Hypertrophic osteoarthropathy

Changed by Samer Alabed, 3 Jun 2018

Updates to Article Attributes

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Hypertrophic osteoarthropathyis characterised by periosteal reaction involving the diaphysis and metadiaphysis of the long bones of distal extremities without an underlying bone lesion. Clubbing of the fingers is seen most commonly in patients with lung, liver, and gastrointestinal disorders.When associated with a pulmonary condition, it is termed hypertrophic pulmonary osteoarthropathy (HPOA) and when associated with cancer is considered a paraneoplastic syndromePrimary hypertrophic osteoarthropathy (also known as Pachydermoperiostosis) is a genetic subtype that presents in children or young adults and involves the epiphyseal region of long bones.

Terminology

Hypertrophic osteoarthropathy has been referred to with multiple different terms, including: Pierre-Marie syndrome, Bamberger syndrome, osteoarthropatia hypertrophicaMankowsky syndrome and Hagner syndrome.

Clinical presentation

It is usually painful and associated with clubbing of the fingers or toes. 

Pathology

The causes of hypertrophic osteoarthropathy include:

Radiographic features

Plain radiograph

Typically seen as long bone metaphyseal and diaphyseal smooth periosteal reaction.

With disease progression, periostitis becomes more prominent or multilayered and extends to the epiphyses 1.

Nuclear medicine
Tc 99m MDP bone scan
  • symmetric linear increase in tracer accumulation along diaphyseal and metaphyseal surfaces of long bones 4

  • "tram-track" appearance 

Differential diagnosis

General imaging differential considerations include:

Consider the differential for a smooth periosteal reaction.

On bone scintigraphy, differentials include:

  • normal variant
    • lateral cortices of the tibiae often appear with a symmetric linear uptake 
  • shin splints
    • can appear similar, but confined to the tibiae
  • chronic venous insufficiency 
    • can cause symmetrical periosteal uptake
    • usually confined to the lower extremities below the knees 
  • -<p><strong>Hypertrophic osteoarthropathy</strong><em> </em>is characterised by periosteal reaction involving the diaphysis and metadiaphysis of the long bones of distal extremities without an underlying bone lesion. Clubbing of the fingers is seen most commonly in patients with lung, liver, and gastrointestinal disorders. When associated with a pulmonary condition, it is termed <strong>hypertrophic pulmonary osteoarthropathy (HPOA)</strong> and when associated with cancer is considered a <a href="/articles/paraneoplastic-syndromes">paraneoplastic syndrome</a>. </p><h4>Terminology</h4><p>Hypertrophic osteoarthropathy has been referred to with multiple different terms, including: <strong>Pierre-Marie syndrome</strong>, <strong>Bamberger syndrome</strong>, <strong>osteoarthropatia hypertrophica</strong>, <strong>Mankowsky syndrome</strong> and <strong>Hagner syndrome</strong>.</p><h4>Clinical presentation</h4><p>It is usually painful and associated with clubbing of the fingers or toes. </p><h4>Pathology</h4><p>The causes of <strong>hypertrophic osteoarthropathy</strong> include:</p><ul>
  • +<p><strong>Hypertrophic osteoarthropathy</strong><em> </em>is characterised by periosteal reaction involving the diaphysis and metadiaphysis of the long bones of distal extremities without an underlying bone lesion. Clubbing of the fingers is seen most commonly in patients with lung, liver, and gastrointestinal disorders.<br>When associated with a pulmonary condition, it is termed <strong>hypertrophic pulmonary osteoarthropathy (HPOA)</strong> and when associated with cancer is considered a <a href="/articles/paraneoplastic-syndromes">paraneoplastic syndrome</a>. <br><strong>Primary hypertrophic osteoarthropathy</strong> (also known as <a title="Pachydermoperiostosis" href="/articles/pachydermoperiostosis">Pachydermoperiostosis</a>) is a genetic subtype that presents in children or young adults and involves the epiphyseal region of long bones.</p><h4>Terminology</h4><p>Hypertrophic osteoarthropathy has been referred to with multiple different terms, including: <strong>Pierre-Marie syndrome</strong>, <strong>Bamberger syndrome</strong>, <strong>osteoarthropatia hypertrophica</strong>, <strong>Mankowsky syndrome</strong> and <strong>Hagner syndrome</strong>.</p><h4>Clinical presentation</h4><p>It is usually painful and associated with clubbing of the fingers or toes. </p><h4>Pathology</h4><p>The causes of <strong>hypertrophic osteoarthropathy</strong> include:</p><ul>
  • -<li><a href="/articles/primary-biliary-cirrhosis">primary biliary cirrhosis</a></li>
  • +<li><a href="/articles/primary-biliary-cholangitis">primary biliary cirrhosis</a></li>
  • -<li>familial (pachydermoperiostosis)</li>
  • +<li>familial (<a title="pachydermoperiostosis" href="/articles/pachydermoperiostosis">pachydermoperiostosis</a>)</li>

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