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CPPD & other Crystal Deposition Disease
Iraj Salehi-Abari MD., Internist Rheumatologist
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Other Crystal Deposition Disease
Calcium Pyrophosphate Dihydrate (CPPD) Basic Calcium Phosphate (BCP): Hydroxy Apatite (HA) Octa Calcium Phosphate (OCP) Tri Calcium phosphate (TCP) Calcium Oxalate (Ca, Ox) Others: Cholesterol Corticosteroid Etc.
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CPPD disease Epidemiology: Prevalence: 0.9/1000 F/M: #1; >1
Age: 80% of Pts> 60Yrs Classification Class 1: Hereditary/ Familial Class 2: Sporadic/ Idiopathic Class 3: Metabolic Class 4: Traumatic/ Surgery (0-3 days postsurgery)
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Pathophysiology ATP ATP ppase AMP + PPi AMP 5NTase A + Pi
PPi PPi ase 2Pi Aging elevated PPi; because Increased ATP ppase Increased 5NTase Decreased PPiase Aging dep. Chondroitin Sulfate dep,. Mg, dep. ALK. Ph., elev. Ca. elev. PPi
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Clinical Feature Type A: Pseudo-Gout Type B: Pseudo-RA
Type C: Pseudo-OA (acute on chronic) Type D: Pseudo-OA (chronic) Type E: Lanthanic; Chondrocalcinosis Type F: Pseudo-Neuropathic Arthritis Others:Pseudo-AS, Hemarthrosis
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Clinical Feature Type A: Pseudo-Gout Type B: Pseudo-RA
Type C: Pseudo-OA (acute on chronic) Type D: Pseudo-OA (chronic) Type E: Lanthanic; Chondrocalcinosis Type F: Pseudo-Neuropathic Arthritis Others:Pseudo-AS, Hemarthrosis
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Clinical Feature Type A: Pseudo-Gout Type B: Pseudo-RA
Type C: Pseudo-OA (acute on chronic) Type D: Pseudo-OA (chronic) Type E: Lanthanic; Chondrocalcinosis Type F: Pseudo-Neuropathic Arthritis Others:Pseudo-AS, Hemarthrosis
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Clinical Feature Type A: Pseudo-Gout Type B: Pseudo-RA
Type C: Pseudo-OA (acute on chronic) Type D: Pseudo-OA (chronic) Type E: Lanthanic; Chondrocalcinosis Type F: Pseudo-Neuropathic Arthritis Others:Pseudo-AS, Hemarthrosis
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Clinical Feature Type A: Pseudo-Gout Type B: Pseudo-RA
Type C: Pseudo-OA (acute on chronic) Type D: Pseudo-OA (chronic) Type E: Lanthanic; Chondrocalcinosis Type F: Pseudo-Neuropathic Arthritis Others:Pseudo-AS, Hemarthrosis
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Clinical Feature Type A: Pseudo-Gout Type B: Pseudo-RA
Type C: Pseudo-OA (acute on chronic) Type D: Pseudo-OA (chronic) Type E: Lanthanic; Chondrocalcinosis Type F: Pseudo-Neuropathic Arthritis Others:Pseudo-AS, Hemarthrosis
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Diagnostic Criteria 1. Identification of CPPD by:
Chemical Analysis or X-Ray Diffraction or Electron Diffraction 2a CPPD morphology: LM, PM 2b chondrocalcinosis in X-Ray 3a. Acute Arthritis Attacks 3b. Subacute or Chronic Arthritis
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Diagnostic Criteria (cont.d)
Categories: Definite: 1 or (2a + 2b) Probable: 2a or 2b Possible: 3
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Approach to Pts with CPPDd
Age< 50 & (+) FH: Familial CPPDd Age <50 & (-)FH: Metabolic CPPDd Age >60 & (-)FH: Idiopathic
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Management Acute: Subacute & Chronic Treatment of underlying disorders
Aspiration Steroid (intra articular) Colchicine NSAIDs Subacute & Chronic Similar to OA Treatment of underlying disorders
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Hydroxy Apatite (HA) Clinical Forms: Periarthritis Arthritis
Gout like type Milwaukee shoulder/ knee synd. Erosive polyarticular type Mixed: HA + CPPD Secondary Arthropathies CRF, DM, Hypothyroidism, Acromegaly CTD:Dermatomyositis, PSS Tumor calcinosis
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Milwaukee shoulder/knee synd
Destructive Arthritis Rare, old age, F/M #4/1 DJD +Tendonitis & Rupture of RC + Hydrops of shoulder DJD of lateral compartment of knee
