Hypertrophic osteoarthropathy is a medical condition combining clubbing and

periostitis Periostitis, also known as periostalgia, is a medical condition caused by inflammation of the periosteum, a layer of connective tissue that surrounds bone. The condition is generally chronic, and is marked by tenderness and swelling of the bone an ...

of the small hand joints, especially the

distal interphalangeal joint Distal interphalangeal joints are the articulations between the phalanges of the hand or foot. This term therefore includes: * Interphalangeal joints of the hand * Interphalangeal joints of the foot The interphalangeal joints of the foot are bet ...

s and the

metacarpophalangeal joint The metacarpophalangeal joints (MCP) are situated between the metacarpal bones and the proximal phalanges of the fingers. These joints are of the condyloid kind, formed by the reception of the rounded heads of the metacarpal bones into shallow cav ...

s. 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 Lung cancer, also known as lung carcinoma (since about 98–99% of all lung cancers are carcinomas), is a malignant lung tumor characterized by uncontrolled cell growth in tissue (biology), tissues of the lung. Lung carcinomas derive from tran ...

. Among patients with lung cancer, it is most associated with adenocarcinoma and least associated with small cell lung cancer. These patients often get clubbing and increased bone deposition on long bones. Their presenting signs and symptoms are sometimes only clubbing and painful ankles.

Cause

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 A paraneoplastic syndrome is a syndrome (a set of signs and symptoms) that is the consequence of a tumor in the body (usually a cancerous one), specifically due to the production of chemical signaling molecules (such as hormones or cytokines) by ...

, affects distant areas and thus is not related to local compression or obstruction effects from the tumor. Other paraneoplastic syndromes include hypercalcemia,

SIADH Syndrome of inappropriate antidiuretic hormone secretion (SIADH) is characterized by excessive unsuppressible release of antidiuretic hormone (ADH) either from the posterior pituitary gland, or an abnormal non-pituitary source. Unsuppressed ADH ca ...

Cushing's syndrome Cushing's syndrome is a collection of signs and symptoms due to prolonged exposure to glucocorticoids such as cortisol. Signs and symptoms may include high blood pressure, abdominal obesity but with thin arms and legs, reddish stretch marks, ...

and a variety of neurological disorders. Thought to be due to fibrovascular proliferation caused by accumulation of megakaryocytes in the digital vessels which are normally filtered by the lungs.

Diagnosis

People with hypertrophic osteoarthropathy may have bone scans showing 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.

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.

Etymology

The eponymous 'Bamberger–Marie syndrome' is named for Austrian internist

Eugen von Bamberger Eugen von Bamberger (5 September 1858 – October 1921) was an Austrian internist born in Würzburg, Germany. He was the son of pathologist Heinrich von Bamberger (1822–1888). He studied medicine at the Universities of Vienna and Würzburg, rec ...

and French

neurologist Neurology (from el, νεῦρον (neûron), "string, nerve" and the suffix -logia, "study of") is the branch of medicine dealing with the diagnosis and treatment of all categories of conditions and disease involving the brain, the spinal c ...

Pierre Marie Pierre Marie (9 September 1853 – 13 April 1940) was a French neurology, neurologist and political journalist close to the French Section of the Workers' International, SFIO. Medical Career After finishing medical school, he served as an int ...

References

External links