Calcium Pyrophosphate Deposition Disease Flashcards
CPP in short
Deposition of CPP crystals into joints
Variety of clinical presentation from chronic polyarticular pain to acute inflammatory mono
CPP pathogenesis up to the deposition of crystals
Inorganic pyrophosphate made from ATP
Chondrocyte membrane known as ANKH controls efflu of ATP out of condrocytes and is therefore responsible for concentration of ePPi
High amounts of PPi in synovial space increases formation of crystals
Alkaline phosphatase degrates Pi, making it unavailable for crystallization
Any process that decrease alkaline phosphatase will favor formation
Stress on chondrocytes increases ATP release and therefore favors crystal formation
Effect of the crystals…pathologic
Induce infalmmatory reaction and increase release of matrix metalloproteinase from synoviocytes and chorndorcytes
Leads to cartilage degeneration
Also depostis in artticular cartilabe and alters mech processes
Other diseases that are associated with CPPD and why
Joint diseases because of chondrocyte stress (osteoarthritis, gout)
Metabolic conditions (hypophosphatasia with low alkaline phosphatase)
Also hyperparathyroidism, hypomagnesaemia, hemochromatosis, hypothyroidism, Wilson’s dz
CPPD epidemiology
Maybe 5% over 60…40% over 80
Acute CPPD
Pseudogout
Acute mono or oligo artthritis…most in knee, wrist, elbow and or ankle
Tends to be asymmetric with multiple joints in the same limb affectred
Systemic sx may be present
Tend to be precipitated by physuologic stress…resolve slowly
Chronic CPPD
POlyarticular involvement with similar joints to osteoarthritis
Chronic CPPD (different than OA), has intermittent flares characterized by an acute inflamm arthritis and progressive joint damage
Chronic also affects the shoulder, wrist, and MCP joints
Rarely presents like rheumatoid arthritis
Acute dx
Over 60 with acute mono or oligo pain
Evelated or ESR and CRP
If joint effusion, then arthrocentesis…look for positively birefringent, rhomboid shaped crystals in synovial fluid (pathognomonic)
Chronic dx
Over 60 with chornic polyarticlar…esp wrists or MCP joints with bouts of inflamm arthritis
If severe OA and joint degeneration, could also have CPPD dz
Radiography CPPD
Visualization of chondrocalcinosis (calcifciation of cartilage)
Common in menisci of the knee, triangular cart of radioulnar wrist, or glenoid labrum
Can also use ultraosund to detect hyperechoic linear densitiies
Acute manamgnet
Anti-inflam therapy
Intra-articular GCs…alternatives are NSAAIDs and colchicine
Chronic managment
NSAIDs and colchicine may decreasefreq of acute flares