Page 2 of 48

Transcription

1 Conventional Radiography (CR), Computed Tomography (TC) and Magnetic Resonance imaging (MRI): What every radiologist should know about Calcium Pyrophosphate Dihydrate (CPPD) Crystal Deposition Disease? Poster No.: C-0602 Congress: ECR 2014 Type: Educational Exhibit Authors: R. Morcillo Carratalá, Y. Herrero Gómez, M. T. Fernández Taranilla, S. Fernandez, V. Artiles, M. Céspedes; Toledo/ES Keywords: Musculoskeletal system, Musculoskeletal joint, Musculoskeletal bone, Conventional radiography, CT, MR, Diagnostic procedure, Localisation, Complications, Pathology, Arthritides, Developmental disease DOI: /ecr2014/C-0602 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. Page 1 of 48

2 Page 2 of 48

3 Learning objectives The aim of this exhibit is to review a wide variety of imaging findings in conventional radiography (CR), computed tomography (CT) and magnetic resonance imaging (MRI) in the evaluation of calcium pyrophosphate crystals deposition (CPPD) disease. In this study, we will review retrospectively every patient with CPPD disease including CR, CT and MRI from our data base of musculoskeletal pathology since 2004 until currently. Page 3 of 48

4 Background CPPD disease is a disorder caused by the deposition of calcium pyrophosphate dihydrate in joints (usually intraarticular) and periarticular tissue. It is the most common crystalline arthropathy. It is also the most common cause of chondrocalcinosis. CPPD disease occurs especially in middle aged or elderly adults, with a female preponderance. The complexity in terminologies, classifications, and clinical phenotypes for this disorder has been evident since decades. Many terms have been used to describe the wide range of clinical syndromes associated with the CPPD disease in joints. An example is the following classification in several clinical patterns: Pseudogout (type A): acute self-limited attacks resembling gout that occurs mainly in the knee. Pseudo-rheumatoid arthritis (type B): intermittent acute attacks with duration of weeks or months resembling rheumatoid arthritis. Pseudo-osteoarthritis: chronic progressive arthropathy with acute exacerbations (type C) or without inflammation (type D). Asymptomatic (type E). Pseudo-neuropathic arthropathy (type F): destructive form with dissolution and fragmentation of joints resembling neuropathic arthropathy. Within the literature there is debate about which terms should be used. The European League Against Rheumatism (EULAR) CPPD Task Force, comprising 15 experts from 10 countries, suggests the following new terminology relating with this disorder with the aim of reducing confusion and the inconsistent use of terms such as "pseudogout": CPP crystals: calcium pyrophosphate crystals. Is the simplified term for calcium pyrophosphate dihydrate crystals. CPPD: umbrella term for all instances of CPP crystals deposition. Asymptomatic CPPD, osteoarthritis with CPPD, acute CPP crystal arthritis, and chronic CPP crystal inflammatory arthritis are included. CC: chondrocalcinosis defines cartilage calcification. Calcification in fibrocartilage and hyaline cartilage is detected by imaging or histological examination and in the overwhelming majority of cases is due to CPPD. It may or may not be an associated arthropathy. Clinical presentations associated with CPPD: Page 4 of 48

