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Forestier’s Disease

Forestier’s Disease, also known as Diffuse Idiopathic Skeletal Hyperostosis (DISH), is a rare and distinctive form of idiopathic spondylosis, that is, a spinal disorder of unknown cause. It is also frequently associated with disturbances in carbohydrate and/or protein and/or lipid metabolism, as well as with tendon calcifications.

EPIDEMIOLOGY

The disease typically manifests in adulthood, after the age of 50, and is more common in males. At present, there is no evidence of genetic conditions predisposing to the development of the disease.

ETIOLOGY, PATHOGENESIS, AND CLINICAL FEATURES OF FORESTIER’S DISEASE

The etiology and pathogenesis are essentially unknown: the mechanisms by which the disease develops and the initiating cause of the pathogenic process remain unclear.

As mentioned above, Forestier’s disease usually appears in adulthood, more often after the age of 50, and in about 50% of cases occurs in individuals with other metabolic disorders, particularly diabetes mellitus, hyperuricemia, and/or dyslipidemia. Obesity is also more frequent in patients with Forestier’s disease compared to the general population. However, the relationship between the disease and these metabolic disturbances is not yet clearly established.

The most affected skeletal region is the spine, where tendon calcifications and even ossification of the anterior longitudinal ligament may occur. This leads to the formation of large intervertebral osteophytic bridges, especially prominent in the lumbar and cervical segments, which interfere with spinal mobility.

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A characteristic feature of the thoracic spine is the “flowing candle wax” appearance: the calcification of the longitudinal ligament is continuous, producing a typical radiological pattern resembling melted candle wax.

In fact, this disease is defined as a polyenthesopathy, meaning it can affect multiple tendon insertions, with calcifications also seen in the rotator cuff, formation of heel spurs, and more.

Fortunately, pain and limitation of spinal mobility are often not severe, despite the striking radiological findings.

In the scientific literature, cases of dysphagia and even respiratory difficulties—mimicking obstructive sleep apnea syndrome (OSAS)—have been described, caused by compression of the soft tissues of the neck due to severe cervical osteophytosis.

DIAGNOSIS

Diagnosis is both radiological and clinical. In the differential diagnosis, ankylosing spondylitis should be considered, as it also involves the sacroiliac joints (which are not affected in Forestier’s disease) and is associated with more severe pain. The presence of metabolic disturbances may also aid in differential diagnosis.

TREATMENT

There is no curative treatment for Forestier’s disease. Management typically involves a combination of anti-inflammatory and analgesic therapy as needed, together with physical therapy.

It is hypothesized, though not confirmed, that correction of associated metabolic disorders may help mitigate disease progression.

Surgical intervention is sometimes required to remove large osteophytes causing critical compression of organs or blood vessels.

REFERENCE LINK:

PubMed search page

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Article with attached radiological images


Dr. Salvatore Nicolosi

General Practitioner contracted with the Italian National Health Service


Original in Italian translated with CHATGPT

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