Forestier disease

Description

Diffuse idiopathic skeletal hyperostosis (DISH), the now more generally accepted name for Forestier disease; is a poorly understood, systemic condition characterised by progressive calcification and ossification of ligaments and entheses. Mainly affecting the thoracic spine, peripheral joints and adjacent entheses can also be involved.

Diagnostic criteria

The most commonly used diagnostic criteria are those set out by Resnick and Niwayama (1975):

• (a) The presence of “flowing” calcification and ossification along the anterolateral aspects of at least 4 contiguous vertebral bodies with or without associated localised pointed excrescences at the intervening vertebral body-disc junctions.
• (b) A relative preservation of disc height in the involved areas and the absence of extensive radiographic changes of “degenerative” disc disease, including vacuum phenomena and vertebral body marginal sclerosis.
• (c) Absence of apophyseal joint bony ankylosis and sacro-iliac joint erosion, sclerosis or bony fusion.

Clinical significance

Although DISH is asymptomatic in most individuals, the condition is often an indicator of underlying metabolic disease, and the presence of spinal or extraspinal ossifications can sometimes lead to symptoms including pain, stiffness, a reduced range of articular motion, and dysphagia, as well as increasing the risk of unstable spinal fractures. The aetiology of DISH is poorly understood, and the roles of the many factors that might be involved in the development of excess bone are not well delineated.

The pathogenesis of DISH is not clear, but several factors may promote the differentiation of mesenchymal cells into bone-forming cells. DISH is often associated with a variety of metabolic derangements, which may increase cardiovascular morbidity Patients with DISH also have an increased risk of complicated spinal fractures, with associated morbidities.

History

1897 – Pierre Marie and Charles Astié gave the name “cyphose hérédo-traumatique” to an old man suffering with angular kyphosis following a fall.

1904 – André Léri (1875-1930) describes pathological changes in the spine of a patient suffering from a similar condition to that described as ‘cyphose hérédo-traumatique‘.

1938 – Meyer and Forster described a similar anatomical condition under the name of “moniliform hyperostosis” affecting the right side of the dorsal spine.

1942 – Albert Oppenheimer (1900-1979) noticed some ossification of vertebral ligaments in old people without involvement of the joint facets. These patients had adequate vertebral mobility and no symptoms. He considered that these features, which he termed Spondylitis Ossificans Ligamentosa, belonged to the type of ossification associated with ankylosing spondylitis.

1949 – Lacapere, in his study of osteophytosis of the spine in dried bones often mentioned outgrowths which he called “melorheostosis of the spine”, a term that may lead to confusion with the disease described under this name by Léri.

1950 – Jacques Forestier (1890-1978) and Jaume Rotés-Quérol (1921-2008) presented their paper Senile Ankylosing Hyperostosis of the spine at the joint meeting between Ligue Française contre le rhumatisme and the Heberden Society, held in Paris, June 1950.

We consider that we have laid down clinical, radiological, and pathological criteria which differentiate a new ankylosing condition of the spine from ankylosing spondylitis. We propose to call this condition “senile ankylosing hyperostosis of the spine“. This name emphasizes: (a) the hyperostosis, a most striking pathological element, and (b) two constant clinical features: spinal rigidity and advanced age. It is certainly not a rare disease, but most often either goes unnoticed or is mistaken for some other form of vertebral ossification.

Forestier and Rotés-Quérol 1950

Associated Persons

• André Léri (1875-1930)
• Albert Oppenheimer (1900-1979)
• Jacques Forestier (1890-1978)
• Jaume Rotés-Quérol (1921-2008)
• Victor Rudolf Ott (1914-1986)
• Donald Resnick (1941-present)
• Gen Niwayama (1929-1986)

Alternative names

• Spondylitis deformans, Spondylitis ossificans ligamentosa
• Hyperostosis vertebralis ankylotica
• Forestier’s disease
• Forestier-Ott disease (common in Germany)
• Ankylosing hyperostosis of Forestier and Rotes-Querol
• Diffuse idiopathic skeletal hyperostosis, DISH
• Senile ankylosing spondylosis, ankylosing hyperostosis

References

Historical references

• Marie P, Astié C. Sur un cas de cyphose hérédo-traumatique. Presse médical 1897; 5: 205-6
• Léri A. Contribution a l’ étude de la pathologie du rachis. Autopsie d’ un cas de cyphose hérédo-traumatique. Bulletins et mémoires de la Société Médicale des Hôpitaux de Paris 1904; 21: 875-880
• Knaggs RL. Spondylitis deformans. British Journal of Surgery, 1925; 12(47): 524–546
• Meyer M, Forster E. Revue du Rhumatisme 1938; 5: 286.
• Oppenheimer A. Calcification and Ossification of Vertebral Ligaments (Spondylitis Ossificans Ligamentosa): Roentgen Study of Pathogenesis and Clinical Significance Radiology 1942; 38: 160-173
• Forestier J, Rotes-Querol J. Senile ankylosing hyperostosis of the spine. Ann Rheum Dis. 1950 Dec;9(4):321-30.
• Ott VR. De l’hyperostose ankylosante vertébrale sénile. Rhumatologie 1952; 3: 104–109
• Ott VR. Zur Frage der Senilen ankylosierenden Hyperostose der Wirbelsäule (Forestier-Rotès) [Senile ankylosing hyperostosis of the vertebral column (Forestier-Rotès)]. Zeitschrift für Rheumaforschung 1952; 11: 95-105

Eponymous term review

• Forestier J. Ankylosing spondylitis at the beginning of the century–illustrations. Rheumatism. 1964; 20: 52-53
• Forestier J, Lagier R. Vertebral ankylosing hyperostosis. Morphological basis, clinical manifestations, situation and diagnosis. Mod Trends Rheumatol. 1971;2:323-37.
• Forestier J, Lagier R. Ankylosing hyperostosis of the spine. Clin Orthop Relat Res. 1971 Jan;74:65-83.
• Resnick D, Shaul SR, Robins JM. Diffuse idiopathic skeletal hyperostosis (DISH): Forestier’s disease with extraspinal manifestations. Radiology. 1975 Jun;115(3):513-24
• Resnick D, Niwayama G. Radiographic and pathologic features of spinal involvement in diffuse idiopathic skeletal hyperostosis (DISH). Radiology. 1976 Jun;119(3):559-68.
• Resnick D, Shapiro RF, Wiesner KB, Niwayama G, Utsinger PD, Shaul SR. Diffuse idiopathic skeletal hyperostosis (DISH) [ankylosing hyperostosis of Forestier and Rotes-Querol]. Semin Arthritis Rheum. 1978 Feb;7(3):153-87. 
• Utsinger PD. Diffuse Idiopathic Skeletal Hyperostosis. Clinics in Rheumatic Diseases 1985; 11(2): 325-351
• Mader R, Jorrit-Jan V, Buskila D. Diffuse idiopathic skeletal hyperostosis: clinical features and pathogenic mechanisms Nat Rev Rheumatol. 2013 Dec;9(12):741-50.
• Pillai S, Littlejohn G. Metabolic factors in diffuse idiopathic skeletal hyperostosis – a review of clinical data. Open Rheumatol J. 2014 Dec 19;8:116-28
• Holgate RL, Steyn M. Diffuse idiopathic skeletal hyperostosis: Diagnostic, clinical, and paleopathological considerations. Clin Anat. 2016 Oct;29(7):870-7.
• Pappone N, Ambrosino P, Di Minno MND, Iervolino S. Is diffuse idiopathic skeletal hyperostosis a disease or a syndrome? Rheumatology (Oxford). 2017 Oct 1;56(10):1635-1636.

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