Crystal Disease (Told)

Question 1

Crystal Disease

- the sadistic six

Answer 1

Monosodium urate (MSU)

Calcium Pyrophosphate Dihydrate (CPPD)

Basic Calcium PO4 (hydroxyapatite)

Calcium Oxalate

Cholesterol Crystals

Monoclonal Proteins

Question 2

when do we think crystals?

Answer 2

If episodic or intermittent attacks, think crystals!

Question 3

Rare birds

Answer 3

cholesterol crystals
monoclonal proteins
calcium phosphate hydroxyapatite
calcium oxylate

Question 4

CALCIUM PO4 (HYDROXYAPATITE) DEPOSITION

Answer 4

CALCIFIC TENDONITIS

• MOST APATITE CRYSTALS
  DO NOT REACT TO PORALIZED
  LIGHT.

Question 5

CALCIUM OXYLATE CRYSTALS

Answer 5

OXYLOSIS OF THE SPINE AND HAND +
NEPHROLYTHIASIS

Nephrolithiasis

terminal tuft calcification

OXYLOSIS OF THE RETINA

Question 6

CHOLESTEROL CRYSTALS

Answer 6

LIQUID LIPID CRYSTALS IN THE JOINTS

LIPID CRYSTALS CAN BE 
SEEN IN LIPID LADEN JOINT 
EFFUSIONS OF  RHEUMATOID 
ARTHRITIS AND CHRONIC  
INFECTION.

POLARIZED LIGHT- look like a picasso painting

Question 7

MONOCLONAL PROTEINS

Answer 7

Multiple Myeloma can present with Plasma cell rich

joint effusions precluding the bone destruction.

Question 8

gout vs pseudo gout

Answer 8

negative– YIPA

positive- pseudogout, little rectangles (not needles)

Question 9

Hyperuricemia & Gout- Epidemiology

Answer 9

Increases with age & body mass
Risk increases with degree & duration of hyperuricemia
Middle-aged males
Incidence of gout in females approaches that in males after menopause
2 – 3% of adult male population

Question 10

Hyperuricemia as a Marker for Atherogenesis

Answer 10

Hyperuricemia (gout) should alert the clinician to an overall increased risk of CVD and especially in those patients with other risk factors for CV events.

Question 11

what we do with urate crystals

Answer 11

can be coated with IgG

or wraped in Apo-E like a weenie in a blanket

Both of these lead to responses by neutrophils, leukocytes, monocytes, fibroblasts, synoviocytes, renal cells

Question 12

increased uric acid comes from

Answer 12

increased production or decreased excretion

or increased excretion can lead to stones

Question 13

Pathogenesis of Hyperuricemia- decreased renal excretion

Answer 13

90% of Cases
Impaired renal function
Dehydration
Acidosis
Low dose salicylates
Diuretics
Pyrazinamide
Cyclosporine
Levodopa
Ethambutol
Nicotinic acid
Hypothyroidism

Question 14

Pathogenesis of Hyperuricemia: Increased Urate Production

Answer 14

10% of Cases
Ethanol
Myeloproliferative disorders
Ineffective erythropoiesis
Widespread psoriasis
Cytotoxic drugs
Glycogen storage disease
G6PD deficiency
HGPRTase deficiency
Increased PRPP synthetase activity

Question 15

Acute Gouty Arthritis

Answer 15

Abrupt onset & mono - articular
* Sudden changes in uric acid levels best predictor of an attack
Classically 1st attack “podagra”
Episodic Disease
Attacks polyarticular with time
Tophi indicative of chronic urate overload
Needle shaped negatively birefringent MSU crystals

Question 16

Stages- clinical course of classical gout

Answer 16

*** Stage I: asymptomatic hyperuricemia. no arthritis. (? independent risk of atherogenesis)

*** Stage II: acute intermittent arthritis

acute attacks 1-2 weeks, intervals 210 years, joints 1-2

progresses to 1-3 weeks, every 6 mos-2 years

progresses to 1week - 2 months every 2-3 wks/ 3-4 months, involving 4-5 joints

*** STAGE III: chronic arthritis with acute exacerbations
Tophi- bone and cartilage
continuous arthritis
acute attacks superimposed

Question 17

Indications for Urate Lowering

Answer 17

Tophacheous disease with erosions
Uric acid nephrolithiasis

Recurrent attacks despite prophylaxis
Prevent Acute cell lysis - e.g., chemo.

Allopurinol contraindicated in acute gout but do not stop during an attack

Asymptomatic hyperuricemia ???
Allopurinol hypersensitivity syndrome

Question 18

calcification of the cartilage is

Answer 18

pseudogout

Question 19

Management of Crystal Disease the Old Fashioned Way

Answer 19

Arthrocentesis
NSAIDs
Corticosteroids - p.o., i.v.
Colchicine - prophylaxis

Uric Acid Lowering Agents:

• Allopurinol, Febuxostat (Uloric ®), Uricosuric Agents
• Not during acute attacks!

Question 20

MEDICAL STRATAGY 2015

Answer 20

ACUTE TX:
NSAIDS
Corticosteroids
Colchicine
Biologics (IL-1 inhibitors)

PROPHYLACTIC TX:

• Xantine Oxidase Inhibitors (Allopurinol, or Fabuxostat)
• Uricosurics (Probenicid, Fenofibrate, Losartan)
• Uricase (Procloticase)

Question 21

Dr. Told’s Guide TREATING CRYSTAL DISEASES

Answer 21

Asymptomatic Hyperuricemia = no treatment but check diuretic use and ASA use and niacin use.

Acute Attack = NSAID’s Indomethacin still is the best. COX-2’s OK others are too slow.
CORTICOSTEROIDS= Good for poor renal function and Injectible use.

Uric Acid Lowering = Colchicine can be a “Crap Shoot” Allopurinal and Probenecid
and Fabuxostat are best bets.

Question 22

important note about managing gout

Answer 22

NEVER ,NEVER STOP
ALLOPURINAL

PATIENTS MAY CHOOSE TO
BE MAINTAINED ON
INTERMITTANT INDOMETHACIN

Question 23

So what about diet?…..

Answer 23

THE STRICTEST OF PURINE FREE DIETS WILL ONLY DECREASE
URIC ACID LEVELS 1 MG%. MEDICATION IS THE BEST Rx

highest things include meats, beans, raisins