SM 235a - Crystalline Diseases Flashcards

1
Q

Which drugs are associated with increased risk of gout?

A
  • Thiazides
  • Cyclosporie
  • Low-dose aminosalicylates
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What kind of crystals are these?

A

Pseudogout

Rhoboid shaped, weakly positively birefringent

(Blue when parallel to the polarizer = positive biregringence)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Describe the synovial fluid in BCP crystal arthritis

A

Not inflammatory

(<2000 WBCs)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Describe the microscopic appearance of CPPD crystals

A

Rhomboid shaped

Weakly positively birefringent = Blue when parallel to the polarizer

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Which crystaline disease does not have inflammatory characteristics?

A

BCP arthropathy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What conditions cause overproduction of uric acid?

What are the consequences?

A
  • Alcohol use
  • Enzyme abnormality (disease onset will be early)
  • Hemolytic disease
  • Myeloproliferative disease

-> increased uric acid -> GOUT

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Serum ____________ concentration is associated with gout

A

Serum uric acid concentration is associated with gout

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What agents are used for the chronic management of gout?

A
  • Xanthine oxidase inhibitors
    • Decrease production of urate
    • Allopurinol (*Hypersensitivity rxn risk*)
    • Febuxostat
  • Uricosuric agents
    • Increase excretion of urate
    • Probenecid (*Kidney stone risk*)
    • Lesinurad
  • Pegloticase
    • IV Uricase
    • Converts uric acid into allantoin
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What are the risk factors for developing gout?

A
  • Increased alcohol consumption
    • Increased ATP degradation = increased urate synthesis
    • Increased lactic acid = urate excretion
    • Beer has the highest purine content of the alcohols
  • Obesity
  • Metabolic syndrome
  • Insulin resistance
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What are the treatments for acute gout?

A
  • Colchicine
  • NSAIDs
  • Intra-articular corticosteroids

Do not start new uric acid lowering treatment during an accute attack

Do not stop uric acid lowering treatment if already taking

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What are the 3 main crystal types that can be found in synovial fluid?

A
  • Basic calcium phosphate (BCP)
  • Calcium pyrophosphate dihydrate (CPPD)
  • Monosodium urate (gout)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What medications are used as prophylaxis for acute gout attacks?

A

NSAIDs, colchicine, steroids

These can also be used to alleviate pain during an acute attack

Long-term management agents are not prophylactic against acute attacks (although lowering uric acid levels on its own should be prophylactic?)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Which inborn errors of metabolism increase one’s susceptibility to gout?

A

Leschi-Nyhan

PRPP synthetase mutation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What kind of crystals cause gout?

A

Monosodium urate monohydrate

  • Negatively birefringent (yellow when parallel to the polarizer)
  • Needle shaped
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What medication increases the excretion of urate?

What condition can this help manage?

A

Uricosuric agents: Probenecid and Lesinurad

Used for chronic management of gout

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

BCP crystals deposition in soft tissue causes _________.

What are the consequences?

A

BCP crystals deposition in soft tissue causes acute calcific periarthritis.

-> intense local inflammation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What is the major risk factor for Calcium Pyrophosphate Dihydrate (CPPD) arthropathy?

A

Aging

Presentation peaks at 65-75 y/o; F>M

  • Tends to affect joints affected by osteoarthritis
18
Q

What foods increase one’s risk of gout?

A
  • Foods rich in purines
    • Purine degradations -> uric acid production
    • Meat, shellfish, ethanol, soft drinks, high-fuctose corn syrup
19
Q

Describe the clinical presentation of gout

A
  • Acute monoarthritis
    • Usually involves the 1st MTP
    • Any joint can be affected
  • Pain often begins in the early AM
    • Very acute
    • Unable to tolerate teh bedsheets touching the affected joint
  • First few attacks are usually self-limited
    • Resolve after 7-10 days without therapy
  • Subsequent attacks may be more freqent, severe, and resistant to therapy
    • If hyperuricemia persists, disease may become chronic and polyarticular
20
Q

This is an image of synovial fluid obtained from a patient with gout and shows negatively birefringent needle shaped crystals. Which way is the polarizer facing?

A

C

  • Needle-shaped crystals will be yellow when parallel to the polarizer
  • This means they are positively birefringent
  • Monosodium urate monohydrate crystals cause gout
21
Q

What are the indications for chronic gout therapy?

A

Begin chronic therapy if any of the following are present

  • > 2-3 gout attacks in 1-2 years
  • Renal stones
  • Tophaceous gout
  • Erosions on x-ray
22
Q

The metabolism of ______ creates uric acid.

This contributes to the pathologenesis of which crystaline disease?

