Calcium Pyrophosphate Dihydrate Deposition Disease.pptx

Radiological Features of CPPD

1. Calcium Pyrophosphate Dihydrate Deposition Disease Presented by Dr MUSA WA FMCK 1

2. Outline Introduction Epidemiology Pathology Aetiology Clinical Presentation Radiological Feature Treatment Differential Diagnosis 2

3. Introduction Calcium pyrophosphate dihydrate disease (CPPD ) is also known as pyrophosphate arthropathy or pseudogout. Is defined by the co-occurrence of arthritis with evidence of CPPD deposition within the articular cartilage. CPPD crystals are deposited in articular + surrounding tissues (bursae, tendon sheaths and the annulus of the intervertebral discs). 3

4. Epidemiology The estimated incidence is around 1:250,000-1,000,000. Usually in 5 th decade of life or older, with increasing prevalence as age increases. Male and female are equally affected. 4

5. Pathology The crystals are weakly positively birefringent on polarized microscopy and have a rhomboid or rod shape (cf. to negatively birefringent needle shaped crystals in gout). 5

6. Aetiology Causes of CPPD can be divided into: Idiopathic Hereditary : AD pattern; mutation in the ANKH gene which encodes a transmembrane inorganic pyrophosphate transporter. Secondary : associated with hemochromatosis, hyperparathyroidism, hypothyroidism, hypomagnesemia, previous joint injury, ochronosis. 6

7. Clinical Presentation CPPD:  occurrence of calcium pyrophosphate crystals, with or without symptoms Asymptomatic CPPD:  chondrocalcinosis +/- changes of osteoarthritis, but clinically asymptomatic Acute CPPD crystal arthritis  (formerly  pseudogout ): self-limiting synovitis in the setting of CPPD Osteoarthritis with CPPD:  typical changes of  osteoarthritis  in the setting of CPPD Chronic CPPD crystal inflammatory arthritis 7

8. Radiologic Features Modalities Plain Radiography: Main stay Ultrasonography CT MRI Nuclear Imaging 8

9. Radiologic Features : Plain Radiography Chondrocalcinosis Classically occurs in the hyaline and fibrocartilage structures of the knee and wrist but can be seen in all joints. Appears as crystal deposit (opacities of calcific density). 9

10. Radiologic Features : Plain Radiography Osteoarthritis-like pattern Narrowed joint space + subchondral sclerosis + subchondral cyst. Unusual distribution : non-weight bearing joint (IC and MCP joints) and symmetrical Predilection for the patellofemoral compartment in the knee and the radiocarpal compartment in the wrist. Erosion is not a feature of CPPD arthropathy, though remodelling of the femoral aspect of the patellofemoral joint is well recognised. 20

11. Radiologic Features : Plain Radiography Hands Distribution: 1 CMC; 2nd + 3rd MCP joints; bilateral symmetric Resembling degenerative joint disease (without DIP and PIP involvement) Small hook-like osteophytes at radial aspect of metacarpal heads 2 & 3 11

12. 12 Frontal Radiograph of the hand showing hook like osteophytes of the 2 nd and 3 rd metacarpal bones on their radial aspect, markedly reduced joint space and sclerosis of the articular surfaces at the 1 st carpometacarpal joint. Soft tissue calcification is also noted

13. Radiologic Features : Plain Radiography Wrist Distribution: triangular fibrocartilage in distal radioulnar joints bilaterally, proximal carpal row joints at lunotriquetral + scapholunate ligaments Calcification (chondrocalcinosis) of triangular fibrocartilage Extensive narrowing / obliteration of joint space between distal radius + scaphoid → destruction of trapezioscaphoid space: Incorporation of scaphoid into articular surface of radius. Prominent cysts. Scapholunate separation (= ligament tear) → scapholunate advanced collapse (SLAC) 13

14. 14 Right Wrist Joint Radiograph, AP and lateral views. It shows obliteration of the joint space between the distal radius and the scaphoid with sclerosis of their articular surface. There is a well defined round lytic lesion with thin sclerotic margins seen in the scaphoid bone in keeping with subchondral cyst. The scaphoid and lunate carpal bones also appear separated. In absence of the history of trauma, CPPD is favoured as possible diagnosis.

15. Radiologic Features : Plain Radiography Knee Distribution: especially meniscus + cartilage of patellofemoral joint. Medial femorotibial + patellofemoral compartments commonly involved simultaneously (as in osteoarthritis) but with greater osseous destruction + fragmentation. Scalloping of the anterior femoral cortex at the level of the patella. Disproportionate narrowing of patellofemoral joint. 15

16. 16 Coned down Lateral knee Radiograph. There is loss of patellofemoral joint space and associated sclerosis of the articulating surfaces. There is scalloping of the anterior cortex of the distal femoral metaphysis. There is also calcification in the synovial and meniscal region in keeping with chondrocalcinosis.

