Hypertrophic osteoarthropathy

Hypertrophic osteoarthropathy is a syndrome characterised by periosteal reaction of the long bones without underlying bone lesion. There are a broad range of manifestations, although typically there is symmetrical involvement of the appendicular skeleton. Accompanying abnormal soft tissue proliferation is variable and may result in clubbing of the fingers and toes.

Hypertrophic osteoarthropathy is largely considered a secondary phenomenon, and nearly always (95-97% of cases) occurs in the setting of a wide variety of other cardiopulmonary, gastrointestinal, endocrine, haematologic, and inflammatory conditions 5. When associated with cancer is considered a paraneoplastic syndrome. A primary form, pachydermoperiostosis, is due to heritable genetic mutation that results in similar clinical manifestations, although tend to have more soft tissue findings 5.

Hypertrophic osteoarthropathy has been given various names including Pierre-Marie syndrome, Bamberger syndrome, osteoarthropatia hypertrophica, Mankowsky syndrome, and Hagner syndrome.

"Hypertrophic osteoarthropathy" may refer to either the primary or secondary syndrome, although general usage implies the secondary form, given the rarity of primary hypertrophic osteoarthropathy (pachydermoperiostosis).

"Hypertrophic pulmonary osteoarthropathy" specifically refers to HO in the setting of a lung disease, and not from other intrathoracic processes such as mediastinal or pleural disease 5.

HO has variable clinical presentation, ranging from asymptomatic morphologic and radiographic findings to pain accompanying the changes of the fingers and toes 5.

The primary form of hypertrophic osteoarthropathy is due to mutations in the genes encoding 15-hydroxyprostaglandin dehydrogenase and solute carrier organic anion transporter family member 2A1, resulting in abnormal accumulation of prostaglandin E2 5.

Although the cause of secondary hypertrophic osteoarthropathy is not established, a similar mechanism involving abnormally elevated levels of circulated hormones has been proposed as a potential etiology. Alternatively, neurogenic etiology has been proposed 5.

Associated disease states include:

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.

  • symmetric linear increase in tracer accumulation along diaphyseal and metaphyseal surfaces of long bones 4

  • "tram-track" appearance 

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 
Share article

Article information

rID: 1479
Section: Gamuts
Synonyms or Alternate Spellings:
  • HPOA
  • Hypertropic pulmonary osteoarthropathy
  • Hypertropic pulmonary osteoarthropathy (HPOA)
  • HOA
  • Hagner syndrome
  • Bamberger syndrome
  • Pierre-Marie syndrome
  • Mankowsky syndrome

Support Radiopaedia and see fewer ads

Cases and figures

  • Drag
    Case 1
    Drag here to reorder.
  • Drag
    Case 2
    Drag here to reorder.
  • Drag
    Case 3
    Drag here to reorder.
  • Drag
    Case 4
    Drag here to reorder.
  • Drag
    Case 5
    Drag here to reorder.
  • Drag
    Case 6
    Drag here to reorder.
  • Drag
    Case 7
    Drag here to reorder.
  • Drag
    Case 8
    Drag here to reorder.
  • Updating… Please wait.
    Loadinganimation

    Alert accept

    Error Unable to process the form. Check for errors and try again.

    Alert accept Thank you for updating your details.