Ann. rheum. Dis. (1974), 33,210 Ann Rheum Dis: first published as 10.1136/ard.33.3.210 on 1 May 1974. Downloaded from Ankylosing hyperostosis 1. Clinical and radiological features JACQUELINE HARRIS, A. R. CARTER, E. N. GLICK, AND G. 0. STOREY From the Department ofRheumatology, Hackney Hospital, and the Department ofRadiology, The London Hospital, London Ankylosing hyperostosis is a condition in which new ankylosing hyperostosis, there has been no report ofa bone is laid down on the right antero-lateral aspect of series of patients with this condition from this the dorsal vertebrae and across the intervertebral country. This paper describes the clinical and radio- spaces forming spurs or bridges. The lumbar and logical features of a series of patients seen at various cervical spines, pelvis, hips, and knees may also be hospitals in the East End of London. Particular involved. There is no single approved name for this reference has been made to the possible association condition. Oppenheimer (1942) described 'Calcifi- with acromegaly. cation and ossification of vertebral ligaments'. Forestier and Rotes-Querol (1950) named it 'senile Material and methods ankylosing hyperostosis of the spine', but in later For inclusion in this survey, the following radiological works (Forestier and Lagier, 1971) the adjective criteria had to be fulfilled: hypertrophic spurs in the dorsal 'senile' was omitted when the condition was described spine predominantly on the right side with at least two in younger patients. Smith, Pugh, and Polley (1955) bony bridges; absence of other spinal disease which might copyright. described 'physiologic vertebral ligamentous calcifi- cause bony bridging (e.g. collapsed vertebra or gross cation' and Haijkova, Streda, and Skrha (1965) scoliosis); absence of sacroiliitis. The study was purposely 'hyperostotic spondylosis'. Beardwell (1969) termed limited to patients with definite and severe involvement. this condition 'ankylosing vertebral hyperostosis' and All patients were questioned about symptoms referable to Julkunen, Heinonen, and Py6rala (1971) referred to the spine and peripheral joints. The reason for attending 'hyperostosis of the spine'. Bywaters and Forestier hospital, trauma to back, occupation, racial origin, and history of diabetes mellitus in the patient or his family (1967) presented a revised system for nomenclature of were also recorded. Spinal movements were graded as http://ard.bmj.com/ spinal diseases at the 6th European Congress of normal, slightly restricted, severely restricted, or nil. The Rheumatology. They advised that this condition be height and weight of each patient were measured, and any named 'ankylosing hyperostosis', and this is the clinical evidence of acromegaly, tylosis, or psoriasis was terminology which we have adopted. noted. An association with diabetes mellitus has been Antero-posterior and lateral radiographs were taken shown by several authors, including Boulet and of the entire spine, and radiographs of the pelvis, hands, Mirouze, (1954), Hajkova' and others (1965), knees, and skull were also obtained in nearly all patients. Bywaters, Doyle, and Oakley (1966), and Ott, All observed abnormalities in the spinal films were noted on September 27, 2021 by guest. Protected with particular reference to the presence of bony spurs or Perkovac, and Regehr (1967). Julkunen and others bridges, their locations (i.e. right, left, anterior, or a (1971) found an association with diabetes and obesity. combination) and to the presence of new bone on the Boulet, Serre, and Mirouze (1954) suggested that anterior surfaces of the vertebral bodies. When present, this condition is due to an increased production of intervertebral disc narrowing with sclerosis of the adjacent growth hormone occurring when the secretion of vertebral plates, apophyseal joint involvement, and osteo- gonadal hormones declines, and in fact, Julkunen, porosis were also recorded. The name 'bony spur' was Karava, and Viljanen (1966) found six cases of used for a projection from a vertebral body which extended hyperostosis of the spine in 21 acromegalic patients. in a vertical direction either up or down beyond the Beardwell (1969) showed a familial incidence and end-plate. The term 'bridge' was used when there appeared to be bony union between two such spurs. The term relationship with tylosis in a Greek Cypriot family. 