Download Hypertrophic Osteoarthropathy

Roxanne Landesman
Gillian Lieberman, MD
September 2005
Hypertrophic Osteoarthropathy
Roxanne Landesman, Harvard Medical School Year III
Gillian Lieberman, MD
Roxanne Landesman
Gillian Lieberman, MD
Hypothetical Patient
• A patient presents with persistent right
ankle pain, and no history of trauma.
• As the 3rd year medical student reviews
the plain film with the radiologist, he is
immediately concerned about the
patient’s …
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Roxanne Landesman
Gillian Lieberman, MD
Hypothetical Case
• A patient presents with persistent right
ankle pain, and no history of trauma.
• As the 3rd year medical student reviews
the plain film with the radiologist, he is
immediately concerned about the
patient’s …
Lungs!
•He recommends a plain film of the contralateral ankle,
and later, a chest film.
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Roxanne Landesman
Gillian Lieberman, MD
Right ankle
•Opaque line of new
bone formation,
•Separated from
underlying cortex by
narrow radiolucent
line
¾Ossifying
periostitis
http://myweb.lsbu.ac.uk/ dirt/museum/p7-262.html
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Roxanne Landesman
Gillian Lieberman, MD
Later …
• The contralateral ankle showed similar
signs.
• A chest film revealed a small mass in the
left lung.
• The patient’s cancer was detected solely
because of ankle pain!
• The medical student had remembered
Hypertrophic pulmonary osteoarthropathy.
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Roxanne Landesman
Gillian Lieberman, MD
Hypertrophic Osteoarthropathy
(HOA)
• Clinical Syndrome:
ƒ Pain in the extremities (often lower)
+/- Clubbing of fingers and toes
+/- Articular symptoms (30-40% of HOA pts)
• Pain, tenderness, swelling
• May mimic rheumatoid arthritis
+/- limb skin thickening
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Roxanne Landesman
Gillian Lieberman, MD
Agenda
•
•
•
•
•
•
•
•
Introduction to HOA
Radiologic menu of tests
Radiologic findings
Concise presentation of three patients’
findings
Summary of HOA findings
Diseases associated with HOA
Pathophysiology of HOA?
Take home message
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Roxanne Landesman
Gillian Lieberman, MD
3-5%
Classification of
HOA
Primary
Secondary
9597%
=Pachydermoperiostosis
Associated with:
Genetic: autosomal dominant
•Intrathoracic, extrathoracic;
neoplastic, infectious,
inflammatory disease processes
Lung cancers
Inflammatory
bowel dz
mesothelioma
Cystic fibrosis
tuberculosis
Hepatobiliary dz
…
lymphoma
thymoma
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Roxanne Landesman
Gillian Lieberman, MD
Patients with HOA
• May or may not have bony symptoms
– Pain, when present, may be minor to severe
• May or may not have clubbing of the digits
• May be patients with a known associated
disease (lung cancer, IBD, …)
• May be patients whom you suspect have a
new metastasis to the lung
• May appear perfectly healthy and present to
you only for bone pain.
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Roxanne Landesman
Gillian Lieberman, MD
Radiologic Examinations of Choice
• Plain film
– Fast, simple, cheap, readily available
– Likely test for patients presenting with limb pain
• Nuclear medicine: bone scan
– Most sensitive
– May also detect metastases
• CT
– Detect underlying cause of 2° HOA
• MRI
– Non-standard
Non yield findings specific for HOA
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Roxanne Landesman
Gillian Lieberman, MD
HOA: Radiologic Findings
• Ossifying periostitis
– [usually] symmetric
– Long bones most common
• Distal shaft, progressing proximally [usually]
– may also affect metacarpals, metatarsals, scapulae, other
bones
• Radiological Differential Diagnosis:
– Thyroid acropachy, chronic venous stasis,
hypervitaminosis A, infantile hyperostosis (Caffey Dz),
even “shin splints” …
Must combine with clinical history and presentation to
achieve specific diagnosis.
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Roxanne Landesman
Gillian Lieberman, MD
Patient A
• 53yo male presented 1 year ago with hematuria,
diagnosed with renal cell carcinoma
• Nephrectomy performed; bone scan negative for
metastases
• Lung metastases detected by CT at 6 month f/u;
patient asymptomatic for lung disease
• Over the next six months, patient develops
increasingly severe pain bilaterally in knees,
lower limbs, wrists, hands – uncontrolled by
oxycodone, celecoxib, indomethacin
• [new] clubbing was recently noted
• Current bone scan:
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Roxanne Landesman
Gillian Lieberman, MD
Increased tracer
uptake in distal
femurs, fibulae,
tibiae, …
Renal osteodystrophy
vs. 2° HOA?
