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