Hypertrophic pulmonary osteoarthropathy

A clinical syndrome of clubbing of the fingers and toes, enlargement of the extremities, and painful, swollen joints.

Manifested by symmetric periostitis involving the radius and fibula, femur, humerus, metacarpals, and metatarsals.

This clinical triad of digital clubbing, arthralgias, and ossifying periostitis presently known as hypertrophic ostearthropathy, and also known as Pi2242e Marie–Bamberger disease.

Clubbed fingers reveal hypervascularization of the distal digits.

Can be primary or secondary in nature, with approximately 3-5% of cases being primary, known as pachydermoperiostosis.

Solitary fibrous tumors represent less than 5% of pleural tumors and has been associated with hypertrophic pulmonary osteoarthropathy in up to 20% of patients.

Primary hypertrophic osteoarthropathy occurs without an underlying cause and is usually familial, and chronic.

Some patients with primary disease eventually develop diseases that underly causes of secondary hypertrophic osteoarthropathy, as late as 6-20 years after the onset of the osteoarthropathy.

95-97% have secondary HPO.

Secondary process is associated with an underlying pulmonary, cardiac, hepatic, or intestinal disease and often has a more rapid course than the primary syndrome.

Pulmonary processes represent the major causes of periostitis.

Plain x-rays are the mainstay of imaging studies, with the major radiographic feature is periostitis, which is seen as symmetric osseous thickening mostly affecting the tubular bones of the limbs, especially the radius, ulna, tibia, and fibula, although the pelvis, carpus, tarsus, metacarpals, metatarsals, and phalanges may be involved.

A paraneoplastic syndrome that occurs in 5-12% of patients with non-small cell lung cancer.

The paraneoplastic syndrome includes clubbing, arthralgias, arthritis and periositis of long bones.

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