- Lab values and history
of pulmonary mets Æ
2° HOA more likely
BIDMC PACS
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Roxanne Landesman
Gillian Lieberman, MD
2° HOA parallels 1 ° disease
• All HOA findings (clinical, radiographic,
scintigraphic) generally disappear with
effective treatment of primary disease
(often within days!)
• Recurrence of primary disease is often
heralded by recurrence of HOA.
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Roxanne Landesman
Gillian Lieberman, MD
Patient B
• 53-year-old male smoker presented with
bilateral lower-limb pain of the hips, knees, and
ankles
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http://www.emedicine.com/radio/topic357.htm
Roxanne Landesman
Gillian Lieberman, MD
Patient B, continued
Biopsy confirmed
squamous carcinoma
http://www.emedicine.com/radio/topic357.htm
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Roxanne Landesman
Gillian Lieberman, MD
Patient C
•29yo female with
inflammatory
bowel disease
•c/o 3 months of
worsening shin
pain
Lateral view showing
increased activity in
the tibial periosteum
(“railroad sign”)
BIDMC PACS
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Roxanne Landesman
Gillian Lieberman, MD
HOA, Summary of Classic
Findings
• Periostitis
– Commonly:
• Bilateral
• Long bones
+/- bone and joint pain
http://www2.odn.ne.jp/~cdp10030/HP/housha18.jpg
+/- Clubbing
BIDMC PACS
+/- other
skin
changes
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www.emedicine.com/radio/topic357.htm
Roxanne Landesman
Gillian Lieberman, MD
2° HOA: Associated Disease
Processes
• Neoplasms or states of chronic infection or
inflammation:
– Pulmonary
•
•
•
•
•
•
Bronchogenic carcinoma (1-12% of these pts)
Other primary lung tumors
Metastases
Cystic Fibrosis
Infections: TB, histo, blasto, pneumocystis
…
Approx
90% are
intrathoracic
– Pleural: mesothelioma, pleural fibroma, …
– Cardiac: cyanotic heart dz with right to left shunt, …
– Intestinal and hepatic: cirrhosis, hepatocellular carcinoma,
lymphoma, other neoplasms, polyposis, inflammatory bowel dz, dysentery,
…
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Roxanne Landesman
Gillian Lieberman, MD
HOA: Pathophysiology
• Unknown/Debated
• Latest theories note that most associated dz
processes involve some degree of RÆL shunting of
blood, thus allowing large platelets to escape
fragmentation in the pulmonary microvasculature and
thus impact in distal capillary beds. There they may
stimulate endothelial cell activation via PDGF, etc,
thus stimulating connective tissue matrix synthesis
leading to clubbing, …
– HOA has been induced in dogs by creating RÆL shunts
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Roxanne Landesman
Gillian Lieberman, MD
A Final Point
• If you detect any aspect of the HOA
syndrome, especially in a previously
healthy patient,
Be on guard for the possibility of a serious
associated disease process!
Focus on the chest, but don’t forget other
possible sites.
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Roxanne Landesman
Gillian Lieberman, MD
References
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Ali A, Tetalman MR, Fordham EW et al. Distribution of hypertropic pulmonary
osteoarthropathy. Am J Radiol 1980; 134: 771-780
Martinez-Lavin M: Hypertrophic osteoarthropathy. Curr Opin Rheumatol 9:83–86, 1997
Andres R, Saenz A et al. Case 4. Hypertrophic osteoarthropathy associated with pulmonary
metastasis of uterine leiomyosarcoma.
J Clin Oncol. 2003 Sep 15;21(18):3540-1
Faulhaber PF. Nuclear and SPECT Teaching Files - Case One -Hypertrophic
Osteoarthropathy. http://www.uhrad.com/spectarc/nucs001.htm. accessed 17 Sep 2005
Rothschild BM, Rothschild C: Recognition of hypertrophic osteoarthropathy in skeletal
remains. J Rheumatol 1998 Nov; 25(11): 2221-7
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Roxanne Landesman
Gillian Lieberman, MD
Acknowledgements
Thanks to
• J. Anthony Parker, MD PhD
• Gillian Lieberman, MD
• Larry Barbaras, our webmaster
• Pamela Lepkowski for her wise advice
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