crystal diseases

Question 1

crystal diseases

Answer 1

monosodium urate
calcium pyrophophate dihydrate
basic CaPO4 (hydroxyapatite)
Ca oxalate
cholesterol crystals
monoclonal proteins

Question 2

hydroxyapetite

Answer 2

CaPO4
calcific tendonitis
crystals also in urine

Question 3

oxylosis of spine and hand + nephrolythiasis

Answer 3

Ca oxylate crystals
black in urine
terminal tuft calcification- almost pathonogmonic, only other ddx is psoriatic arthritis
can also have oxylosis of retina

Question 4

cholesterol crystals

Answer 4

waxy, oily, flimsy crystals seen in lipid laden joint effusions of RA and chronic infection

Question 5

hyperuricemia

Answer 5

marker for atherogenesis

monitor for CVD

Question 6

initial immune response to urate crystals

Answer 6

IgG coating -> neutros, monocytes, fibroblasts, etc

Question 7

later immune response to urate

Answer 7

apo-E coating inhibits immune reponse

Question 8

decreased renal excretion

Answer 8

90% of cases
impaired renal fnx
dehydration
acidosis
low dose salicylates
diuretics
pyraxinamide
cyclosporine
levodopa
ethambutol
nicotinic acid
hypothyroid

Question 9

increased urate production

Answer 9

10% of cases
ethanol
meyloproliferative disorders
ineffective erythropoiesis
widespread psoriasis
cytotoxic drugs
glycogen storage disease
G6PD deficiency
HGPRTase deficiency
increased PRPP synthetase

Question 10

gout polarization

Answer 10

negatively birefringent

Question 11

stage I gout

Answer 11

asymptomatic hyperuricemia

Question 12

stage II gout

Answer 12

acute intermittent arthritic attacks
progresses from acute attacks lasting 1-2 weeks to attacks lasting up to 2 months
intervals progress form 2-10/yr to every 2-3 weeks
progresses from 1-up to 5 joints

Question 13

stage III gout

Answer 13

chronic arthritis with acute exacerbations
tophi
constant pain

Question 14

pseudogout aka

Answer 14

calcium pyrophosphate deposition disease (CCPD)

Question 15

CCPD clinical presentation

Answer 15

inflammatory arthritis
incidence increases with age
rhomboid, weakly positively birefringment
CHONDROCALCINOSIS (50%, 80yrs) 
knee >hip, shoulder, wrist

Question 16

associated medical conditions of CCPD

Answer 16

hyperparathyroidism
hypothryrodism
hemochromatosis
wilsons disease
OA

Question 17

sjogren syndrome

Answer 17

keratoconjunctivits sicca (KCS)
mouth, eyes, lungs, vagina

Question 18

primary sjogren

Answer 18

occurs in isolation
predominately women 40-60
dry mucous membraes
exocrine gland dysfunction
RA and other autoAbs common

Question 19

secondary type

Answer 19

associated with other diseases: RA, SLE, primary biliary cirrhosis, scleraderma, polymyocitis, hashimotos, polyarteritis, interstitial pulmonary fibrosis

Question 20

Sjogrens Dx

Answer 20

must have 4 of the following:
autoimmune exocrinopathy
ocular symptoms
oral symptoms
ocular signs (schirmers or rose bengal)
characteristic histo
salivary involvement
autoAbs (RF, SS-A, SS-B)

Question 21

what staining pattern is most specific to sjogrens

Answer 21

nucleolar

Question 22

other labs for sjogrens

Answer 22

CBC: anemia, leukopenia, eocinophelia
hypergammaglobulinmeia
RA + 70%
ANA + 95%

Question 23

clinical presentations of sjorens

Answer 23

parotoid enlargement 
dental carries
infected stensons duct
slavary duct stone
sialectasis of submandibular gland

Question 24

sjogrens histo

Answer 24

infiltration of neutrophils

missing glands

Question 25

Sjogrens Tx

Answer 25

tx symptoms
artificial tears (methyl cellulose)
cyclosproin drops
oral hygiene and hydration
pliocarpine
cevimeline
watch for lymphoma