Crystal Disease (Told) - MT Flashcards
There are 6 types of Crystals that Dr. Told mentioned can cause disease, what are they?
- Monosodium urate (MSU)
- Calcium pyrohosphate dihydrate (CPPD)
- Basic Calcium PO4 (hydroxyapatite)
- Calcium oxalate
- Monoclonal proteins
- Cholesterol crystals
…..Sadistic 6
What type of crystals can deposit in the retina and caue visual field defects and blindness?
- Calcium oxylate crystals
Which is the most common rare crystal type to cause disease?
Calcium phosphate hydroxyapatite
followed by….
calcium oxylate
Cholesterol crystals
monoclonal proteins
Calcium PO4 (hydroxy apatite) deposition causes what?
Do most of these crystals react to polarized light?
calcific tendonitis
no
Calcium oxylate crystals can cause ______ in the spine and hands, and ______ in the kidneys.
- terminal tuft calcification
- nephrolythiasis (stones)
What kind of crystals can be seen in both joint effusions of rheumatoid arthirtis and chronic infection?
Cholesterol lipid crystals
In multiple myeloma, what is the classification of the antibody that is found in the plasma cell rich joint effusions that mediate bone destruction?
S100A4 antibodies
- Describe what you would observe on a birefringent light test in a person with Gout vs. Pseudogout.
- What type of crystals are found in these respective diseases?
- Gout:
Monosodium urate crystals
negative birefringent test
(vertical in plane yellow needles, horizontal blue needles)
- Pseudo-gout:
Calcium pyrophosphate crystals
positive birefringent test
(vertical in plane blue squares, horizontal yellow bodies)
Hyperuricemia and gout appears in about 2-3% of adult male population and incidence in females approach males after menopause. Describe weight and age of the people at risk for or who have this condition.
- obese, increased risk with increased weight
- middle aged males, also increased risk with increased age
With the presence of hyperuricemia, a physician should concurrently invsestigae for what other disease/pathology?
- Cardiovascular disease
(hyperuricemia is a marker for atherogenesis)
especially in patients with other risk factors for Cardiovascular events
Describe the cause of inflammatioon in hyperuricemia.
- urate crystals are coated with IgG antibodies,
***which attract neutrophils, other leukocytes, monocytes, fibroblasts, synoviocytes, renal cells.
All of these cells release cytokines, PGE2, oxygen radicals, and collagenase which cause inflammation
In the pathogenesis of hyperuricemia, what is the most common cause of hyperuricemia? Increased urate production or decreased renal excretion?
90% decreased renal excretion
10% increased urate production
an endocrine disorder that can decrease renal excretion and cause hyperuricemia is?
Hypothyroidism
What substance in a social hx can be abused and cause increased urate production?
ethanol, alcohol
*basically any disease, drug, or process that causes excess cell destruction can cause an increase in urate production and lead to hyperuricemia (10% of the time)
If a patient has a crystal disease, how will he/she most likely describe the timeframe of the pain?
intermittent, episodic attacks
A patient comes in complaining of new onset joint pain in his 1st right metatarsaphalangeal joint, which occurs for a few hours every week since a month ago. He has a history of HTN and hyperlipidemia, and is on a diuretic and statin drug. His birefringent test is negative.
- What is your diagnosis?
- What treatment is most appropriate at this time?
- Acute Gouty arthritis
- NSAIDs or Corticosteroid for acute pain relief, Allopurinol for long term prevention.
-
What would you see in a patient with chronic gouty arthritis? x2
- what is the stage given to this type of gout?
- Tophi
- Polyarticular attacks
- stage III
* acute attacks superimposed
What is a tophi?
deposits of monosodium urate crystals in muscle, cartilage, and bone that appear in stage III gout.
4 indications for urate lowering medication include?
- tophaceous disease with erosions
- uric acid nephrolithiasis
- recurrent attacks depsite prophylaxis
- Chemotherapy (acute cell lysis)
A 80 y/o patient presents with inflammatory arthritis in her left knee, she also has chondrocalcinosis, and is being treated for hyperparathyroidism.