5 Asymptomatic CPPD: CPPD with no apparent symptoms. Often this form of presentation is identified incidentally in conventional radiography for other reasons. Osteoarthritis with CPPD: CPPD in a joint with a pattern of degenerative joint disease, on imaging or histological examination, with radiographically distinctive features. It may or may not be demonstrable chondrocalcinosis on imaging and the affected joints may not be symptomatic. Acute CPP crystal arthritis: onset is acute, with self-limiting attacks that may occur spontaneously or develop after trauma, surgery, or a severe medical illness. The affected joint is erythematous, hot, swollen, and tender. These features are highly suggestive of acute crystal inflammation though not specific to one crystal. The presence of these features involving the knee, wrist or shoulder in patients with upper age 65 years suggests acute CPP crystal arthritis. Gout is the main differential diagnosis (acute CPP crystal arthritis replaces the term 'pseudogout'). CPPD may also coexist with gout. Chronic CPP crystal inflammatory arthritis: CPPD with chronic mono/ oligoarthritis or polyarthritis with inflammatory symptoms and signs, occasionally with non specific elevation of C reactive protein and erythrocyte sedimentation rate. Rheumatoid arthritis and other chronic inflammatory joint diseases in older adults represent the main differential diagnosis. CPPD disease may be classified in several types: Idiopathic or sporadic: is the most common form of presentation. The incidence for this form increases with various factors, such as previous joint trauma, advanced age, and osteoarthritis. Production of extracellular inorganic pyrophosphate is increased and levels of pyrophosphatases seem to be decreased. The inorganic pyrophosphate binds with calcium to form CPP crystals which are then deposited about joints and periarticular tissue. Familiar forms: are rare. Mutation in a gene located on chromosome five that encodes a protein involved in the transport of pyrophosphate, identified as ankylosis protein homolog human gene (ANKH), seems to be implicated in the familial forms of CPPD. Association with others diseases: CPPD disease may also result from underlying metabolic abnormalities, especially hyperparathyroidism, hemochromatosis, hypomagnesaemia, and hypophosphatasia. Page 5 of 48

6 The presence of CPPD disease in patients with under age 50 years, especially polyarticular chondrocalcinosis or atypical osteoarthritis, familial predisposition and metabolic abnormalities should be considered and a biochemical screen should be included (serum calcium, parathyroid hormone, alkaline phosphatase, magnesium, and iron studies). Screening of family members should also be indicated if hemochromatosis, hypomagnesaemia or hypophosphatasia are confirmed. Identification of characteristic CPP crystals in synovial fluid is the key diagnostic method for CPPD: parallelepipedic, predominantly intracellular crystals with absent or weak positive birefringence under direct polarised light. Page 6 of 48

7 Findings and procedure details In this review, we will describe the spectrum of imaging findings in the CPPD disease in common and rare sites. The most common sites of presentation are (in decreasing order of frequency) knees, wrists, hands and hips. Other affected areas include elbows, shoulders, foot, and spine. IMAGING FINDINGS IN CR Routine radiographs usually reveal the pathology of CPDD disease more accurately. Therefore, conventional radiography is the key imaging method for the rapid diagnosis of CPPD disease. The spectrum of imaging findings in the CPPD disease in CR are: 1- Chondrocalcinosis: is a key feature. It is seen in either hyaline or fibrocartilage. Fibrocartilage calcification appears as irregular radiodense areas that are typically in the center of the joint, most commonly in knee menisci, symphysis pubis, and triangular fibrocartilage of the wrist (Fig. 1 on page 12). Other sites include acetabular labrum, glenoid labrum, and annulus fibrosus (Fig. 2 on page 12). Knee, symphysis pubis and wrist should be examined closely with radiographs in suspected cases for this disorder. Hyaline cartilage calcification is represented as a parallel thin line in close proximity to the subchondral bone, occurring mainly in knee, wrist, elbow, and hip (Fig. 3 on page 13). However, crystals may also be deposited in the synovium (amorphous opacity in the joint margin, such as metacarpophalangeal joints) and tendon insertion sites (calcifications are thin and linear, such as Achilles or quadriceps), as well as bursae, joint capsules, and ligaments (Fig. 4 on page 14). 2- Pyrophosphate arthropathy: with radiographically distinctive features: Pattern of structural changes resembling degenerative joint disease: uniform articular space narrowing, osteophytes, subchondral sclerosis, and subchondral cyst formation. CPPD should be considered as cause of atypical osteoarthritis when trauma is excluded. In very specific joints as well as with a specific distribution within a joint. Therefore, the distribution pattern is a distinctive feature: patellofemoral compartment in knee, radiocarpal in wrist, second and third Page 7 of 48

