Hypertrophic Osteoarthropathy - Lieberman's eRadiology Learning ...
Hypertrophic Osteoarthropathy - Lieberman's eRadiology Learning ...
Hypertrophic Osteoarthropathy - Lieberman's eRadiology Learning ...
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Roxanne Landesman<br />
Gillian Lieberman, MD<br />
September 2005<br />
<strong>Hypertrophic</strong> <strong>Osteoarthropathy</strong><br />
Roxanne Landesman, Harvard Medical School Year III<br />
Gillian Lieberman, MD
Roxanne Landesman<br />
Gillian Lieberman, MD<br />
Hypothetical Patient<br />
• A patient presents with persistent right<br />
ankle pain, and no history of trauma.<br />
• As the 3rd year medical student reviews<br />
the plain film with the radiologist, he is<br />
immediately concerned about the<br />
patient’s …<br />
2
Roxanne Landesman<br />
Gillian Lieberman, MD<br />
Hypothetical Case<br />
• A patient presents with persistent right<br />
ankle pain, and no history of trauma.<br />
• As the 3rd year medical student reviews<br />
the plain film with the radiologist, he is<br />
immediately concerned about the<br />
patient’s …<br />
Lungs!<br />
•He recommends a plain film of the contralateral ankle,<br />
and later, a chest film.<br />
3
Roxanne Landesman<br />
Gillian Lieberman, MD<br />
Right ankle<br />
•Opaque line of new<br />
bone formation,<br />
•Separated from<br />
underlying cortex by<br />
narrow radiolucent<br />
line<br />
Ossifying<br />
periostitis<br />
http://myweb.lsbu.ac.uk/ dirt/museum/p7-262.html<br />
4
Roxanne Landesman<br />
Gillian Lieberman, MD<br />
Later …<br />
• The contralateral ankle showed similar<br />
signs.<br />
• A chest film revealed a small mass in the<br />
left lung.<br />
• The patient’s cancer was detected solely<br />
because of ankle pain!<br />
• The medical student had remembered<br />
<strong>Hypertrophic</strong> pulmonary osteoarthropathy.<br />
5
Roxanne Landesman<br />
Gillian Lieberman, MD<br />
<strong>Hypertrophic</strong> <strong>Osteoarthropathy</strong><br />
• Clinical Syndrome:<br />
(HOA)<br />
Pain in the extremities (often lower)<br />
+/- Clubbing of fingers and toes<br />
+/- Articular symptoms (30-40% of HOA pts)<br />
• Pain, tenderness, swelling<br />
• May mimic rheumatoid arthritis<br />
+/- limb skin thickening<br />
6
Roxanne Landesman<br />
Gillian Lieberman, MD<br />
Agenda<br />
• Introduction to HOA<br />
• Radiologic menu of tests<br />
• Radiologic findings<br />
• Concise presentation of three patients’<br />
findings<br />
• Summary of HOA findings<br />
• Diseases associated with HOA<br />
• Pathophysiology of HOA?<br />
• Take home message<br />
7
3-5% 5%<br />
Roxanne Landesman<br />
Gillian Lieberman, MD<br />
Primary<br />
=Pachydermoperiostosis<br />
Pachydermoperiostosis<br />
Genetic: autosomal dominant<br />
Classification of<br />
HOA<br />
Secondary<br />
Associated with:<br />
95- 95<br />
97%<br />
•Intrathoracic<br />
Intrathoracic, , extrathoracic;<br />
extrathoracic;<br />
neoplastic, neoplastic,<br />
infectious,<br />
inflammatory disease processes<br />
Lung cancers<br />
Inflammatory<br />
mesothelioma<br />
bowel<br />
mesothelioma<br />
bowel dz<br />
Cystic fibrosis<br />
tuberculosis<br />
Hepatobiliary dz lymphoma<br />
thymoma<br />
…<br />
8
Roxanne Landesman<br />
Gillian Lieberman, MD<br />
Patients with HOA<br />
• May or may not have bony symptoms<br />