17. Radiologic Features : Plain Radiography Spine Chondrocalcinosis / calcifications of outer fibres of annulus fibrosus of (lumbar) spine resembling syndesmophytes ; NEVER in nucleus pulposus. Crowned dens syndrome: periodontal calcification (above and sides of the top of the dens). DDx: Ochronosis (in nucleus pulposus). Other Locations Pelvis (sacroiliac joint, symphysis): vertical radio opaque line in symphysis pubis Shoulder (glenoid), hip (labrum), elbow, ankle, acromioclavicular joint 17

18. 18 Lateral Radiograph of the Cervical Spine. It shows narrowing of all disc spaces especially the C2/C3 with marginal anterior erosion seen on C3. Calcification of the anterior longitudinal ligament is also noted.

19. Radiologic Features : Ultrasonography Synovitis: hypoechoic synovial thickening with increased vascularity on colour or power doppler. Degree of abnormal blood flow proportional to severity of clinical manifestation. CPPD calcifications/deposits: Echogenic materials located within the articular cartilage itself(cf. to gout’s surface of articular cartilage) and other surrounding tissues. 19

20. 20 Coned down grey scale Ultrasound images. (A) Linear hyperechoic material within the hyaline cartilage of the knee. (B) Hyperechoic deposit within the Achilles tendon (C) Hyperechoic deposit within the synovial fluid (D) Hyperechoic deposit within the meniscal fibrocartilage.

21. Radiologic Features : CT May show hyperdense mass (of calcific density) with lobulated configuration and septum-like hypodense areas within it typically seen in the ligamentum flavum or within joint capsule. Others features that may be seen are: Fine granular calcification Subchondral cyst as well as fracture Crowned dens syndrome (CT gold standard) Routine x-rays usually reveals CPPD more accurately. Breakdown of prosthesis can mimic CPPD. 21

22. 22 Coned Down Axial and Coronal CT images in Bone window showing granular calcifications arranged in a crown like fashion around the dens and in the transverse ligament suggestive of crowned dens syndrome. DDx: CPPD and HADD

23. Radiologic Features : MRI T1-weighted Low-signal intensity with punctate signal void T2-weighted Varying signal intensity Rim enhancement rarely noted T1-weighted + Gad Peripheral enhancement Assoc. fracture: low intensity line on T1, T2 weighted spin echo. Findings can mimic prosthetic joint breakdown and meniscal tear. 23

24. 24 Sagittal ( a  and  b ) and axial (c and d) MRI demonstrated spinal cord compression by round lesions (black and white arrows) at C4 which appeared as a low signal mass on both T1 ( a  and  c ) and T2 ( b  and  d ) weighted images

25. Radiologic Features : Nuclear Imaging Increased uptake of bone-seeking radiopharmaceuticals ( 99m Tc-labelled disphosponate ) in affected joints. Extraosseous calcific deposits may also show increase uptake. Highly sensitive but no specific: localized tissue hyperemia , recent joint trauma or surgery, arthritis of any cause can produce identical results. 25

26. Differential Diagnosis Gout: periarticular crystal deposited cf. to intra-articular deposition in CPPD. Affects lower limb, typically 1 st metatarsophalangeal joint. Causes bony erosion. Calcium Hydroxyapatite Deposition Disease (HADD): also p eriarticular crystal deposited cf. to intra-articular deposition in CPPD. Affect shoulder mostly. Osteoarthritis : usually have the typical weight-bearing distribution.  Giant Cell Tumour (GCT) : subchondral cysts of CPPD can be large and simulate subchondral GCT 26

27. Treatment Depends on clinical manifestation Asymptomatic: no treatment If asymptomatic + secondary cause: treat underlying condition. Symptomatic Lifestyle: Ice packs Medications: NSAIDs, steroids (intra-articular, systemic), Colchicine, Intra-articular Na hyaluronate, Hydroxychloroquine (prevent flareup) Medical procedure: arthrocentesis Prognosis -Outcome is generally good, aggressive treatment can lead to complete resolution. 27

28. Reference Dahnert W. Radiology Review Manual. 8 th Ed. Philadelphia: Wolters Kluwer; 2017.p.227-228. Andreas A, Grainger & Allison’s Diagnostic radiology 6th edition. p.1131-1132. 28

29. Thanks for Listening 29