8 metacarpophalangeal joints in hand, hips, elbows, and shoulders (Fig. 5 on page 15). Often with a bilateral distribution, but not necessarily symmetric. It can be visualized in the absence of chondrocalcinosis. Subchondral cysts are a distinctive feature: they are common and with frequency are very large, more prominent than in osteoarthritis. They tend to be numerous and grouped, with sclerotic and ill-defined borders (Fig. 6 on page 16, Fig. 7 on page 17 ). The density of bone is normal in the overwhelming majority of cases. Productive bony changes may are present with sclerosis, variable osteophyte formation, osteochondral fragments, and often with numerous and millimeter intraarticular bodies. Occasionally the osteoarthritic changes may be so severe as to resemble a neuropathic joint (Fig. 8 on page 18). It does not have typical bony erosions. IMAGING FINDINGS IN CT AND MRI CT and MR imaging, have a relatively limited role in the majority of patients with CPPD disease. However, they may be requested to assess CPPD in patients with atypical or rare presentations of the disease, such as in the temporomandibular joint, or in the upper cervical spine (Fig. 9 on page 19). CT imaging is useful for identifying calcium deposits involving the joint space or periarticular fine granular calcifications. Chondrocalcinosis may also be seen in CT (Fig. 10 on page 20). CT also will allow for very good visualisation of degenerative changes of the affected joints (articular space narrowing, osteophytes, subchondral sclerosis, subchondral cyst formation), as well as associated fractures (Fig. 11 on page 21). MR imaging is very useful to evaluate the extent of disease and involvement of soft tissues and synovium. The MR image may also shows the pattern of degenerative joint disease typical of CPPD, especially very large subchondral cysts and numerous intraarticular bodies that may be calcified (Fig. 12 on page 22, Fig. 13 on page 23, Fig. 14 on page 24) MRI has low sensitivity for detecting calcification, but it may visualize massive deposition. At MR imaging, CPP crystals depositions have a wide spectrum of variable appearance, depending on crystal concentration and the amount of associated granulation tissue and fibrosis. CPP crystals depositions usually show low-signal intensity on T1-weighted, Page 8 of 48

9 variable signal intensity on T2-weighted images, and a variable enhancement pattern on post-contrast MR images. At MRI, the typical manifestations of acute inflammation are periarticular edema, synovitis, joint effusions and enhancement of the periarticular soft-tissue structures, but these imaging findings may be seen in other inflammatory arthropathies, such as gout. IMAGING FINDINGS IN SPECIFIC JOINTS 1- Knee: is the most common site for CPPD. Chondrocalcinosis is observed in fibrocartilaginous menisci as wedgeshaped calcification and in hyaline cartilage as curvilinear calcification paralleling the femoral condyles or tibial plateau (Fig. 15 on page 25). Crystals may also be deposited in the synovium, quadriceps and gastrocnemius tendons, or cruciate ligaments (see Fig. 15 on page 25). The degenerative changes may be seen in all compartments of the knee joint, but often are more disproportionate in the patellofemoral compartment (Fig. 16 on page 26). Arthropathy is less marked in the medial and lateral tibiofemoral joint spaces. CPPD disease should be considered if isolated patellofemoral involvement of the knee joint. There is often a scalloped defect in the anterior aspect of distal femur by abutment of the patella against the femur when the knee is in extension (Fig. 17 on page 27). Often calcified intraarticular bodies are present (Fig. 18 on page 28). The loss of all spaces joint of the knee with extensive subcondral sclerosis and bone fragmentation may resemble a neuropathic joint (see Fig. 8 on page 18). 2- Wrist: Chondrocalcinosis is seen in the triangular fibrocartilage and in hyaline cartilage surrounding any of the carpal bones, especially in lunatetriquetral region (Fig. 19 on page 29). Crystals may also be deposited in capsule and synovium, as well as in ligaments, mainly between the lunate and scaphoid or between the lunate and triquetrium (see Fig. 19 on page 29). The disruption of scapholunate ligament may result in dissociation of two bones. The degenerative changes are seen most commonly in the radiocarpal joint (Fig. 20 on page 30), but they may also be seen in any carpal bones (see Fig. 14 on page 24). If there is scapholunate dissociation (Fig. 21 on page 31), it may be present proximal migration of the scaphoid and distal migration of the lunate, with subsequent degenerative changes between the scaphoid and distal radius as well as between the lunate Page 9 of 48

