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, it is considered a paraneoplastic syndrome. A primary form, pachydermoperiostosis, is due to a 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 (also known as pachydermoperiostosis).
"Hypertrophic pulmonary osteoarthropathy" specifically refers to hypertrophic osteoarthropathy in the setting of a lung disease, and not from other intrathoracic processes such as mediastinal or pleural disease 5.
Hypertrophic osteoarthropathy has a 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 aetiology. Alternatively, neurogenic aetiology has been proposed 5.
Associated disease states include:
- gastrointestinal tract and liver
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.
Tc-99m MDP bone scan
symmetric linear increase in tracer accumulation along diaphyseal and metaphyseal surfaces of long bones 4
General imaging differential considerations include:
- pachydermoperiostosis (primary hypertrophic osteoarthropathy)
- chronic venous insufficiency
- thyroid acropachy
- hypervitaminosis A
Consider the differential for a smooth periosteal reaction.
On bone scintigraphy, differentials include:
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- 5. Yap FY, Skalski MR, Patel DB, Schein AJ, White EA, Tomasian A, Masih S, Matcuk GR. Hypertrophic Osteoarthropathy: Clinical and Imaging Features. (2017) Radiographics : a review publication of the Radiological Society of North America, Inc. 37 (1): 157-195. doi:10.1148/rg.2017160052 - Pubmed