Rheumatology: Crystal Arthropathies Flashcards

1
Q

The _____ are a group of diseases characterized by tissue deposition of monosodium urate (MSU) crystals due to hyperuricemia.

A

crystal arthropathies

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2
Q

Name the 4 manifestations of crystal arthopathies.

A
  1. gouty arthritis
  2. tophi (deposits of MSU)
  3. gouty nephropathy
  4. uric acid nephrolithiasis (kidney stones)
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3
Q

____ usually refers to acute or chronic arthritis 2a to MSU crystals.

A

Gout

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4
Q

Hyperuricemia without symptoms is referred to as _____, not gout.

A

asymptomatic hyperuricemia

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5
Q

What are the 4 stages of gouty arthritis?

A
  1. asymptomatic hyperuricemia
  2. acute gouty arthritis
  3. intercritical gout
  4. chronic tophaceous gout
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6
Q

What are the features of asymptomatic hyperuricemia?

A

elevated serum uric acid (>7.0mg/dL) w/o gouty arthritis, tophi, or uric acid nephrolithiasis

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7
Q

What are the features of acute gouty arthritis?

A
  • Acute onset of painful, warm, red, swollen joint, especially at night or morning.
  • MTP of great toe most common (podagra) but also can be insteps, ankles, heels, knees, wrists, fingers, and elbows.
  • Resolves after 3-10 days.
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8
Q

What is intercritical gout?

A

asymptomatic intervals between acute attacks of gout

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9
Q

What is chronic tophacous gout?

A
  • subcutaneous, synovial, or subchondral bone deposits of MSU crystals
  • commonly on digits of hands and feet, olecranon bursa, extensor surface of the forearm, Achilles tendon, or antihelix of the ear
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10
Q

Gout typically occurs in _____, age _____.

A

men; 30+ (peak incidence 50)

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11
Q

What other conditions are associated with gout?

A

EtOH abuse
obesity
insulin resistance
hypertension

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12
Q

How is gout definitively diagnosed?

A

synovial fluid evaluation

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13
Q

In gout, the intracellular crystals in the PMNs are _____-shaped and _____ when parallel to the axis.

A

needle; yellow

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14
Q

In gout, the synovial fluid is inflammatory (______-_____ leukocytes/mm3) with predominance of ______.

A

20,000-100,000; neutrophils

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15
Q

Hyperuricemia can result from ______ or _____ of urate.

A

increased production; decreased renal excretion

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16
Q

The majority of pts (90%) with 1a gout are _____ of uric acid.

A

underexcretors

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17
Q

Urinary uric acid excretion occurs thru a 4 compartment model:

A
  1. glomerular filtration
  2. pro-secretory filtration
  3. secretion back into the tubule
  4. post-secretory reabsorption
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18
Q

Net tubular reabsorption is about ____ of the filtered uric acid, therefore only ____ of the UA is excreted in the urine.

A

90%; 10%

19
Q

What is the urate/organic ion exchanger in the proximal tubule?

A

URAT1

20
Q

Certain drugs/metabolites decrease the renal excretion of uric acid by activating URAT1, including ____.

A

nicotinate, pyrazinoate, diuretics, and low-dose aspirin

21
Q

Certain drugs/metabolites increase the urinary excretion of UA by inhibiting URAT1, including _____.

A

probenecid, sulfinpyrazone, a metabolite of losartan, and high-dose aspirin

22
Q

Uric acid is a product of _____ metabolism bc humans lack the enzyme, _____.

A

purine; uricase

23
Q

In rare cases, overproduction of UA can be due to superactivity of _____ or deficiencies of ______.

A

PRPP; HGPRT

24
Q

Complete deficiency of HGPRT results in ______.

A

Lesch-Nyhan syndrome

25
Q

What are the features of Lesch-Nyhan Syndrome?

A

mental retardation, spasticity, choreoathetosis, and self-mutilation

26
Q

What factors affect urate solubility?

A
  1. temperature (cold)
  2. dehydration
  3. trauma
  4. pH (less soluble at low pH)
27
Q

Uric acid crystals interact with synovial lining cells leading to the activation of ____ and _____.

A

monocytes; mast cells

28
Q

How is acute gout treated/prevented?

A
weight loss
reduction of dietary purines
limitation of fructose and EtOH
NSAIDs
colchicine
corticosteroids
29
Q

How is chronic gout treated?

A
  • uricosuric (probenecid)

- xanthine oxidase inhibitor (allopurinol, febuxostat)

30
Q

What does CPDD stand for?

A

calcium pyrophosphate dihydrate deposition disease

31
Q

CDDD is a type of arthritis associated with the release of ______ crystals into the joint space and chondrocalcinosis.

A

calcium pyrophosphate dehydrate (CPPD)

32
Q

_____ is the term used to describe an acute episodic arthritis due to CPPD crystals.

A

Pseudogout

33
Q

Psuedogout attacks are characterized by?

A

sudden onset pain, swelling, warmth, and redness of a large joint, most often the knee

34
Q

Unlike gout, in pseudogout the ____ joint is rarely involved.

A

first MTP

35
Q

CPDD is a disease of the ______, with associations to ____ and _____ disease.

A

elderly; hyperparathyroidism, hemachromatosis

36
Q

What are risk factors for CPDD?

A

osteoarthritis, trauma, surgery

37
Q

In CPDD, the synovial fluid will have _____-shaped crystals and will be _____ when parallel to the red compensator light.

A

rhomboid; blue

38
Q

In CPDD, the synovial fluid will be inflammatory (____-____ leukocytes/mm3) with predominately _____.

A

2000-80,000; neutrophils

39
Q

What does PPi stand for?

A

pyrophosphate

40
Q

The source of PPi is from the metabolism of ______, particularly from articular chondrocytes.

A

nucleoside triphosphates (NTP)

41
Q

What gene can be mutated in CPDD?

A

ank gene (ANKH)

42
Q

CPDD crystals do not form spontaneously in the synovium- they are released by the _______ of pre-formed crystals in the cartilage matrix.

A

“shedding phenomenon”

43
Q

What is the treatment of CPDD?

A

anti-inflammatory drugs similar to gout