10 and capitate, demonstrating on radiography a "step-ladder" configuration. This dissociation between lunate and scaphoid may lead to scapholunate advanced collapse (SLAC) wrist deformity. Other typical site of arthropathy for CPPD is the scaphoid-trapezium joint (Fig. 22 on page 32). 3- Hand: Deposition of CPP crystals in hyaline cartilage, synovium, and joint capsule may be seen surrounding any of the metacarpophalangeal joints, especially in second and third of these joints (Fig. 23 on page 33). The arthropathy is observed specifically in second and third metacarpophalangeal joints (Fig. 24 on page 34). Interphalangeal joints usually are spared. Hemochromatosis represents the main differential diagnosis: more extensive involvement of the metacarpophalangeal joints with characteristic large osteophytic beak (hooklike or drooping osteophyte) on the medial aspect of the head of the second and third metacarpals 4- Hip: Chondrocalcinosis is seen in the fibrocartilage of the acetabular labrum and in hyaline cartilage as curvilinear calcification paralleling the femoral head (Fig. 25 on page 35). Arthropathy for CPPD may frequently cause uniform joint space loss with associated degenerative changes and axial migration of the femoral head within the acetabulum. Unlike the osteoarthritis, osteophytes are less common and subchondral cysts formation larger and may dominate the picture (Fig. 26 on page 36). 5- Elbow: CPP crystals may be deposited in hyaline cartilage, synovium, tendon (mainly triceps tendon) and joint capsule around the elbow (Fig. 27 on page 37). Arthropathy for CPPD may be seen in all compartments of the elbow joint (Fig. 28 on page 38). Bilateral distribution of osteoarthritic changes in both elbow joints suggests the diagnosis of CPPD disease. 6- Shoulder: Deposition of CPP crystals includes hyaline cartilage, synovium, joint capsule, subdeltoid and subacromial bursae, and tendons in the area of the rotator cuff attachment simulating hydroxiapatite desposition disease (Fig. 29 on page 39, Fig. 30 on page 40). Some patients with chronic tendinitis and burstitis by CPPD have degeneration and break of the rotator cuff with loss of the subacromial space (see Fig. 13 on page 23). Page 10 of 48

11 Observed calcification around the acromioclavicular joint suggests a diagnosis earlier and definitive (see Fig. 30 on page 40). Arthropathy for CPPD should beconsidered if both glenohumeral joints have bilateral and symmetrical degenerative changes. It is characteristic a large osteophyte on the medial and inferior aspect of the humeral head (Fig. 31 on page 41). 7- Foot: Chondrocalcinosis and deposition of CPP crystals are unusual but may be seen surrounding any of the foot bones, especially around the metatarsophalangeal joints and talonavicular region (Fig. 32 on page 42). Swelling soft tissues around the first metatarsophalangeal joint with periarticular calcification may simulate gout. Crystals may also be deposited at the attachment of the Achilles tendon or may be seen in retrocalcaneal bursa with associated retrocalcaneal bursitis (Fig. 33 on page 43). Arthropathy for CPPD is less common. In some patients the involvement of the tarsal area has been reported, especially the talocalcaneonavicular joint (see Fig. 33 on page 43). 8- Spine: cervical, thoracic, and lumbar spine may be affected. Chondrocalcinosis is seen in the annulus fibrosus of the intervertebral disk, more frequently in the outer annular fibers (see Fig. 9 on page 19; Fig. 34 on page 44). These annular calcifications may be extensive and widespread, but the nucleus pulposus is not commonly calcified. Crystals may also be deposited in different spinal ligaments: anterior longitudinal ligament, posterior longitudinal ligament, supraspinous and interspinous ligaments, ligamentum flavum, and transverse ligament of the dens (see Fig. 9 on page 19). Deposits of CPP crystals partially calcified around the odontoid process may simulate a mass and may cause cervicomedullary compression. This is known as crowned dens syndrome and it is a rare cause of acute neck pain. Arthropathy for CPPD is characterized by a disc space narrowing that is quite common and that may affect at multiple levels, with considerable vertebral sclerosis and vacuum phenomenon (see Fig. 9 on page 19). The apophyseal facet joints may also be affected with associated degenerative change that may result in spondyloisthesis. Page 11 of 48

12 Images for this section: Fig. 1: Figure 01. Chondrocalcinosis. (A) AP radiograph shows typical chondrocalcinosis in fibrocartilaginous menisci of the knee (white arrows). (B) PA radiograph reveals classic chondrocalcinosis in the triangular fibrocartilage of the wrist (black arrow). (C) AP radiograph shows chondrocalcinosis in fibrocartilage of the symphysis pubis (orange arrow). Page 12 of 48

