Hypertrophic osteoarthropathy

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Hypertrophic osteoarthropathy (also known as hypertrophic pulmonary osteoarthropathy, Bamberger-Marie syndrome or Osteoarthropathia hypertrophicans) is a medical condition combining clubbing and periostitis of the small hand joints, especially the distal interphalangeal joints and the metacarpophalangeal joints. Distal expansion of the long bones as well as painful, swollen joints and synovial villous proliferation are often seen. The condition may occur alone (primary), or it may be secondary to diseases like lung cancer. It is especially associated with non-small cell lung carcinoma. These patients often get clubbing and increased bone deposition on long bones. Their presenting symptoms are sometimes only clubbing and painful ankles.

Video Hypertrophic osteoarthropathy

Etiology

Hypertrophic osteoarthropathy is one of many distant effect disorders due to cancer, with lung cancer being the most common cause but also occurring with ovarian or adrenal malignancies. A distant effect disorder, or a paraneoplastic syndrome, affects distant areas and thus is not related to local compression or obstruction effects from the tumor. Other paraneoplastic syndromes include hypercalcemia, SIADH, Cushing's syndrome and a variety of neurological disorders.

Maps Hypertrophic osteoarthropathy

Diagnosis

Bone scan: shows parallel lines of activity along the cortex of the shafts and ends of tibiae,femurs and radii. especially around the knees, ankles and wrists. this activity may decrease after treatment of the underlying cause.

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Treatment

Non-steroidal anti-inflammatory drugs (NSAIDs) can give significant relief of the symptoms. Treatment of lung cancer or other causes of hypertrophic osteoarthropathy results in regression of symptoms for some patients.

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Etymology

The eponymous 'Bamberger-Marie syndrome' is named for Austrian internist Eugen von Bamberger and French neurologist Pierre Marie.

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See also

• Periosteal reaction
• Hypertrophic osteopathy

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References

• Rumney, W. J.; Schofield, Frank W. (1950). "Pulmonary Hypertrophic Osteo-Arthropathy (Marie's Disease)". Canadian Journal of Comparative Medicine and Veterinary Science. 14 (12): 385-392. PMC 1791230 . 

Source of article : Wikipedia