogen or raloxifene for postmeno women

75
Q

meds that cause drug induced lupus?

A

hydralazine
procainamide
methyldopa

76
Q

lupus nephritis ab? tx?

A

ds-DNA

dx with biopsy and then tx w iv cyclophosphamide –> oral mycophenylate motefil

77
Q

pannus formation in joints?

A

RA

78
Q

periarticular osteopenia?

A

RA

79
Q

tx for scleroderma renal crisis?

A

DO NOT GIVE STEROIDS

give ace inhibitor