Calcium crystal disease

Question 1

What is central in pathogenesis of calcium pyrophosphate dihydrate crystal deposition (CPPD) disease ?

Answer 1

dysregulated chondrocyte differentiation to hypertrophy and inorganic pyrophosphate metabolism

Question 2

Autosomal dominant familial CPPD crystal deposition disease is linked to what?

Answer 2

mutations in ANKH => gene encoding PPi transporter

Question 3

What drives cell responses to CPPD crystals and CPPD crystal induced inflammation?

Answer 3

NLRP3 inflammasome activation and consequent caspase-1 activation and IL-1B processing and secretion

Question 4

How is degenerative arthropathy caused by CPPD crystal deposition different from primary osteoarthritis?

Answer 4

involves joints uncommonly affected from primary osteoarthritis like the MCP, wrist, and elbow joints

Question 5

If a Dx of CPPD deposition disease is before age 55 and polyarticular, what else should be on the DDx?

Answer 5

primary metabolic or familial disorder;

hyperparathyroidism (always considered in CPPD deposition) even if older than age 55

Question 6

What is helpful in diagnosis of CPPD crystal deposition disease? why?

Answer 6

high resolution ultrasound due to radiographic chondrocalcinosis not being detectable in all joints affected by disease

Question 7

What crystal deposition disease is linked with osteoarthritis?

Answer 7

basic calcium phosphate in articular cartilage causes OA to increase in severity

Question 8

What is the difference in BCP crystals and CPPD crystals when viewing them?

Answer 8

BCP has NO BIREFRINGENCE

CPPD and urate have birefringence

Question 9

What is the relationship of genetics and CPPD crystal deposition disease?

Answer 9

most are idiopathic/sporadic but early onset familial disease occurs

familial CPPD crystal deposition disease linked to ANKH on chromosome 5p

Question 10

In CPPD crystal deposition disease, what are particulary susceptible to pathologic calcification?

Answer 10

lose avascular connective tissue matrices of articular hyaline cartilage, fibrocartilaginous menisci; certain ligaments and tendons

Question 11

Grossly, how do CPPD crystals appear?

Answer 11

extensive foci of chalky white particulate deposits within articular cartilage

Question 12

Histologically, CPPD crystal appearance?

Answer 12

hypertrophic chondrocytes adjacent to crystal aggregates w/in enlarged chondrons

Question 13

Under polarized light micro, CPPD crystal appearance?

Answer 13

rod and rhomboid shapes that are positively birefringent

Question 14

What is a big difference in pseudogout and gout as far as manifestations?

Answer 14

gout=> MTP joint

pseudogout=> large joint (knee)

Question 15

T/F acute synovitis due to HA (hydroxyapatite) crystal deposition is unusual

Answer 15

true

Question 16

Acute inflammatory syndromes like subacromial bursitis and pseudopodagra may occur in association with what?

Answer 16

perarticular HA crystal deposition in bursae, tendons, ligaments and soft tissues

Question 17

Patients with advanced chronic renal failure may develop what?

Answer 17

symptomatic articular and periarticular BCP crystal depositions that may be destructive and involve the axial skeleton

Question 18

XRay reveals chondrocalcinosis in elderly person. What would you tell them?

Answer 18

it is common in elderly and does not necessarily indicate that CPPD crystal deposition disease is the cause of the symptomatic articular disease as it is often asymptomatic

Question 19

What should be used to confirm CPPD crystal deposition?

Answer 19

polarized light microscopy for positively birefringence (some CPPD crystals are non-birefringent)

Question 20

CPPD crystal deposition disease Tx is what?

Answer 20

alleviation and prophylaxis of acute arthritic attacks