Paget disease of the bone is a common, chronic bone disorder characterised by excessive abnormal bone remodeling. It frequently affects the pelvis, spine, skull and proximal long bones and has characteristic radiographic features.
It is relatively common and can affect up to 4% of individuals over 40 and up to 11% over the age of 80 1. There may a slight male predilection.
The majority (approximately three quarters) of patients are asymptomatic at the time of diagnosis. Presenting symptoms include:
- localised pain and tenderness
- increased focal temperature due to hyperaemia (due to hypervascularity)
- increased bone size: historically changing hat size was a give-away
- bowing deformities
- kyphosis of the spine
- decreased range of motion
- signs and symptoms relating to complications (see below)
Polyostotic disease is more common than the monostotic type 1. The most frequent sites of involvement are:
- pelvis (often asymmetric)
- proximal long bones
The aetiology is not entirely known, however viral infection in association with genetic susceptibility has been postulated.
There are three stages classically described (but is part of continuous spectrum)
- lytic (incipient active): predominated by osteoclastic activity
- mixed (active): osteoblastic as well as osteoclastic activity
- sclerotic/blastic (late inactive)
- serum alkaline phopatase (ALP) elevated
- urine hydroxyproline increased
The early phase features osteolytic (lucent) region which is later followed by coarsened trabeculae and bony enlargement. Sclerotic changes occur much later in the disease process.
- osteoporosis circumscripta: large well defined lytic lesion
- cotton wool appearance: mixed lytic and sclerotic lesions of skull
- diploic widening: both inner and outer calvarial tables are involved, with the former usually more extensively affected
- Tam O'shanter sign: frontal bone enlargement, with the appearance of the skull falling over the facial bones, like a Tam O'shanter hat.
- picture frame sign: Paget disease of the spine frequently manifests with cortical thickening and sclerosis encasing the vertebral margins, which gives rise to the on radiographs in mixed phase disease
- squaring of vertebra: on lateral radiographs flattening of the normal concavity of the anterior margin of the vertebral body also adds to the rectangular appearance
- vertical trabecular thickening: in Paget disease is coarser than the more delicate pattern seen in hemangiomas with which it can be confused
- cortical thickening and sclerosis of the iliopectineal and ischiopubic lines
- acetabular protrusio
- enlargement of the pubic rami and ischium
These findings are often asymmetric and for some reason, may be more commonly seen on the right side.
- blade of grass or candle flame sign: begins as a subchondral area of lucency with advancing tip of V shaped osteolysis extending towards the diaphysis. (In rare cases, the disease is isolated to the diaphysis, most commonly in the tibia, rather than subchondral bone, which can cause diagnostic confusion)
- lateral curvature of the femur
- anterior curvature of tibia
Paget disease related signs include:
- Tam O'Shanter sign
- blade of grass sign
- osteoporosis circumscripta
- jigsaw pattern bone or mosaic pattern bone
- picture frame vertebra
- cotton wool appearance of bone
- banana fracture
- Looser zones
Highly sensitive but not specific. Traditionally has been said to demonstrate marked increased uptake in all phases of disease, although in the burnt out sclerotic quiescent phase uptake may be normal 1.
Treatment and complications
- osseous weakening resulting in deformity and fractures
- neural compression
- secondary development of tumours (e.g. osteosarcoma: ≈1% of cases), which is often highly resistant to treatment
- high output congestive cardiac failure
- hyperparathyroidism (~10%)
For skull lesions consider:
- hyperostosis frontalis interna: thickening of the internal table of the frontal bone
- different age group
- fibrous dysplasia usually affects the outer table more prominently
History and etymology
It was first described by Sir James Paget in 1877 in a case report of a patient he had observed over some twenty years5.
The condition was originally named by Paget "osteitis deformans", implying an inflammatory aetiology. The term "osteodystrophica deformans" is now preferred.
- 1. Smith SE, Murphey MD, Motamedi K et-al. From the archives of the AFIP. Radiologic spectrum of Paget disease of bone and its complications with pathologic correlation. Radiographics. 22 (5): 1191-216. Radiographics (full text) - Pubmed citation
- 2. Walsh JP. Paget's disease of bone. Med. J. Aust. 2004;181 (5): 262-5. Med. J. Aust. (link) - Pubmed citation
- 3. Tjon-a-tham RT, Bloem JL, Falke TH et-al. Magnetic resonance imaging in Paget disease of the skull. AJNR Am J Neuroradiol. 6 (6): 879-81. AJNR Am J Neuroradiol (abstract) - Pubmed citation
- 4. Dell'Atti C, Cassar-Pullicino VN, Lalam RK et-al. The spine in Paget's disease. Skeletal Radiol. 2007;36 (7): 609-26. doi:10.1007/s00256-006-0270-6 - Free text at pubmed - Pubmed citation
- 5. Paget J. On a Form of Chronic Inflammation of Bones (Osteitis Deformans). Med Chir Trans. 2011;60: 37-64.9. Free text at pubmed - Pubmed citation
Synonyms & Alternative Spellings
|Synonyms or Alternative Spelling||Include in Listings?|
|Paget disease of the bone||✗|
|Paget disease of bone||✗|
|Paget disease of the bones||✗|
|Paget disease of bones||✗|
|Paget's disease of bones||✗|
|Paget's disease of the bones||✗|