– Pain, when present, may be minor to severe<br />
• May or may not have clubbing of the digits<br />
• May be patients with a known associated<br />
disease (lung cancer, IBD, …)<br />
• May be patients whom you suspect have a<br />
new metastasis to the lung<br />
• May appear perfectly healthy and present to<br />
you only for bone pain.<br />
9
Roxanne Landesman<br />
Gillian Lieberman, MD<br />
Radiologic Examinations of Choice<br />
• Plain film<br />
– Fast, simple, cheap, readily available<br />
– Likely test for patients presenting with limb pain<br />
• Nuclear medicine: bone scan<br />
– Most sensitive<br />
– May also detect metastases<br />
• CT<br />
– Detect underlying cause of 2° HOA<br />
• MRI<br />
– Non-standard<br />
Non yield findings specific for HOA<br />
10
Roxanne Landesman<br />
Gillian Lieberman, MD<br />
HOA: Radiologic Findings<br />
• Ossifying periostitis<br />
– [usually] symmetric<br />
– Long bones most common<br />
• Distal shaft, progressing proximally [usually]<br />
– may also affect metacarpals, metatarsals, scapulae, other<br />
bones<br />
• Radiological Differential Diagnosis:<br />
– Thyroid acropachy, chronic venous stasis,<br />
hypervitaminosis A, infantile hyperostosis (Caffey Dz),<br />
even “shin splints” …<br />
Must combine with clinical history and presentation to<br />
achieve specific diagnosis.<br />
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Roxanne Landesman<br />
Gillian Lieberman, MD<br />
Patient A<br />
Patient A<br />
• 53yo male presented 1 year ago with hematuria,<br />
diagnosed with renal cell carcinoma<br />
• Nephrectomy performed; bone scan negative for<br />
metastases<br />
• Lung metastases detected by CT at 6 month f/u;<br />
patient asymptomatic for lung disease<br />
• Over the next six months, patient develops<br />
increasingly severe pain bilaterally in knees,<br />
lower limbs, wrists, hands – uncontrolled by<br />
oxycodone, celecoxib, indomethacin<br />
• [new] clubbing was recently noted<br />
• Current bone scan:<br />
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Roxanne Landesman<br />
Gillian Lieberman, MD<br />
Increased tracer<br />
uptake in distal<br />
femurs, fibulae,<br />
tibiae, …<br />
Renal osteodystrophy<br />
vs. 2° 2 HOA?<br />
- Lab values and history<br />
of pulmonary mets <br />
2° ° HOA more likely<br />
BIDMC PACS<br />
13
Roxanne Landesman<br />
Gillian Lieberman, MD<br />
2° HOA parallels 1 ° disease<br />
• All HOA findings (clinical, radiographic,<br />
scintigraphic) generally disappear with<br />
effective treatment of primary disease<br />
(often within days!)<br />
• Recurrence of primary disease is often<br />
heralded by recurrence of HOA.<br />
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Roxanne Landesman<br />
Gillian Lieberman, MD<br />
Patient B<br />
• 53-year-old male smoker presented with<br />
bilateral lower-limb pain of the hips, knees, and<br />
ankles<br />
http://www.emedicine.com/radio/topic357.htm<br />
15
Roxanne Landesman<br />
Gillian Lieberman, MD<br />
Patient B, continued<br />
Biopsy confirmed<br />
squamous carcinoma<br />
http://www.emedicine.com/radio/topic357.htm<br />
16
Roxanne Landesman<br />
Gillian Lieberman, MD<br />
•29yo female with<br />
inflammatory<br />
bowel disease<br />
•c/o 3 months of<br />
worsening shin<br />
pain<br />
Patient C<br />
Lateral view showing<br />
increased activity in<br />
