Hypertrophic Osteoarthropathy - Lieberman's eRadiology Learning ...

Roxanne Landesman 

Gillian Lieberman, MD 

September 2005 

Hypertrophic Osteoarthropathy 

Roxanne Landesman, Harvard Medical School Year III 

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 … 

2



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. 

3



Right ankle 

•Opaque line of new 

bone formation, 

•Separated from 

underlying cortex by 

narrow radiolucent 

line 

Ossifying 

periostitis 

http://myweb.lsbu.ac.uk/ dirt/museum/p7-262.html 

4



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. 

5



Hypertrophic Osteoarthropathy 

• Clinical Syndrome: 

(HOA) 

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 

6



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 

7

3-5% 5% 



Primary 

=Pachydermoperiostosis 

Pachydermoperiostosis 

Genetic: autosomal dominant 

Classification of 

HOA 

Secondary 

Associated with: 

95- 95 

97% 

•Intrathoracic 

Intrathoracic, , extrathoracic; 

extrathoracic; 

neoplastic, neoplastic, 

infectious, 

inflammatory disease processes 

Lung cancers 

Inflammatory 

mesothelioma 

bowel 

mesothelioma 

bowel dz 

Cystic fibrosis 

tuberculosis 

Hepatobiliary dz lymphoma 

thymoma 

… 

8



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. 

9



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 

10



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. 

11



Patient A 

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: 

12



Increased tracer 

uptake in distal 

femurs, fibulae, 

tibiae, … 

Renal osteodystrophy 

vs. 2° 2 HOA? 

- Lab values and history 

of pulmonary mets 

2° ° HOA more likely 

BIDMC PACS 

13



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. 

14



Patient B 

• 53-year-old male smoker presented with 

bilateral lower-limb pain of the hips, knees, and 

ankles 

http://www.emedicine.com/radio/topic357.htm 

15



Patient B, continued 

Biopsy confirmed 

squamous carcinoma 

http://www.emedicine.com/radio/topic357.htm 

16



•29yo female with 

inflammatory 

bowel disease 

•c/o 3 months of 

worsening shin 

pain 

Patient C 

Lateral view showing 

increased activity in 

the tibial periosteum 

(“railroad sign”) 

BIDMC PACS 

17



HOA, Summary of Classic 

• Periostitis 

– Commonly: 

• Bilateral 

• Long bones 

+/- bone and joint pain 

+/- Clubbing 

+/- other 

skin 

changes 

Findings 

http://www2.odn.ne.jp/~cdp10030/HP/housha18.jpg 

www.emedicine.com/radio/topic357.htm 

BIDMC PACS 

18



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 

• … 

– Pleural: mesothelioma, pleural fibroma, … 

– Cardiac: cyanotic heart dz with right to left shunt, … 

– Intestinal and hepatic: cirrhosis, hepatocellular carcinoma, 

Approx 

90% are 

intra- intra 

thoracic 

lymphoma, other neoplasms, polyposis, inflammatory bowel dz, dysentery, 

… 

19



HOA: Pathophysiology 

• Unknown/Debated 

• Latest theories note that most associated dz 

processes involve some degree of RL 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 RL shunts 

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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. 

21



References 

• 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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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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