13 Fig. 2: Figure 02. Chondrocalcinosis. (A) AP radiograph shows chondrocalcinosis in acetabular labrum of the hip (yellow arrow). AP (B) and lateral (C) radiographs of the lumbar spine reveal chondrocalcinosis in annulus fibrosus of the intervertebral disk (black arrows). Page 13 of 48

14 Fig. 3: Figure 03. Chondrocalcinosis. (A) AP radiograph shows chondrocalcinosis in hyaline cartilage as curvilinear calcification paralleling the femoral head (black arrow). (B) AP radiograph shows chondrocalcinosis in hyaline cartilage as curvilinear calcification paralleling the femoral condyle and tibial plateu (white arrows). (C) AP radiograph shows chondrocalcinosis in hyaline cartilage as curvilinear calcification paralleling the humeral head (yellow arrows). Page 14 of 48

15 Fig. 4: Figure 04. CPP crystals deposition. (A, B) PA radiographs show calcification in the capsule, ligaments and synovium around the metacarpophalangeal joints (white arrows). (C) AP radiograph shows CPP crystals deposition in the subdeltoid bursa (blue arrow). (D) Lateral radiograph of the knee reveals thin and linear calcifications in the gastrocnemius tendon (yellow arrow). (E) Axial CT image shows calcification in transverse ligament of the dens (black arrow). Page 15 of 48

16 Fig. 5: Figure 05. Pyrophosphate arthropathy: atypical osteoarthritis in very especific joints with specific distribution. (A) Lateral radiograph of the knee shows femoropatellar compartment in arthropathy for CPPD. (B) AP radiograph shows shoulder in arthropathy for CPPD. (C) AP radiograph shows elbow in arthropathy for CPPD. (D) PA radiograph reveals the second and third metacarpophalangeal joints in arthropathy for CPPD.(E) PA radiograph reveals the radiocarpal joint in arthropathy for CPPD. Page 16 of 48

17 Fig. 6: Figure 06. Subchondral cysts formation. AP (A) and lateral (B) radiographs of the knee show large subchondral cyst with sclerotic borders on the anterior aspect of the medial tibial plateau (yellow arrows). There are also chondrocalcinosis and subchondral cysts formation on the posterior and superior aspect of the patella. Page 17 of 48

18 Fig. 7: Figure 07. Subchondral cysts formation. AP (A) and lateral (B) radiographs of the knee show numerous and grouped subchondral cysts with sclerotic and ill-defines borders on the posterior aspect of the lateral tibial plateau (white arrows). Important degenerative changes in femoropatellar compartment are present. The medial and lateral tibiofemoral joint spaces are preserved. Page 18 of 48

19 Fig. 8: Figure 08. Productive bone changes. AP (A) and lateral (B) radiographs of the knee show collapse of all joint spaces with extensive subchondral sclerosis, important osteophyte formation, and calcified intraarticular bodies. Subluxation, joint destruction and heterotopic new bone formation are present in this knee. These imaging findings resemble those of a neuropathic joint. Page 19 of 48

20 Fig. 9: Figure 09. Upper cervical spine in CPPD disease. (A) Lateral radiograph of the cervical spine shows calcification in annulus fibrosus of the intervertebral disk (yellow arrow). Sagital (B) and axial (C) CT images reveal deposits of CPP crystals in annulus fibrosus of the intervertebral disk (white arrows), and disc space narrowing at multiple levels, demonstrating the vacuum phenomenon at one disk level (blue arrow). There is also calcification of the transverse ligament of the dens (black arrows). Page 20 of 48

21 Fig. 10: Figure 10. Chondrocalcinosis in CR and CT. Coronal CT image (A) and AP radiograph (B) of the knee show typical chondrocalcinosis (white arrows). Axial CT image (C) and AP radiograph (D) show typical chondrocalcinosis in fibrocartilage of the symphysis pubis (black arrows). Coronal CT image (E) and PA radiograph (F) of the wrist reveal classic chondrocalcinosis in the triangular fibrocartilage (blue arrows). There is also calcification in ligaments, between the lunate and scaphoid (yellow arrows), and between the lunate and triquetrium (orange arrows). Page 21 of 48

