calcium crystal disease HY

Question 1

risk factor for development of pseudogout and calcium pyrophosphate dihydrate (CPPD) deposition

Answer 1

age

Question 2

4 conditions associated with CCPD

Answer 2

hyperparathyroidism, hemochromatosis, chronic hypomagnesemia, and hypophosphatasia

Question 3

what CCPD crystals look like under polarized light?

Answer 3

and rhomboid shapes and are positively birefringent

Question 4

Diagnosis of CPPD deposition disease before age 55, particularly if CPPD deposition is polyarticular, should prompt differential diagnostic consideration of ____________and_____________, and _______________ should always be considered in CPPD deposition disease presenting in patients older than the age of 55.

Answer 4

Diagnosis of CPPD deposition disease before age 55, particularly if CPPD deposition is polyarticular, should prompt differential diagnostic consideration of a primary metabolic or familial disorder, and hyperparathyroidism should always be considered in CPPD deposition disease presenting in patients older than the age of 55.

Question 5

_______________inflammasome activation and consequent caspase-1 activation and interleukin (IL)-1β processing and secretion drive cell responses to CPPD crystals and CPPD crystal-induced inflammation.

Answer 5

NLRP3 (cryopyrin) inflammasome activation and consequent caspase-1 activation and interleukin (IL)-1β processing and secretion drive cell responses to CPPD crystals and CPPD crystal-induced inflammation.

Question 6

Dysregulated _____________ differentiation to hypertrophy and inorganic pyrophosphate (PPi) metabolism are central in pathogenesis of calcium pyrophosphate dihydrate (CPPD) crystal deposition disease.

Answer 6

Dysregulated chondrocyte differentiation to hypertrophy and inorganic pyrophosphate (PPi) metabolism are central in pathogenesis of calcium pyrophosphate dihydrate (CPPD) crystal deposition disease.

Question 7

Autosomal _____________familial CPPD crystal deposition disease has been linked in multiple kindreds to certain mutations in ____________, a gene encoding a PPi transporter.

Answer 7

Autosomal dominant familial CPPD crystal deposition disease has been linked in multiple kindreds to certain mutations in ANKH, a gene encoding a PPi transporter.

Question 8

what to use to diagnose CCPD?

Answer 8

High-resolution ultrasound

Question 9

_____________________ crystal deposition in articular cartilage is intimately linked with osteoarthritis, particularly with osteoarthritis of increased severity.

Answer 9

Basic calcium phosphate (BCP) crystal deposition in articular cartilage is intimately linked with osteoarthritis, particularly with osteoarthritis of increased severity.

Question 10

___________(unlike urate and CPPD crystals) do not demonstrate birefringence, and specialized methods are required to conclusively identify BCP crystals in specimens from the joint.

Answer 10

BCP crystals (unlike urate and CPPD crystals) do not demonstrate birefringence, and specialized methods are required to conclusively identify BCP crystals in specimens from the joint.

Question 11

The vast majority of CPPD crystal deposition disease is _____________, but early-onset familial disease also occurs.

Answer 11

The vast majority of CPPD crystal deposition disease is idiopathic/sporadic, but early-onset familial disease also occurs.

Question 12

Linkage of familial CPPD crystal deposition disease to the gene ___________on chromosome _________ (which encodes a transmembrane protein with functions including PPi transport) is well established.

Answer 12

Linkage of familial CPPD crystal deposition disease to the gene ANKH on chromosome 5p (which encodes a transmembrane protein with functions including PPi transport) is well established.

Question 13

In the elderly, what can CCPD micim? what can it present as?

Answer 13

mimics:gout, infectious arthritis, primary osteoarthritis, RA, or polymyalgia rheumatica

can present as fever of unknown origin.

Question 14

Pseudogout is a major cause of acute monoarticular or oligoarticular arthritis in the elderly; attacks typically involve a large joint, most often the __________, and less often the wrist or ankle, and, unlike gout, rarely the first ________________.

Answer 14

Pseudogout is a major cause of acute monoarticular or oligoarticular arthritis in the elderly; attacks typically involve a large joint, most often the knee, and less often the wrist or ankle, and, unlike gout, rarely the first metatarsophalangeal joint.

Question 15

Chronic degenerative arthropathy in CPPD deposition disease commonly affects certain joints that are typically spared in _______ (e.g., metacarpophalangeal joints, wrists, elbows, glenohumeral joints).

Answer 15

Chronic degenerative arthropathy in CPPD deposition disease commonly affects certain joints that are typically spared in primary OA (e.g., metacarpophalangeal joints, wrists, elbows, glenohumeral joints).

Question 16

how does calcium pryophosphate deposit in joints?

Answer 16

linear calcification

Question 17

Unlike urate and CPPD crystal deposition, acute synovitis due to ________ crystal deposition is unusual

Answer 17

Unlike urate and CPPD crystal deposition, acute synovitis due to hydroxyapatite crystal deposition is unusual

Question 18

what can patients with chronic renal failure develop in terms of BCP crystal deposition?

Answer 18

Patients with advanced chronic renal failure, particularly on dialysis, may develop symptomatic articular and periarticular BCP crystal deposition, which may be destructive and involve the axial skeleton. They may resemble or be associated with CPPD deposition disease.

Question 19

Presence of radiographic evidence for chondrocalcinosis is a ________finding in the aged and does not necessarily indicate that the patient’s symptomatic articular problem is due to CPPD deposition disease, which is often asymptomatic.

Answer 19

Presence of radiographic evidence for chondrocalcinosis is a common finding in the aged and does not necessarily indicate that the patient’s symptomatic articular problem is due to CPPD deposition disease, which is often asymptomatic.

Question 20

The use of compensated polarized light microscopy is essential to confirm the presence of________** birefringent** CPPD crystals, though it should be noted that some CPPD crystals are nonbirefringent.

Answer 20

The use of compensated polarized light microscopy is essential to confirm the presence of positively birefringent CPPD crystals, though it should be noted that some CPPD crystals are nonbirefringent.

Question 21

Patients with arthritis in whom CPPD deposition disease is part of the differential diagnosis can be screened by_______.

Answer 21

plain radiograph

high-resolution ultrasound (more sensitive approach)

Question 22

what does tx for CCPD deposition disease include? is prophylaxis well established?

Answer 22

CPPD deposition disease treatment involves 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

Question 23

what is the approach of tx of pseudogout similar to?

Answer 23

The approach to pseudogout treatment is similar to that for acute gout.

Question 24

which treatments have shown proven benefit in the tx of CCPD?

Answer 24

NSAIDs or COX-2 inhibitors
Intra-articular corticosteroids
Systemic corticosteroids
ACTH
Prophylactic low-dose colchicine