the tibial periosteum<br />
(“railroad sign”)<br />
BIDMC PACS<br />
17
Roxanne Landesman<br />
Gillian Lieberman, MD<br />
HOA, Summary of Classic<br />
• Periostitis<br />
– Commonly:<br />
• Bilateral<br />
• Long bones<br />
+/- bone and joint pain<br />
+/- Clubbing<br />
+/- other<br />
skin<br />
changes<br />
Findings<br />
http://www2.odn.ne.jp/~cdp10030/HP/housha18.jpg<br />
www.emedicine.com/radio/topic357.htm<br />
BIDMC PACS<br />
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Roxanne Landesman<br />
Gillian Lieberman, MD<br />
2° HOA: Associated Disease<br />
Processes<br />
• Neoplasms or states of chronic infection or<br />
inflammation:<br />
– Pulmonary<br />
• Bronchogenic carcinoma (1-12% of these pts)<br />
• Other primary lung tumors<br />
• Metastases<br />
• Cystic Fibrosis<br />
• Infections: TB, histo, blasto, pneumocystis<br />
• …<br />
– Pleural: mesothelioma, pleural fibroma, …<br />
– Cardiac: cyanotic heart dz with right to left shunt, …<br />
– Intestinal and hepatic: cirrhosis, hepatocellular carcinoma,<br />
Approx<br />
90% are<br />
intra- intra<br />
thoracic<br />
lymphoma, other neoplasms, polyposis, inflammatory bowel dz, dysentery,<br />
…<br />
19
Roxanne Landesman<br />
Gillian Lieberman, MD<br />
HOA: Pathophysiology<br />
• Unknown/Debated<br />
• Latest theories note that most associated dz<br />
processes involve some degree of RL shunting of<br />
blood, thus allowing large platelets to escape<br />
fragmentation in the pulmonary microvasculature and<br />
thus impact in distal capillary beds. There they may<br />
stimulate endothelial cell activation via PDGF, etc,<br />
thus stimulating connective tissue matrix synthesis<br />
leading to clubbing, …<br />
– HOA has been induced in dogs by creating RL shunts<br />
20
Roxanne Landesman<br />
Gillian Lieberman, MD<br />
A Final Point<br />
• If you detect any aspect of the HOA<br />
syndrome, especially in a previously<br />
healthy patient,<br />
Be on guard for the possibility of a serious<br />
associated disease process!<br />
Focus on the chest, but don’t forget other<br />
possible sites.<br />
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Roxanne Landesman<br />
Gillian Lieberman, MD<br />
References<br />
• Ali A, Tetalman MR, Fordham EW et al. Distribution of hypertropic pulmonary<br />
osteoarthropathy. Am J Radiol 1980; 134: 771-780<br />
• Martinez-Lavin M: <strong>Hypertrophic</strong> osteoarthropathy. Curr Opin Rheumatol 9:83–86, 1997<br />
• Andres R, Saenz A et al. Case 4. <strong>Hypertrophic</strong> osteoarthropathy associated with pulmonary<br />
metastasis of uterine leiomyosarcoma.<br />
J Clin Oncol. 2003 Sep 15;21(18):3540-1<br />
• Faulhaber PF. Nuclear and SPECT Teaching Files - Case One -<strong>Hypertrophic</strong><br />
<strong>Osteoarthropathy</strong>. http://www.uhrad.com/spectarc/nucs001.htm. accessed 17 Sep 2005<br />
• Rothschild BM, Rothschild C: Recognition of hypertrophic osteoarthropathy in skeletal<br />
remains. J Rheumatol 1998 Nov; 25(11): 2221-7<br />
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Roxanne Landesman<br />
Gillian Lieberman, MD<br />
Acknowledgements<br />
Thanks to<br />
• J. Anthony Parker, MD PhD<br />
• Gillian Lieberman, MD<br />
• Larry Barbaras, our webmaster<br />
• Pamela Lepkowski for her wise advice<br />
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