22 Fig. 11: Figure 11. The knee in patient with CPPD disease. Sagital (A) and axial (B) CT images show marked patellofemoral joint space narrowing (orange arrows) with osteophyte formation, subchondral sclerosis and extensive subchondral cyst formation. Patella longitudinal fracture is present (white arrow). Tibiofemoral compartment is preserved (blue arrows). Page 22 of 48

23 Fig. 12: Figure 12. Knee in patient with CPPD disease. AP (A) and lateral (B) radiographs of the knee show large subchondral cyst with sclerotic and ill-defines borders on the posterior aspect of the lateral tibial plateau (black arrows). Collapse of femoropatellar compartment is present. The medial and lateral tibiofemoral joint spaces are preserved. Fat-supressed coronal T2-weighted (C) and sagital T2-weighted (D) MR images demonstrating presence of large subchondral cyst (yellow arrows). MR images show also collapsed patellofemoral joint space with loss of articular cartilage and osteophytes (white arrows). Note the preserved tibiofemoral compartments. Page 23 of 48

24 Fig. 13: Figure 13. Shoulder in patient with CPPD arthropathy. (A) AP radiograph of the shoulder shows total loss of the glenohumeral joint space with subchondral sclerosis, subchondral cyst formation, osteophytes, and calcified intraarticular bodies (black arrow). There is also loss of the subacromial space. Axial T2-weighted (B) and fat-supressed coronal T2-weighted (C) MR images reveal loss of the glenohumeral joint space (blue arrows), subchondral cysts formation (orange arrows), and millimieter intraarticular bodies which show low-signal intensity (yellow arrows). There is also break of the supraspinous tendon (white arrow) with loss of the subacromial space. Page 24 of 48

25 Fig. 14: Figure 14. Wrist in patient with CPPD arthropathy. (A) PA radiograph of the wrist shows osteoarthritic changes involving the radiocarpal joint (black arrows) and several carpal bones (blue arrows). Coronal T1-weighted (B) and sagital T1-weighted (C) MR images reveal loss of the radiocarpal joint space with several subchondral cysts formation of sclerotic borders in capitate, scaphoid, lunate, triquetrium, and distal radius (orange arrows). (D, E) Post-contrast fat-supressed coronal T1-weighted MR images show synovitis around the radiocarpal joint and carpal bones (yellow arrows). Note the prominent cysts subchondral in the capitate (white arrow). Page 25 of 48

26 Fig. 15: Figure 15. CPPD disease in knee. AP (A) and lateral (B) radiographs of the knee show typical chondrocalcinosis in fibrocartilaginous menisci (white arrows) and hyaline cartilage (yellow arrows). There is also calcification of the quadriceps tendon (blue arrow), as well as of the gastrocnemious tendon (black arrow). Note osteoarthritic changes more disproportionate in the patellofemoral compartment. Page 26 of 48

27 Fig. 16: Figure 16. Knee in patient with CPPD arthropathy. AP (A) and lateral (B) radiographs of the knee show degenerative changes more disproportionate in the patellofemoral compartment (black arrow) and less marked in the medial and lateral tibiofemoral joint spaces (yellow arrows). Note the prominent subchondral cyst on the posterior aspect of the lateral tibial plateau (white arrows). There is also chondrocalcinosis present. Page 27 of 48

28 Fig. 17: Figure 17. Knee in CPPD arthropathy. (A-C) Lateral radiographs of the knee show scalloped defect in the anterior aspect of distal femur by abutment of the patella against the femur (white arrows). Note degenerative changes disproportionate in the patellofemoral compartments. There is also chondrocalcinosis present. Page 28 of 48

29 Fig. 18: Figure 18. Knee in CPPD arthropathy. (A-C) Lateral radiographs of the knee show calcified intraarticular bodies (white arrows). Note degenerative changes disproportionate in the patellofemoral compartments and scalloped defects in the anterior aspect of distal femur. There is also chondrocalcinosis present. Page 29 of 48