A

The metabolism of purines creates uric acid.

Buildup of uric acid leads to gout

23
Q

What medication decreases the production of urate?

What condition can this help manage?

A

Xanthine oxidase inhibitors

Ex: Allopurinol, febuxostat

Used for chornic management of gout

24
Q

What medication is the enzyme uricase given intraveneously?

What condition does this medication treat?

A

Pegloticase

Converts urate into allantoin

Used for the chronic management of gout

25
Q

What are the indications for colchicine?

A

Acute management of CPPD and Gout

Prophylaxis againt acute gout attacks

26
Q

Where do basic calcium phosphate (BCP) crystals deposit?

What pathologies does this cause?

A

Remember the ABC’s of BCP

  • Soft tissue -> Acute calcific periarthritis
  • Joints -> BCP arthropathy
  • Tendons -> Calcific tendonitis
27
Q

BCP deposition in the tendons causes ________.

What are the consequences?

A

BCP deposition in the tendons causes Calcific Tendinitis.

  • Most commonly seen in the shoulder
  • Buritis, impingement syndrome
  • Can also involve other joints
    • Hand, wrist, hip, knee, foot, neck
28
Q

BCP crystal deposition in joints causes ____________.

What are the consequences?

A

BCP crystal deposition in joints causes BCP Arthropathy.

  • Shoulder -> Milwaukee shoulder syndrome
    • Severe degenerative arthritis of the shoulder joint
    • Loss of rotator cuff, cannot abduct arm, huge swelling
    • Bilateral involvement is common
  • Finger -> Philadelphia finger
    • Same thing but in the finger
  • Can affect other joints as well
29
Q

Describe the goals of management for an acute gout attack.

How are they achieved?

A
  • Rapid and safe pain relief: decrease inflammation
    • NSAIDs
    • Colchicine
    • Corticosteroids
  • Prevent further attacks
    • Xanthine oxidase inhibitors (Decrease urate production)
    • Uricosuric agents (Increase urate excretion)
    • Pegloticase (Convert uric acid into allantoin
    • Anakinra (Anti-IL1)
  • Prevent the formation of tophy and destructive arthritis: keep uric acid levels low
30
Q

What conditions cause underexcretion of uric acid?

What are the consequences?

A
  • Alcohol consumption
  • Drugs
  • Decreased renal function

-> hyperuricemia -> GOUT

31
Q

Describe the clinical presentation of CPPD

A

CPPD = pseudogout

  • Most often presents the same way gout does
  • Can also present as chronic polyarthritis (like RA), or progressive osteoarthritis
  • May be asymptomatic
32
Q

What are the 4 stages of Gout?

A
  • Asymptomatic hyperuricemia
  • Acute gouty arthritis
  • Intercritical gout
    • Quiescent intervals between gout attacks
  • Chronic tophaceous gout
    • After ~10 years of uncontrolled disease
33
Q

What kind of crystals are these?

Which disease are they associated with?

A

Monosodium urate monohydrate crystals

Gout

  • Needle-shaped
  • Yellow when parallel to the polarizer
34
Q

What is the treatment for CPPD?

A
  • NSAIDs
  • Intra-articular or oral steroids
  • Colchicine
  • There is no true prophylaxis; can only treat acute attacks
35
Q

What kind of crystals are these?

How do you know?

A

Basic Calcium Phosphate (BCP)

  • “Shiny coins” on ordinary light microscopy
  • Not birefringent on polarized light microscopy
  • Alizarin red stain
36
Q

Monosodium urate monohydrate crystals are associated with which disease?

A

Gout

37
Q

What characterizes all crystalline arthropathies?

A
  • Inflammation in the joint
    • Synovial fluid will have WBC cound 2000-50,000, just like other inflammatory joint conditions
    • Except BCP crystals - not inflammatory
  • Crystals in the synovial fluid
38
Q

What causes gout?

A

Hyperuricemia

  • Underexcretion (most common cause)
    • Alcohol use
    • Decreased renal function
    • Drugs
      • Diuretics (thiazides), cyclosporine, ethambutol, ASA
  • Overproduction
    • Alcohol use
    • Enzyme abnormality (disease onset will be early)
    • Hemolytic disease
    • Myeloproliferative disease
39
Q

Why do men <60 years old have a higher prevalence of gout than women <60 years old?

A

Uric acid levels start to increase in men at puberty

  • Estrogen is uricosuric => it is protective against gout
  • Prevalence in males and females equalizes post-menopause
40
Q

What is the treatment for BCP arthropathy?

A

Treat if symptomatic

  • NSAIDs
  • Physical Therapy
  • Intra-articular steroids
  • Surgery if indicated