ALLERGY/IMMUNOLOGY/Rheum Flashcards
low B cells and low Ig
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
CVID vs Brutons agammaglobulinemia
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
triad of digeorge syndrome
22q,11.2 deletion
congenital heart dz, t cell deficiency, and hypocalcemia
poisoning with what can cause stocking glove neuropathy?
tx?
arsenic (pesticides)
dimercaprol or DMSA
poison ivy/oak..type of rxn?
type 4
delayed, t cell and macrophage mediated
rhomboid crystals on synovial fluid analysis
pseudogout
calcium pyrophosphate crystal, positive birefringence
age >50, headache with localized tenderness and elevated ESR
dx?
tx?
temporal arteritis
glucocorticoids
anaphylactoid reaction versus anaphylaxis
difference in mechanism?
difference in treatment?
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
angioedema mechanism? associated with recent start of what medication? dx? tx?
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
CVID etiology? presentation? dx? tx?
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
which immunodeficiency disorder has increased risk for lymphoma?
CVID
x-linked (Bruton) agammaglobulinemia
presentation?
dx?
tx?
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
SCID
presentation/mechanism?
tx?
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)
IgA deficiency
presentation?
increased risk of?
tx?
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
HyperIgE syndrome
presentation?
recurrent skin staph ifxs
wiskott aldrich syndrome
presentation?
tx?
normal B and T cells + thrombocytopenia + eczema
tx: bone marrow transplant
chronic granulomatous disease presentation? common bugs? dx? tx?
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 to diagnose chronic granulomatous disease?
nitroblue tetrazolium testing
ice pack test
used to dx myasthenia gravis
applied over eyelids improves ptosis! (inhibits ach breakdown at NMJ)
ppsv23 vs pcv13 vaccines
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
drug currently approved to treat ALS?
riluzole
doesnt stop ALS but may prolong survival
what can give a false positive VDRL in women?
antiphospholipid antibody syndrome
Osteoarthritis
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
mc joint for gout?
mtp of the big toe
most accurate test for gout?
aspiration of joint showing needle-shaped crystals with negative birefringence
tx for acute gout?
NSAIDS are BETTER than colchicine
use injected corticosteroids when there is no response to nsaids or nsaids are contraindicated
side effects of colchicine?
diarrhea and bone marrow suppression
do not start ____ during acute gout attack, but can continue it if already on the med
allopurinol
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
low back pain, what is it?
hx of cancer, sudden onset of focal neuro deficit and point vertebral tenderness + hyperreflexia
cord compression
low back pain, what is it?
positive straight leg raise (pain into butt and LE), loss of LE reflexes
sciatica = disk herniation
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
low back pain, what is it?
younger, pain worsens with rest and improves with activity, decreased chest mobility
ankylosing spondylitis
low back pain, what is it?
bowel and bladder incontinence, erectile dysfunction, bilateral leg weakness and saddle anesthesia
cauda equina
tx for disk herniation
nsaids and continuation of normal activity (NOT bed rest)
when there is obvious cord compression, next step is…
steroids BEFORE imaging to decrease pressure on cord
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
best treatment for fibromyalgia?
amitriptyline
what dx test for carpel tunnel?
NOT MRI
do electromyography or nerve conduction testing
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
most accurate test for rotator cuff tear?
MRI
does plantar fasciitis improve or get worse with use?
improves! worst in AM
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
what is the more specific test for RA?
anti-CCP»_space;RF
___ is spared in RA
DIP
what syndrome = RA + splenomegaly + neutropenia
felty syndrome
what syndrome = RA + penumoconniosis + lung nodules
caplan syndrome
mc cause of death in RA?
CAD`
before surgery, an RA patient needs ____
cervical spine xray
many RA patient have C1/C2 subluxation which could be problematic with intubation
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
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
testing for SLE
ANA
anti-ds DNA
anti Smith
anti-SSA antiSSB
sjogrens syndrome
Ro and La
in an acute SLE flare, what labs change?
complement drops
anti-dsDNA rises
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)
in APL, to prevent recurrent abortion what is the tx?
heparin and aspirin
warfarin is contraindicated in pregnancy!
limited scleroderma
aka?
CREST
calcinosis, raynauds, esophageal dysmotility, sclerodectyly, telangiectasia
anticentromere ab
(no lung, kidney or heart involvement)
SCLERODERMA
CREST + HEART/LUNG/KIDNEY FIBROSIS
scl70 (anti-topoisomerase)
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
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)
what organ is spared in polyarteritis nodosa?
lungs
pain in pelvis and shoulders…hard to get up from chair and or brush hair in >50yo
normal labs + elevated ESR
polymyalgia rheumatica
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)
wegener granulomatosis
upper and lowery respiratory tract findings plus renal issues
c-anca +
best test is lung biopsy
tx: prednisone and cyclophosphamide
what 2 diseases are + for p-anca?
churg strauss and microscopic polyangiitis
churg strauss
presentation?
most accurate test?
tx?
lung and renal issues + eosinophilia and asthma
biopsy
prednisone and cyclophosphamide
HSP
GI pain or bleeding, skin purpura, arthralgias, and hematuria in KIDS
biopsy is best dx (leukoclastic vasculitis)
tx: steroids
hx of hep C and nonblanching purpura + glomerulonephritis think…
tx?
cryoglobulinemia
treat underlying hep c with interferon and ribavirin (steroids dont work!)
best initial test for ankylosing spondylitis?
most accurate?
SI joint xray (bamboo spine = fusion of vertebral joints)
MRI
pencil in cup on xray
sausage digits
best tx?
psoriatic arthritis
NSAIDS –> methotrexate if resistant
reiter syndrome triad
joint pain
uveitis/conjunctivitis
urethritis/genital
rare side effects of bisphosphonates?
osteonecrosis of the jaw
osteopenia vs osteoporosis in scores
osteopenia is bone density between 1 and 2.5 standard devliations below normal
osteoporosis >2.5 below normal
osteoporosis tx
vitamin d and calcium –> bisphosphonates –> estrogen or raloxifene for postmeno women
meds that cause drug induced lupus?
hydralazine
procainamide
methyldopa
lupus nephritis ab? tx?
ds-DNA
dx with biopsy and then tx w iv cyclophosphamide –> oral mycophenylate motefil
pannus formation in joints?
RA
periarticular osteopenia?
RA
tx for scleroderma renal crisis?
DO NOT GIVE STEROIDS
give ace inhibitor
