Calcium Crystal Dz's Flashcards
(73 cards)
Dysregulated chondrocyte differentiation to hypertrophy and inorganic pyrophosphate (PPi) metabolism are what?
Central in the pathogenesis of Calcium Pyrophosphate Dihydrate (CPPD) crystal deposition disease
Autosomal dominant familial CCPD crystal deposition disease has been linked to mutations in?
Multiple kindreds to certain mutations in ANKH, a gene encoding a PPi transporter
NLRP3 (cryopyrin) inflammasome activation and consequent caspase-1 activation and IL1beta processing and secretion do what?
Drive cell responses to CPPD crystals and CPPD crystal-induced inflammation
Degenerative arthropathy caused by CPPD crystal deposition disease often involves what?
Joints uncommonly affected by primary osteoarthritis such as the metacarpophalangeal, wrist, and elbow
Diagnosis of CPPD deposition dz before age 55, particularly if CPPD deposition is polyarticular, should prompt what?
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
High resolution ultrasound appears partocularly helpful in dx of CPPD CDD because?
Radiographic chondrocalcinosis is detectable in all joints affected by the dz
Basic calcium phosphate (BCP) crystal deposition in articular cartilage is linked with?
Osteoarthritis, particularly with osteoarthritis of increased severity
BCP crystals (unlike urate and CPPD) do not demonstrate what? What does this mean?
Birefringence; therefore specialized methods are required to conclusively identify BCP crystals in specimens from the joint
The vast majority of CPPD CDD is?
Idiopathic/sporatic but early onset familial also occurs
Linkage of familial CPPD CDD to what gene on what chromosome is well established?
ANKH gene on chromosome 5p; encodes transmembrane protein with functions including PPi transport)
The loose avascular connective tissue matrices of articular hyaline cartilage, fibrocartilaginous menisci, and of certain ligaments and tendons are susceptible to what?
Pathologic calcification
Joint cartilage pathologic calcification reflect complex interplay between what things?
Organic and inorganic biochem of Pi and PPi metabolism, aging, dysregulated chondrocyte growth factor responsiveness and differentiation, and other factors
In the elderly, CPPD deposition can mimic what other conditions?
Gout, infectious arthritis, primary osteoarthritis, TA, or polymyalgia rheumatica, it can also present as fever of unknown origin
Pseudogout is a major cause of acute monoarticular or oligoarticular arthritis in the elderly, what do attacks usually involve?
A large joint, most often the knee, and less often the wrist or ankle and unlike gout it rarely affects the first metatarsophalangeal joint
Chronic degenerative arthropathy in CPPD deposition disease commonly affects certain joints that are typically what? (compared to OA)
Spared in OA, like the metacarpophalangeal, wrists, elbow, and glenohumeral
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?
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
Patients with advanced chronic renal failure, particularly on dialysis, may develop what? What could they be associated with
Symptomatic articular and periarticular BCP crystal deposition, which may be destructive to the skeleton, they may resemble or be associated with CPPD CDD.
Presence of radiographic evidence for chondrocalcinosis is a common finding in what group of people, what does not necessarily indicate?
In the aged, it does not necessarily indicate that the patient’s symptomatic articular problem is due to CPPD deposition
The use of compensated polarized light microscopy is essential for CPPD, why?
Confirm the presence of the birefringent CPPD crystals, though it should be noted that some CPPD crystals are NOT birefringent
Patients with arthritis in whom CPPD CDD is part of the DDx can be screened how?
By plain radiographs, but high resolution ultrasound of the affected joint is a useful and sensitive approach
CPPD deposition disease treatment involves what?
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
The approach to pseudogout treatment is similar to what?
Treatment of normal gout
Uric acid is the biologically active end product of what?
Human Purine metabolism
Serum urate concentrations are determined by what?
Balance between urate production and elimination; hyperuricemia = urate overproduction or underexcretion or both
