Title: Calcium Pyrophosphate Deposition Disease Scott Gabbard
1Calcium Pyrophosphate Deposition DiseaseScott
Gabbard
2Prevalence
- Pathologic surveys indicate about 4 of the adult
population have articular CPPD deposits at the
time of death - Recent studies demonstrate radiographic evidence
of chondrocalcinosis in up to 50 of those older
than 85
3Pathogenesis
- CPPD crystal formation is initiated in cartilage
located near the surface of chondrocytes - Data suggests that the disorder is associated
with excessive cartilage production of
pyrophosphate which leads to calcium
pyrophosphate supersaturation and CPPD crystal
deposition
4Pathogenesis
- CPPD crystals are phagocytosed by neutrophils
- This results in release of lysosomal enzymes and
chemotactic factors
5Etiology
- Most attacks are idiopathic
- Disease associations include
- Joint trauma
- Familial chondrocalcinosis
- Hemochromatosis
- Gitelmans syndrome
- Hypomagnesemia hypophosphatemia
6Manifestations
- CPPD has the capacity to mimic any type of
arthiritis - Asymptomatic disease
- Most joints with radiographic evidence of CPPD
deposition have mild to no symptoms of disease
7Manifestations
- Pseudogout
- Subacute attacks of arthritis involving one or
several extremity joints - These attacks closely resemble those of urate
gout both in the typical accompanying signs of
severe acute inflammation and in the occasional
occurrence of cluster attacks
8Manifestations
- Pseudo-Rheumatoid Arthritis
- Multiple joints are involved with attacks of
articular inflammation lasting up to several
months - Symptoms include significant morning stiffness
fatigue synovial thickening localized edema
and restricted joint motion
9Manifestations
- Pseudo-Osteoarthritis
- 50 percent of patients with symptomatic CPPD
crystal deposition disease show progressive joint
degeneration - The joints most commonly affected include the
knees followed by the wrists MCPs hips
shoulders elbows and spine
10Differential diagnosis
- Gout
- Septic arthritis
- Rheumatoid arthritis
- Polymyalgia rheumatica
- Malignancy
- Seronegative spondyloarthropathies
- Hemochromatosis
- Lyme arthritis
11Diagnosis
- Synovial fluid
- Positively birefringent rhomboid crystals
- During acute pseudogout attacks will find
phagocytosed crystals within PMNs
12Diagnosis
- Intracellular phagocytosis of CPPD crystal
13Diagnosis
14Diagnosis
- Chondrocalcinosis
- Punctate and linear radiodensities in
fibrocartilage and articular cartilage
15Diagnosis
- Chondrocalcinosis of knee joint
16Diagnostic criteria
- Definitive diagnosis of CPPD deposition requires
- Demonstration of CPPD crystals in synovial fluid
by x-ray diffraction or - Positively birefringent crystals seen by
compensated polarized light and typical cartilage
calcification on x-ray
17Treatment
- Acute Pseudogout
- Removal of crystals by joint aspiration
- Administration of NSAIDs or colchicine
- Intraarticular injection of steroids such as
triamcinolone - Joint immobilization
18Treatment
- Colchicine
- Blocks release of chemotactic factors for
neutrophils and mononuclear cells and inhibits
neutrophil-endothelial cell binding
19Treatment
- Probenecid
- Inhibits transmembrane anion transport thus
decreasing extracellular pyrophosphate production
20References
- Becker Michael A. Clinical manifestations and
diagnosis of calcium pyrophosphate crystal
deposition disease. UpToDate. 2007. - Becker Michael A. Pathogenesis and etiology of
calcium pyrophosphate crystal deposition disease.
UpToDate. 2007. - Becker Michael A. Treatment of calcium
pyrophosphate crystal deposition disease.
UpToDate. 2007. - Klippel John. Primer on the Rheumatic Diseases.
The Arthritis Foundation 2001 - http//www.clevelandclinic.org/health/health-info/
Pictures/Pseudogout.gif Cleveland Clinic
Foundation - http//www.wheelessonline.com/image9/cppd2.jpg
- Lahita Robert. Educational Review Manual of
Internal Medicine Volume 1 Rheumatologic
Diseases CCGMP
21Im outta here!