- What is her diagnosis?
- How would you diagnose?
- calcium pyrophosphate depositin disease (CPPD)
- birefringent test, which would be weakly positive for blue rhomboid crystals (pseudogout)
in addition to hyperparathyroidism, what other medical cnoditions are associated with CPPD? x4
- hypothyroidism
- hemochromatosis
- wilson’s disease
- Osteoarthritis
Management of acute crystal disease would include? x4
- NSAIDs (indomethacin)
- Corticosteroids
- colchicine
- IL-1 biologic inhibitor
prophylactic tx of crystal diseases include what 3 categries of pharmaceuticals?
- xantihine oxidae inhibitors like allopurinol and fabuxostat
- Uricosurics (probenicid, losartan)
- Uricases (procloticase)
How would Dr. Told treat each of the respective patients?:
- guy with asymptomatic hyperuricemia
- gal presenting with acute attacks of gout
- prophylaxis of dude with gout
- no treatment but check diuretic use and ASA use and niacin use
- NSAIDs (indomethacin the best), or Corticosteroids if renal function poor
- Allopurinol or fabuxostat or probenecid over skethcy colchine
In a gouty acute attack should, a patient stop taking allopurinol if they are already on it for prophylaxis?
no
Strict purine free diet will decrease uric acid levels by about?
1 MG%
* medications are the best option
Primary sjogren syndrome occurs predominately in women age ________. Presents with dry eyes, mouth, and __________. Has aspects of __________ gland dysfunction. RA and other autoantibodies common.
- 40-60
- respiratory tract
- exocrine
Secondary type sjogren syndrome is associated with a bunch of autoimmune diseases. x9
other rheumatic diseases
- RA, SLE, PBC, scleroderma, polymyocitis, Hashimotos thyroiditis, polyarteritis, Interstitial pulmonary fibrosis
What are the 6 preliminary criteria of classification of sjogren syndrome?
- how many have to be present to have a sensitivity of 93.5% and a specificity of 94%?
- autoimmune exocrinopathy
- ocular sx
- oral sx
- ocular signs (schirmer’s/rose bengal)
- salivary gland involvement
- autoantibodies (RF, SS-A, SS-B)
- 4/6
What are the 3 types of auto antibodies found in 49% of patients with SLE and 75% of patients with Sjogren syndrome?
- anti Ro60 SSA
- anti Ro52 SSA
- Anti LA SSB
CBC of a person with sjogrens would show?
Anemia, leukopenia, eosinophelia
A patient with Sjogren would be positive for RA _____% of the time, and positive for ANA ______% of the time.
- RA 70%
- ANA 95%
leading 3 sx manifestation of sjogren syndrome are
- Fatigue 80%
- Articular involvement 50%
- Tendomyalgia 40%
What GI nd Urogenital sx can also present in Sjogrens?
GI - esophageal involvement, autoimmune hepatitis
GU - Bladder involvement
in addition to dental caries, saliary duct stones, and infected stensons duct, _______ gland enlargement can present in sjogren syndrome.
- Parotid
sialogram of a submandibular gland would show ______ in a patient with sjogren syndrome.
- sialectasis
Lip gland histopathology would show what in a patient with sjogren syndrome?
Lymphocytic infiltration with glandular and ductal atrophy
SICCA symptoms of sjogren syndromme include what 3 sx?
- dry oral mucosa
- dry vaginal mucosa
- dry conjunctiva
describe a Schirmer test in a patient with sjogren syndrome
wetting of filter paper strip takes longer and indicates reduced lacrimal secretions
a positive Rose bengal staining test would indicate?
inflammation and irritation of conjunctival layer
Low trachiobronchial secretions in sjogren syndrome lead to ____________.
nonspecific interstitial pneumonia (NSIP)
Tx of sjogren syndrome focuses on?
symptomatic treatment
treatment for sjogren syndrome can include? x4
- artificial tears methyl cellulose
- cyclosporin drops
- pilocarpine
- cevimeline
What should be monitored in a patient with sjogren syndrome? x3
- oral hygiene
- hydration
- careful watch for lymphoma