30 Fig. 19: Figure 19. Wrist in CPPD disease. (A-E) PA radiographs of the wrist reveal classic chondrocalcinosis in the triangular fibrocartilage (white arrows) and in hyaline cartilage in lunatetriquetral region (black arrows). There is also calcification in ligaments, between the lunate and scaphoid (yellow arrows), and between the lunate and triquetrium (orange arrows). Page 30 of 48

31 Fig. 20: Figure 20. Wrist in CPPD arthropathy. (A-D) PA radiographs of the wrist reveal loss of radiocarpal joint spaces (black arrows) with subchondral sclerosis and subchondral cysts formation (white arrows). Classic chondrocalcinosis in the triangular fibrocartilage of the wrist is present. Page 31 of 48

32 Fig. 21: Figure 21. Wrist in CPPD arthropathy. (A) PA radiograph of the wrist reveals scapholunate dissociation with degenerative changes involving scaphoid, lunate, capitate, and trapezium. (B) PA radiograph of the wrist shows scapholunate dissociation with osteoarthritic changes involving the radiocarpal joint and mainly the lunate. (C) PA radiograph of the wrist shows between lunate and scaphoid with osteoarthritic changes involving the radiocarpal joint and adjacent carpal bones. Subluxation and joint destruction are present in this wrist. Page 32 of 48

33 Fig. 22: Figure 22. Wrist in CPPD arthropathy. (A) PA radiograph of the wrist reveals degenerative changes in the scaphoid-trapezium joint (black arrow). Calcification in the triangular fibrocartilage of the wrist is present (blue arrow). (B) Coronal CT image shows osteoarthritic changes between scaphoid and trapezium, as well as between scaphoid and trapezoid (yellow arrow). (C) Coronal CT image shows loss of joint space between scaphoid and trapezoid with subchondral cysts formation (white arrow). Page 33 of 48

34 Fig. 23: Figure 23. Metacarpophalangeal joints in CPPD disease. (A-D) PA radiographs reveal deposition of CPP crystals in hyaline cartilage, synovium, joint capsule, and ligaments surrounding the second and third metacarpophalangeal joints (white arrows). There is also calcifications around the first and fifth metacarpophalangeal joints (yellow arrows). Page 34 of 48

35 Fig. 24: Figure 24. Metacarpophalangeal joints in CPPD arthropathy. (A-C) PA radiographs show loss of the joint spaces with osteophyte formation and subchondral sclerosis specifically in the second and third metacarpophalangeal joints (white arrows). There is also deposition of CPP crystals around the second and third metacarpophalangeal joints (yellow arrows). Page 35 of 48

36 Fig. 25: Figure 25. Hip in CPPD disease. (A, B) AP radiographs show chondrocalcinosis in hyaline cartilage as curvilinear calcification paralleling the femoral head (white arrows). (C) AP radiograph shows calcification in acetabular labrum (black arrow). Note the joint space loss, large subchondral cysts formation, and axial migration of the femoral head within the acetabulum. Page 36 of 48

37 Fig. 26: Figure 26. Hip in CPPD arthropathy. (A, B) AP radiographs show uniform joint space loss with associated degenerative changes and axial migration of the femoral head within the acetabulum (white arrows). Note the prominent subchondral cysts formation (black arrows). Calcification in acetabular labrum is present (yellow arrow). Page 37 of 48

38 Fig. 27: Figure 27. Elbow in CPPD disease. (A) AP radiograph shows calcification in the capsule and synovium around the humeroradial joint (white arrow). There is also loss of the humeroulnar joint space with subchondral sclerosis and osteophyte formation (yellow arrow). Note the prominent subchondral cyst formation on the lateral aspect of the lateral humeral epicondyle (black arrow). (B) AP radiograph reveals calcification in hyaline cartilage paralleling the medial humeral epicondyle (orange arrows). Degenerative changes in the superior radioulnar joint are present. Page 38 of 48

39 Fig. 28: Figure 28. Elbow in CPPD arthropathy. AP (A) and lateral (B) radiographs of the elbow show loss of all joint spaces with subchondral sclerosis, osteophyte formation (yellow arrows), subchondral cysts formation (black arrow), and calcified intraarticular bodies (white arrow). Page 39 of 48

