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Rheum Key Points > Calcium Crystal Dz's > Flashcards

Calcium Crystal Dz's Flashcards

1
Q

Dysregulated chondrocyte differentiation to hypertrophy and inorganic pyrophosphate (PPi) metabolism are what?

A

Central in the pathogenesis of Calcium Pyrophosphate Dihydrate (CPPD) crystal deposition disease

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2
Q

Autosomal dominant familial CCPD crystal deposition disease has been linked to mutations in?

A

Multiple kindreds to certain mutations in ANKH, a gene encoding a PPi transporter

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3
Q

NLRP3 (cryopyrin) inflammasome activation and consequent caspase-1 activation and IL1beta processing and secretion do what?

A

Drive cell responses to CPPD crystals and CPPD crystal-induced inflammation

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4
Q

Degenerative arthropathy caused by CPPD crystal deposition disease often involves what?

A

Joints uncommonly affected by primary osteoarthritis such as the metacarpophalangeal, wrist, and elbow

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5
Q

Diagnosis of CPPD deposition dz before age 55, particularly if CPPD deposition is polyarticular, should prompt what?

A

Differential dx consideration of a primary metabolic of familial disorder, and hyperparthyroidism should always be considered in CPPD deposition disease presenting inpatients older than 55

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6
Q

High resolution ultrasound appears partocularly helpful in dx of CPPD CDD because?

A

Radiographic chondrocalcinosis is detectable in all joints affected by the dz

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7
Q

Basic calcium phosphate (BCP) crystal deposition in articular cartilage is linked with?

A

Osteoarthritis, particularly with osteoarthritis of increased severity

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8
Q

BCP crystals (unlike urate and CPPD) do not demonstrate what? What does this mean?

A

Birefringence; therefore specialized methods are required to conclusively identify BCP crystals in specimens from the joint

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9
Q

The vast majority of CPPD CDD is?

A

Idiopathic/sporatic but early onset familial also occurs

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10
Q

Linkage of familial CPPD CDD to what gene on what chromosome is well established?

A

ANKH gene on chromosome 5p; encodes transmembrane protein with functions including PPi transport)

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11
Q

The loose avascular connective tissue matrices of articular hyaline cartilage, fibrocartilaginous menisci, and of certain ligaments and tendons are susceptible to what?

A

Pathologic calcification

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12
Q

Joint cartilage pathologic calcification reflect complex interplay between what things?

A

Organic and inorganic biochem of Pi and PPi metabolism, aging, dysregulated chondrocyte growth factor responsiveness and differentiation, and other factors

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13
Q

In the elderly, CPPD deposition can mimic what other conditions?

A

Gout, infectious arthritis, primary osteoarthritis, TA, or polymyalgia rheumatica, it can also present as fever of unknown origin

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14
Q

Pseudogout is a major cause of acute monoarticular or oligoarticular arthritis in the elderly, what do attacks usually involve?

A

A large joint, most often the knee, and less often the wrist or ankle and unlike gout it rarely affects the first metatarsophalangeal joint

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15
Q

Chronic degenerative arthropathy in CPPD deposition disease commonly affects certain joints that are typically what? (compared to OA)

A

Spared in OA, like the metacarpophalangeal, wrists, elbow, and glenohumeral

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16
Q

Unlike urate and CPPD CDD acute synovitis due to HA crystal deposition is unusual and how can it present? How is it described in young women?

A

Acute inflammatory syndromes including subacromial bursitis and a form of pseudopodagra described in young women may occur in association with periarticular HA crystal deposition in bursae, tendons, ligaments, and soft tissues

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17
Q

Patients with advanced chronic renal failure, particularly on dialysis, may develop what? What could they be associated with

A

Symptomatic articular and periarticular BCP crystal deposition, which may be destructive to the skeleton, they may resemble or be associated with CPPD CDD.

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18
Q

Presence of radiographic evidence for chondrocalcinosis is a common finding in what group of people, what does not necessarily indicate?