'osteophyte' was reserved for a bony projection from the Ott (1953) and Smith and others (1955) considered the margin of a vertebral body which tended to project disease to be an exaggerated form of osteoarthrosis laterally and did not extend vertically beyond the end- of the spine. plate of the vertebral body. In practice, this differentiation Although many European authors have described was usually possible, but there were a few examples which the clinical, radiological, and pathological features of were difficult to classify, particularly in the cervical region. Accepted for publicaiton October 24, 1973. Address for correspondence: Dr A. R. Carter, Department of Radiology, The London Hospital, London El 1BB Ankylosing hyperostosis, I 211 Ann Rheum Dis: first published as 10.1136/ard.33.3.210 on 1 May 1974. Downloaded from In difficult cases, bony projections in the anterior and CERVICAL SPINE posterior directions which were associated with disc Eleven patients complained of severe pain in the space narrowing were termed osteophytes. The films of cervical spine whilst five experienced mild discomfort. the hands, knees, and pelvis were assessed for evidence of All had radiological abnormalities-seven hyperos- osteoarthrosis and the skull and hands for acromegalic three a combina- changes. tosis, six cervical spondylosis, and The following laboratory investigations were per- tion of the two conditions. formed: haemoglobin, white cell count, erythrocyte sedi- mentation rate, latex fixation, serum calcium, phosphorus, PERIPHERAL JOINTS alkaline phosphatase, and spot blood sugar. The urine in the was tested for glucose. In five of the younger patients 24 patients complained of pain peripheral serum growth hormone levels were estimated by the joints: shoulders (10), knees (8), hips (5), feet and method of Hartog, Gaafar, Meisser, and Fraser (1964). ankles (5), hands (4). Two patients had more generalized pain characteristic of polymyalgia rheumatica. Twenty patients were suffering from Results various pathological conditions: ischaemic heart disease (4), bronchitis (2), gout (1), chondrocalcinosis (1) Clinical (6), thyrotoxicosis (1), gallstones (1), hiatus hernia 34 patients were studied: seventeen men aged 47 to 82 (1), duodenal ulcer (1), old poliomyelitis (1), Paget's years (mean 68) and seventeen women aged 47 to 83 disease (2). years (mean 66). Only eleven came from the British There were four diabetics (duration 1 to 20 years), Isles. Nineteen were Jewish-usually from Russia or but only one required insulin therapy. Four others Poland-two West Indian, one Cypriot, and one had a family history of diabetes mellitus in siblings or Italian. This racial distribution probably reflects the mother. None ofthe non-diabetics had glycosuria and number of immigrants living in this area rather than all had spot blood sugars of less than 180 mg./100 ml. a racial predominance. Glucose-tolerance tests were performed in six patients No patient gave a history of spinal trauma and and all were normal. none had done heavy manual work. Five patients had Using tables for average weights of adults (Docu-copyright. no backache but the rest had pain in some region of menta Geigy, 1970), 30 patients were overweight. the back. Thirteen had Heberden's nodes. None appeared clinically acromegalic, and none had psoriasis or DORSAL SPINE tylosis. Thirteen patients had had some pain in the dorsal One patient was anaemic with a haemoglobin of spine; of these, two had experienced pain many years 10 8 g. per cent. and an erythrocyte sedimentation previously and six had only mild intermittent dis- rate of 60 mm./lst hr. No cause has been found for http://ard.bmj.com/ comfort. The remaining five complained of more these abnormalities. The blood count and sedimen- definite pain which was variously described as con- tation rate were normal in all other patients at the stant, worse on coughing, worse on lifting, brief, time of survey. The latex-fixation test was negative in nocturnal, and to the right of the spine. The duration all cases and calcium and phosphorus estimations of symptoms ranged from 2 to 28 years. Five com- were normal in the 30 patients who had these investi- plained of stiffness of the dorsal spine. There was no gations performed. The main laboratory test to show relationship between symptoms and the extent of any abnormality was the alkaline phosphatase level on September 27, 2021 by guest. Protected dorsal spine involvement as seen radiologically. Two which was mildly raised in seven cases. patients derived some relief from indomethacin or Growth hormone levels were estimated in five of phenylbutazone. the younger patients (three women aged 67, 59, and Rotation of the dorsal spine was tested with the 56 years and two men aged 47 and 58 years). None patient sitting with arms folded. 28 had some limi- was diabetic. All had normal growth hormone levels, tation of movement, but none had a completely rigid i.e. fasting up to 60 pg./litre suppressing to less than spine. There was a mild kyphosis in seven patients. 10,ug./litre in response to glucose. LUMBAR SPINE 23 patients had pain in the lumbar spine; the descrip- (2) Radiological tion was usually vague but was not usually that of a The most characteristic pattern of bony spurs and disc lesion. Five complained of stiffness. Movements bridges was seen in the dorsal spine, where they were usually limited, but the lumbar spine was never predominated on the right and anterior aspects of the rigid although forward flexion and extension were vertebrae, particularly in the lower dorsal region with limited in 25 patients and lateral flexion in 29. the maximum number being situated at the D8/9 Nineteen patients with symptoms referable to the level (Figs 1, 2 and 3).