40 Fig. 29: Figure 29. Shoulder in CPPD disease. (A, B) AP radiographs show chondrocalcinosis in hyaline cartilage as curvilinear calcification paralleling the humeral head (yellow arrows). Note the calcification at the supraspinous tendon attachment (white arrow). Page 40 of 48

41 Fig. 30: Figure 30. Shoulder in CPPD disease. (A, B) AP radiographs show CPP crystals deposition in the subdeltoid bursa (black arrow). There is also calcification around the acromioclavicular joint (white arrows). Page 41 of 48

42 Fig. 31: Figure 31. Shoulder in CPPD arthropathy. (A) AP radiograph of the shoulder demonstrates loss of the glenohumeral joint space with characteristic large osteophyte on the medial and inferior aspect of the humeral head (black arrow). (B) AP radiograph of the shoulder shows loss of the glenohumeral joint space with subchondral sclerosis, osteophyte formation, subchondral cysts formation, and calcified intraarticular bodies (white arrows). Page 42 of 48

43 Fig. 32: Figure 32. Foot in CPPD disease. (A-C) AP radiographs show calcifications around the fourth and fifth metatarsophalangeal joints (white arrows), the first cuneiform (yellow arrow), and the talonavicular region (black arrows). Page 43 of 48

44 Fig. 33: Figure 33. Foot in CPPD disease. (A) AP radiograph shows swelling soft tissues with calcifications around the first metatarsophalangeal joint (white arrows) simulating gout. (B) AP radiograph reveals CPP crystals deposition in the retrocalcaneal bursa (yellow arrow). There is also loss of the talocalcaneal joint space with extensive subchondral sclerosis and osteophyte formation (black arrows). Ossification is seen at the attachment of the plantar aponeurosis. Page 44 of 48

45 Fig. 34: AP (A) and lateral (B) radiographs of the lumbar spine reveal chondrocalcinosis in annulus fibrosus of the intervertebral disk (black arrows). (C) Sagital CT image confirms the presence of chondrocalcinosis (white arrows). Page 45 of 48

46 Conclusion The radiographic features of CPPD crystal deposition disease are quite characteristic or typical. The presence of chondrocalcinosis and a pattern of degenerative joint disease in very specific joints as well as in a specific distribution within a joint should suggest the diagnosis of CPDD disease. Consequently radiologist must perfectly recognize imaging features with CR, CT and MRI in the evaluation of this disorder to make an earlier diagnosis and in order to differentiate them from other arthropathies. Page 46 of 48

47 Personal information Rafael Morcillo Carratalá 2nd year Radiology Resident at Hospital Virgen de la Salud, Toledo, Spain; Y. Herrero Gómez, Department of Radiology, Hospital Virgen de la Salud, Toledo, Spain; M.T. Fernández Taranilla, Department of Radiology, Hospital Virgen de la Salud, Toledo, Spain; S. Fernández Zapardiel, Department of Radiology, Hospital Virgen de la Salud, Toledo, Spain; V. Artiles Valle, Department of Radiology, Hospital Virgen de la Salud, Toledo, Spain; M. M. Céspedes Mas, Department of Radiology, Hospital Virgen de la Salud, Toledo, Spain; Page 47 of 48

48 References 1- Zhang W, Doherty M, Bardin T, et al. European League against Rheumatism recommendations for calcium pyrophosphate deposition. Part I: terminology and diagnosis. Ann Rheum Dis. 2011;70: Brower AC. Arthritis in black and white. Philadelphia: W.B. Saunders; Manaster BJ. Handbook of skeletal radiology. 2nd ed. St. Louis: Mosby; Resnick D. Bone and Joint Imaging. 2nd ed. Philadelphia: WB Saunders; Clyde A.Helms, James B. Vogler, David A. Simms, Harry K.Genant. CPPD crystal deposition disease or pseudogout. RadioGraphics. 1982;2: Dalbeth N, McQueen F. Use of imaging to evaluate gout and other crystal deposition disorders. Curr Opin Rheumatol. 2009;21: Gary D Wright, Michael Doherty. Calcium pyrophosphate crystal deposition is not always 'wear and tear' or aging. Annals of the Rheumatic Diseases. 1997;56: Goto S, Umehara J, Aizawa T, Kokunun S. Crowned Dens syndrome. J Bone Joint Surg Am. 2007;89: Page 48 of 48