A

In the aged, it does not necessarily indicate that the patient’s symptomatic articular problem is due to CPPD deposition

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19
Q

The use of compensated polarized light microscopy is essential for CPPD, why?

A

Confirm the presence of the birefringent CPPD crystals, though it should be noted that some CPPD crystals are NOT birefringent

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20
Q

Patients with arthritis in whom CPPD CDD is part of the DDx can be screened how?

A

By plain radiographs, but high resolution ultrasound of the affected joint is a useful and sensitive approach

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21
Q

CPPD deposition disease treatment involves what?

A

Alleviation and prophylaxis of acute arthritic attacks, but therapy to lessen chronic and anatomically progressive sequelae of crystal deposition is not well developed for CPPD disease

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22
Q

The approach to pseudogout treatment is similar to what?

A

Treatment of normal gout

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23
Q

Uric acid is the biologically active end product of what?

A

Human Purine metabolism

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24
Q

Serum urate concentrations are determined by what?

A

Balance between urate production and elimination; hyperuricemia = urate overproduction or underexcretion or both

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25
Q

What has been identified as playing a central role in excretion of urate by the kidney?

A

Organic Anion Transporters (OATs)

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26
Q

Hyperuricemia is defined as serum urate levels greater than?

A

6.8 mg/dL, this is the soluble limit

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27
Q

Gout pathogenesis requires the accumulation of monosodium urate at levels sufficient to drive the precipitation of crystals which does what?

A

Initiation of inflammatory response

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28
Q

Monosodium urate crystals activate what? This is a multimolecular cytosolic complex that processes and generates what?

A

Activates NLRP3 (NALP3) inflammasome; generates IL-1beta, IL18 and IL33

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29
Q

The initiation of gouty inflammation by local white blood cells induces and influx of what into the joint?

A

Neutrophils

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30
Q

After the neutrophils get into the joint in gout, what happens?

A

They encounter urate crystals and become activated and propagate further inflammation

31
Q

Low level inflammation is present in chronic gout and tophaceous gout, what role do macrophages play?

A

They continue to produce cytokines and proteases, thereby facilitating cartilage and bone destruction

32
Q

Hyperuricemia is defined as?

A

Serum urate > 6.8mg/dL

33
Q

Acute gout is treated with?

A

NSAID, colchicine, corticosteroids, or adernocorticotrophic hormone; effectiveness of tx depends on how quickly therapy is initiated

34
Q

Before starting a specific urate-lowering agent, what should be tried?

A

Low dose colchicine or an NSAID in an attempt to prevent further attacks

35
Q

Regardless of whether an XO inh, a uricosic agent, or a uricase is used to treat hyperuricemia, the patient should receive what?

A

The lowest dose possible that maintains serum urate below 6.8, preferably below 6

36
Q

In addition to allopurinol, what 2 drugs are available are urate lowering agents for the treatment of gout?

A

Febuxostat and Pegloticase

37
Q

Individuals who are hyperuricemic should be screened for what?

A

HTN, CAD, diabetes, obesity, and alcoholism

38
Q

Using a specific urate-lowering agent to manage asymptomatic hyperuricemia is note recommended, however what should be managed?

A

Associated condictions such as HTN, CAD, diabetes, obesity, and alcoholism should be managed in these patients, as well as those with symptomatic gout

39
Q

Hyperuricemia is common and directly associated with what?

A

Serum creatinine, BMI, age, BP, and alcohol intake

40
Q

Serum urate levels are low in childhood and increase when in men and when in women?

A

In puberty in men and menopause in women

41
Q

The prevalence of gout ranged from 1-15% in populations with a clear increase in incidence in recent years, why is this?

A

Perhaps due to the assumption of a western diet and obesity epidemic

42
Q

Many environmental factors are associated with gout such as?

A

Alcohol (beer), and diet

43
Q

What foods promote hyperuricemia and gout?

A

Alochol, seafood, red meat

44
Q

Consumption of some foods may be protective, such as?

A

Milk and yogurt

45
Q

Rare forms of early hyperuricemia and gout have what basis?

