AJR:196, June 2011 WS53

AJR Integrative Imaging

LIFELONG LEARNING
FOR RADIOLOGY
Imaging of Paget Disease of Bone and Its
Musculoskeletal Complications: Self-Assessment Module
Daphne J. Theodorou
1
, Stavroula J. Theodorou
2
, Yousuke Kakitsubata
3
Keywords: bone diseases, osteitis deformans, Paget disease
DOI:10.2214/AJR.10.7303
Received October 5, 2010; accepted without revision October 5, 2010.
1
Department of Radiology, Ioannina General Hospital, Ioannina, Greece.
2
Department of Radiology, Ioannina University Hospital, 13 Papadopoulos St, Ioannina 45444, Greece. Address correspondence to S. J. Theodorou.
3
Department of Radiology, Miyazaki Konan Hospital, Miyazaki, Japan.
AJR 2011; 196:WS53WS56 0361803X/11/1966WS53 American Roentgen Ray Society
ABSTRACT
The educational objectives for this self-assessment mod-
ule are for the participant to exercise, self-assess, and im-
prove his or her skills in diagnostic radiology with regard to
imaging of Paget disease of bone and its musculoskeletal
complications.
INTRODUCTION
This self-assessment module on imaging of Paget disease
of bone and its musculoskeletal complications has a self-
assessment component and an educational component. The
educational component consists of two required articles
that the participant should read. The self-assessment com-
ponent consists of 10 multiple-choice questions with solu-
tions. All of these materials are available on the ARRS
Website (www.arrs.org). To claim CME and SAM credit,
each participant must enter his or her responses to the ques-
tions online.
EDUCATIONAL OBJECTIVES
By completing this educational activity, the participant will
exercise, self-assess, and improve his or her understanding of:
A. How to use imaging to recognize and diagnose Paget dis-
ease of bone.
B. How to use imaging to recognize and diagnose musculo-
skeletal complications of Paget disease of bone.
REQUIRED READING
1. Theodorou DJ, Theodorou SJ, Kakitsubata Y. Imaging
of Paget disease of bone and its musculoskeletal com-
plications: review. AJR Integrative Imaging 2011;
196[suppl 2]:S64S75
RECOMMENDED READING
1. Smith SE, Murphey MD, Motamedi K, Mulligan ME,
Resnik CS, Gannon FH. From the archives of the AFIP:
radiologic spectrum of Paget disease of bone and its
complications with pathologic correlation. RadioGraph-
ics 2002; 22:11911216
INSTRUCTIONS
1. Complete the educational and self-assessment compo-
nents included in this issue.
2. Visit www.arrs.org and log in.
3. Select Self-Assessment Modules from the Lifelong Learn-
ing box in the lower left of the page.
4. Add the SAM to your shopping cart and order the online
SAM as directed. (The SAM, including questions, must
be ordered to be accessed even though the activity is free
to ARRS members.) After purchasing the SAM, click on
OK; you will be returned to the ARRS home page.
5. Click on the My Education tab at the top of the page,
then on My Online Products. (Note: You must be logged
in to access this personalized page.)
6. You can also access the purchased SAM by logging on to
http://edu.arrs.org/myProducts/.
7. Answer the questions online to obtain SAM credit.
1.5 CME
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Theodorou et al.
WS54 AJR:196, June 2011
QUESTION 1
What is the etiologic origin of Paget disease?
A. A viral infection.
B. A genetic disorder.
C. A neoplasmic process.
D. The etiology is unknown.
QUESTION 2
Which of the following pathologic characteristics is the
hallmark of Paget disease of bone?
A. Increased bone resorption.
B. Abundant new bone formation.
C. Abnormal bone resorption and apposition.
D. Compensatory formation of good-quality bone.
QUESTION 3
Which of the following patient groups is most prone to
Paget disease of bone?
A. People in Asia and Africa.
B. Women.
C. Middle-aged and elderly persons.
D. People with degenerative disorders of the spine.
QUESTION 4
Paget disease of bone is more common in which of the
following locations?
A. Lumbosacral spine.
B. Shoulders.
C. Ribs.
D. Small bones in the hand.
QUESTION 5
Which segment of the spine is most often involved in
Paget disease?
A. Lumbar.
B. Sacral.
C. Cervical.
D. Thoracic.
QUESTION 6
What percentage of patients with Paget disease exhibit
monostotic disease?
A. 5%.
B. 1035%.
C. 4560%.
D. 6590%.
QUESTION 7
In clinical terms, patients with Paget disease:
A. Are uniformly asymptomatic.
B. Present with pathologic fracture.
C. Experience skeletal complications only.
D. Have clinical symptoms and signs that vary with the
distribution of the disease.
QUESTION 8
Which biochemical index is the most useful for
monitoring activity of Paget disease?
A. Alkaline phosphatase.
B. Serum osteocalcin.
C. Pyridinium cross links.
D. Serum calcium.
QUESTION 9
On MR images of Paget disease, which feature best
differentiates pagetic involvement from a sarcomatous
transformation?
A. Low T1- and T2-weighted signal intensity.
B. Signal intensity similar to that of fatty marrow.
C. Decreased internal T1-weighted signal intensity.
D. High internal signal on T2-weighted sequences.
QUESTION 10
What is the current mainstay of treatment in Paget
disease of bone?
A. Calcitonin.
B. Bisphosphonates.
C. Mithramycin.
D. Surgery.
Solution to Question 1
Virus inclusions similar to those detected in pagetic osteo-
clasts have been identifed in other disease processes as well
[1]. Option A is not the best response. Genetic factors and a
neoplastic origin have been associated with pathogenesis of
Paget disease, but the precise role and contribution of these
components in causing Paget disease remain unknown [2].