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X-Ray Findings (Milwaukee)
DJD Destruction Calcification Upward subluxation of humerous RC tearing (H to Ac< 6 mm) Pseudo articulation of H & Ac DJD of lateral compartment of knee
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Synovial Fluid (Milwaukee)
Milky WBC < 1000/mm3 Crystals: Seen only with EM Shiny coin in LM No polarizing
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Management (Milwaukee)
Decreased using of shoulder Recurrent aspiration Intra articular steroid NSAIDs (?) Supportive physiotherapy Surgery
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Calcium Oxalate Skin Calcification Chondrocalcinosis
Erosive Arthropathy Tenosynovitis, Bursitis Vascular Calcification Cardiomyopathy Corneal Deposition Deposition in finger, wrist, elbow, knee, ankle, feet
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Calcium Oxalate (Cont,d)
Miliary Calcification in finger & vessels: Characteristic Synovial Fluid: WBC < 2000 Crystal: Bipyramidal in LM (+) Birefringent in PM Treatment; Colchicine, NSAIDs
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Clinical Feature Type A: Pseudo-Gout Type B: Pseudo-RA
Type C: Pseudo-OA (acute on chronic) Type D: Pseudo-OA (chronic) Type E: Lanthanic; Chondrocalcinosis Type F: Pseudo-Neuropathic Arthritis Others:Pseudo-AS, Hemarthrosis
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Alkaptonuria(AKU): Alkaptonuria: Dark urine disease
Autosomal recessive Hemogentisic acid dioxygenase (HGD, HGO) deficiency The third enzyme in tyrosine degradation Elevated levels of Homogentisic acid (HGA) It polymerizes, forming a pigment that is deposited in connective tissue: Ochronosis
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Alkaptonuria(AKU): Secodary OA in Shoulder, Hip and Knee
Axial involvement Urine turns dark brown or black if left standing or after alkalinization Levels of HGA are increased in blood, urine and tissue samples No effective therapy, dietary restriction of tyrosine and phenylalanine, Ascorbic acid, Nitisinone
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Calcium Oxalate Skin Calcification Chondrocalcinosis
Erosive Arthropathy Tenosynovitis, Bursitis Vascular Calcification Cardiomyopathy Corneal Deposition Deposition in finger, wrist, elbow, knee, ankle, feet
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Calcium Oxalate Skin Calcification Chondrocalcinosis
Erosive Arthropathy Tenosynovitis, Bursitis Vascular Calcification Cardiomyopathy Corneal Deposition Deposition in finger, wrist, elbow, knee, ankle, feet
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Calcium Oxalate Skin Calcification Chondrocalcinosis
Erosive Arthropathy Tenosynovitis, Bursitis Vascular Calcification Cardiomyopathy Corneal Deposition Deposition in finger, wrist, elbow, knee, ankle, feet
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Calcium Oxalate Skin Calcification Chondrocalcinosis
Erosive Arthropathy Tenosynovitis, Bursitis Vascular Calcification Cardiomyopathy Corneal Deposition Deposition in finger, wrist, elbow, knee, ankle, feet
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Calcium Oxalate Skin Calcification Chondrocalcinosis
Erosive Arthropathy Tenosynovitis, Bursitis Vascular Calcification Cardiomyopathy Corneal Deposition Deposition in finger, wrist, elbow, knee, ankle, feet
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Others: Wilson disease Chondrocalcinosis of hand and knee
Hereditary Hemochromatosis (HH): The full spectrum of CPPD disease Squared-off bone ends and hook-like osteophytes in the MCPs (2, 3)
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Calcium Oxalate Skin Calcification Chondrocalcinosis
Erosive Arthropathy Tenosynovitis, Bursitis Vascular Calcification Cardiomyopathy Corneal Deposition Deposition in finger, wrist, elbow, knee, ankle, feet
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