A

Metabolic and genetic

46
Q

Gout often runs in families, probably because of inherited factors that do what?

A

Affect serum urate levels through renal clearance

47
Q

Recent genome wide association studies have identified polymorphisms in several candidate genes that encode what? What will this tell us?

A

Urate transporters in the renal prox tubule as determinants of serum urate levels and risk of gout

48
Q

Three stages of gout are?

A

Asymptomatic hyperuricemia, acute and intercritical gout, and chronic gouty arthritis

49
Q

A period of asymptomatic hyperuricemia lasts up to how long and then what happens?

A

20 years before the initial attack of gout or nephrolithiasis

50
Q

The first attack of gout generally occurs for when in men and when in women?

A

40-60 for men, 60 for women

51
Q

What class of drugs raises serum urate levels?

A

Diuretics, and many more

52
Q

Most attacks of gout, especially early in the course are monoarticular, which joint do they go after first?

A

The first metatarsophalangeal joint (podagra) and have a characteristic abrupt and painful onset

53
Q

The DDx for acute gout is usually what?

A

Infectious arthritis or other crystal induced synovitis, particularly pseudogout

54
Q

Ultrasonography is? (in terms of diagnosis)

A

A useful adjunct in the dx of acute and chronic gout

55
Q

In untreated or undertreated individuals chronic gout is characterized by?

A

Development of tophi and progressive joint damage

56
Q

Gout is associated with?

A

Obesity, hypertriglyceridemia, glucose intol, metabolic syndrome, HTN, atherosclerosis, and hypothyroidism

57
Q

What is frequently also associated with hyperuricemia and gout?

A

Renal insufficiency

58
Q

Hyperuricemia is a common cause of what?

A

Nephrolithiasis and rarely chronic hyperuricemia may cause urate nephropathy and acute hyperuricemia may lead to uric acid nephropathy in tumor lysis syndrome.

59
Q

Alcohol use, lead into, and cyclosporine can lead to what?

A

Hyperuricemia and gout

60
Q

Dx of gout should prompt what?

A

Search for the coexistence of associated conditions

61
Q

Primary hyperuricemia and gout are caused by what in 90% and what in 10%?

A

Decreased renal uric acid excretion; overproduction of urate

62
Q

2ndary hyperuricemia and gout are usually related to decreased what?

A

Renal urate clearance as a direct or indirect consequence of the primary disease process

63
Q

4 known specific inborn errors of purine metabolism with overproduction of urate account for what % of cases?

A

less than 1

64
Q

Asymptomatic hypeuricemia is generally not treated but identification should make you do what?

A

Search for the cause and/or associated conditions

65
Q

Episodes of acute gouty arthritis can be treated with?

A

Colchicine, NSAIDs, adrenocorticotropic hormone and systemic or intra-articular steroids

66
Q

Prophylaxis against acute attacks with colchicine or NSAID can be effect but what doesnt change?

A

The underlying process in the absence of concaminant urate lowering therapy

67
Q

Starting urate lowering therapy after a single attack of gout remains debatable, what are the indications for urate lowering therapy?

A

Recurrent gout, urate nephrolithiasis, tophaceous gout, and/or evidence of gout-induced joint damage

68
Q

XO inhibitors and uricosurics are effective at?

A

Lowering the amount of serum urate in most patients

69
Q

Uricases such as pegloticase should be reserved for?

A

refractory tophaceous gout

70
Q

What serum level of urate is targeted in tx?

A

Less than 6

71
Q

What is prophylactic treatment and for how long?

A

Colchicine, NSAID, or (less pref) systemic steroids for at least 6 months after initiation of urate lowering therapy

72
Q

Is long term compliance a problem in recurrence in gout?

A

Yes; forming a therapeutic alliance with the patient is critical

73
Q

What else can help control gout besides medication?

A

Lifestyle alterations like reduced alcohol consumption but this is more important for management of associated conditions such as obesity and hyperlipidemia

Rheum Key Points (7 decks)

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