Options B and C are not the best responses. Option D is the
best response.
Solution to Question 2
Paget disease is characterized by abnormal bone remod-
eling with highly exaggerated bone resorption and apposi-
tion [2]. Option C is the best response. The primary event in
Paget disease is intense focal resorption followed by disor-
derly bone formation. Thus, increased bone resorption and
abundant new bone formation are both components of the
pagetic process, following one another. Options A and B are
not the best responses. The end result of this anarchic bone
behavior is formation of disorganized, weakened new bone.
Option D is not the best response.
Solution to Question 3
Paget disease is common in Australia, New Zealand,
Western Europe, and the United States, and it is rare in
Scandinavia, Asia, the Middle East, and Africa [3, 4]. Op-
tion A is not the best response. Epidemiologic studies show
that there is a slight male predilection in the condition. Op-
tion B is not the best response. Also, studies have indicated
that the frequency of Paget disease increases remarkably
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AJR:196, June 2011 WS55
Imaging of Paget Disease
with advancing age [3, 59]. Option C is the best response.
Epidemiologic data have not reported Paget disease to oc-
cur more frequently in people with degenerative diseases of
the spine. Option D is not the best response.
Solution to Question 4
Paget disease most commonly affects the lumbar spine
(3075% of cases), the pelvis (3075%), the sacrum (30
60%), the femur (2535%), and the cranium (2565%) [10].
Option A is the best response. The shoulder girdle, particu-
larly the proximal humerus (31%) and scapula (24%), are
less commonly affected sites [11]. Option B is not the best
response. Pagetic involvement of the ribs, fbulas, and small
bones in the hands and feet is infrequent [2]. Options C and
D are not the best responses.
Solution to Question 5
The anatomic distribution of Paget disease is usually
asymmetric and most commonly affects the lumbar spine
(3075%), the pelvis (3075% of cases), the sacrum (30
60%), the femur (2535%), and the cranium (2565%) [10].
Option A is the best response. Although sacral involvement
with Paget disease is common, it is less frequent than lumbar
involvement; thus, option B is not the best response. Less
frequently, however, cervical and thoracic involvement can
be observed [2]. Options C and D are not the best responses.
Solution to Question 6
Polyostotic disease (6590%) is more frequent than
monostotic disease [2, 9]. In some patients, the disease is
initially or totally monostotic, a pattern that is evident in
1035% of cases [2]. Option B is the best response. Options
A, C, and D are not the best responses.
Solution to Question 7
In Paget disease, almost one ffth of persons with skeletal
involvement detectable on radiographs are entirely asymp-
tomatic, so osseous abnormality is diagnosed frst as an in-
cidental fnding on radiographs obtained for unrelated pur-
poses [2]. Option A is not the best response. When present,
pathologic fracture, refecting the structural weakness of
the altered bone, with resulting pain and angulation or re-
duced mobility of joints and secondary osteoarthritis can
be crippling [11, 12, 13]. Option B is not the best response.
The disease is a painful and deforming process manifested
occasionally with severe symptoms and signs that may in-
clude various skeletal, neuromuscular, and cardiovascular
complications [8, 9, 14]. Option C is not the best response.
Clinical symptoms vary with the distribution of the disease.
Option D is the best response.
Solution to Question 8
Patients with Paget disease have a characteristic elevation
of the serum alkaline phosphatase level secondary to intense
osteoblastic activity. Alkaline phosphatase level is considered
an important parameter, refecting overall disease activity [1,
15]. Option A is the best response. Serum osteocalcin levels
may be elevated as well but are a less reliable index of disease
activity [15]. Option B is not the best response. Other useful
indexes of bone resorption are the pyridinium cross links [1,
15], whereas serum calcium level is usually normal unless
fracture or secondary hyperparathyroidism occurs [2]. Op-
tions C and D are not the best responses.
Solution to Question 9
In the late blastic inactive phase, pagetic bone shows low
signal intensity on both T1- and T2-weighted images, suggest-
ing the presence of compact bone or fbrous tissue. Option A
is not the best response. Preservation of fatty marrow signal
in pagetic bone generally excludes diagnosis of superimposed
sarcoma [2]. Option B is the best response. In the early mixed
active phase, involved bone shows heterogeneous, low T1-
weighted signal intensity and high T2-weighted signal intensi-
tyalso referred to as the speckled appearancethat cor-
responds to granulation tissue, hypervascularity, and edema
[3]. Options C and D are not the best responses.
Solution to Question 10
Calcitonin generally inhibits bone resorption and provides
timely pain relief, although fares in bone resorption may oc-
cur with cessation of therapy, necessitating retreatment [16].
Option A is not the best response. The current mainstay of
treatment in Paget disease, however, is the second-generation
bisphosphonates (disodium pamidronate, alendronate, rise-
dronate), which are potent inhibitors of bone resorption [17].
Option B is the best response. Mithramycin is a cytotoxic an-
tibiotic that is best reserved for those cases resistant to other
forms of medical treatment. Option C is not the best re-
sponse. Surgical treatmentthat is, total joint replace-
mentis generally reserved for those patients with severe
articular involvement and is associated with various compli-
cations [12, 14, 18]. Option D is not the best response.
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F O R Y O U R I N F O R M AT I O N
The readers attention is directed to the review article on which this SAM is based, which begins on page S64.
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This article has been cited by:
1. Dennis M. MarchioriSpine Patterns 1010-1060. [CrossRef]
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