crystal diseases Flashcards
crystal diseases
monosodium urate calcium pyrophophate dihydrate basic CaPO4 (hydroxyapatite) Ca oxalate cholesterol crystals monoclonal proteins
hydroxyapetite
CaPO4
calcific tendonitis
crystals also in urine
oxylosis of spine and hand + nephrolythiasis
Ca oxylate crystals
black in urine
terminal tuft calcification- almost pathonogmonic, only other ddx is psoriatic arthritis
can also have oxylosis of retina
cholesterol crystals
waxy, oily, flimsy crystals seen in lipid laden joint effusions of RA and chronic infection
hyperuricemia
marker for atherogenesis
monitor for CVD
initial immune response to urate crystals
IgG coating -> neutros, monocytes, fibroblasts, etc
later immune response to urate
apo-E coating inhibits immune reponse
decreased renal excretion
90% of cases impaired renal fnx dehydration acidosis low dose salicylates diuretics pyraxinamide cyclosporine levodopa ethambutol nicotinic acid hypothyroid
increased urate production
10% of cases ethanol meyloproliferative disorders ineffective erythropoiesis widespread psoriasis cytotoxic drugs glycogen storage disease G6PD deficiency HGPRTase deficiency increased PRPP synthetase
gout polarization
negatively birefringent
stage I gout
asymptomatic hyperuricemia
stage II gout
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
stage III gout
chronic arthritis with acute exacerbations
tophi
constant pain
pseudogout aka
calcium pyrophosphate deposition disease (CCPD)
CCPD clinical presentation
inflammatory arthritis incidence increases with age rhomboid, weakly positively birefringment CHONDROCALCINOSIS (50%, 80yrs) knee >hip, shoulder, wrist
associated medical conditions of CCPD
hyperparathyroidism hypothryrodism hemochromatosis wilsons disease OA
sjogren syndrome
keratoconjunctivits sicca (KCS) mouth, eyes, lungs, vagina
primary sjogren
occurs in isolation predominately women 40-60 dry mucous membraes exocrine gland dysfunction RA and other autoAbs common
secondary type
associated with other diseases: RA, SLE, primary biliary cirrhosis, scleraderma, polymyocitis, hashimotos, polyarteritis, interstitial pulmonary fibrosis
Sjogrens Dx
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)
what staining pattern is most specific to sjogrens
nucleolar
other labs for sjogrens
CBC: anemia, leukopenia, eocinophelia
hypergammaglobulinmeia
RA + 70%
ANA + 95%
clinical presentations of sjorens
parotoid enlargement dental carries infected stensons duct slavary duct stone sialectasis of submandibular gland
sjogrens histo
infiltration of neutrophils
missing glands
