FROM AN ATLAS OF GENERAL AFFECTIONS OF THE SKELETON 15. PAGET’S DISEASE Synonym-Osteitis Deformans

This chronic disease is characterised by slowly spreading changes in one or more bones, the changes consisting of decalcification coupled with hyperostosis and with the marrow spaces in the transformed bone occupied, partially or completely, by vascular fibrous tissue. Czerny (1873) suggested the name osteitis deformans, but ever since the publication in 1877 of Paget’s paper, in which he described with characteristic lucidity the clinical features of eight cases, his name has been coupled universally with the disease. In 1908 Elmslie collected ninety cases and reviewed the whole subject, and in 1938 Brailsford discussed the changes in 154 cases which he had examined radiologically. Hereditary and familial influences are evident in some cases. In seven of Elmslie’s ninety cases other members of the family were affected. Since that date many instances have been recorded in parents and offspring, and of brothers and sisters. \Ve have twice met with two brothers afflicted with this disease. Age-The majority of cases are seen between fifty and seventy years of age. Though uncommon before the age of forty, several unquestionable cases have been diagnosed at thirty years. Since in some of these more than one bone was affected, the disease was unlikely to have been a recent development. Sex-Males are rather more commonly affected than females. Incidence-Paget’s disease is one of the obscure diseases of the skeleton which are relatively common, many cases being discovered incidentally when investigated radiologically for symptoms entirely unconnected with the bone condition. Incidence figures most likely to approach accuracy are obtainable only from radiological departments. Inquiry some ten years ago from the radiologists of the teaching hospitals of London revealed that the average annual number of Paget cases seen at each hospital was approximately fifty. Distribution-The disease may be confined to one bone, at least for a period of years. The tibia is a common site for a solitary lesion. Sooner or later in most cases several bones are affected : only exceptionally is the disease so widespread as to be almost generalised. Published lists of the bones most commonly affected, in order of frequency, vary, but they all agree in placing the pelvis at the head of the list. It showed changes in 243 cases out of a total of 367 reviewed by Dickson et al. (1945). The femur, tibia, lower spine and skull are all common sites. The humerus, clavicle and forearm bones are distinctly less common, while the hand and foot, with the possible exception of the tarsal bones, are seldom affected, a point in which the disease differs from polyostotic fibrous dysplasia. The ribs and the manubrium sterni -occasionally even the whole of the sternum-may be affected. \Vhen the skull is involved the facial bones very seldom show changes, an important point of difference from leontiasis ossea. In the tibia the changes spread from the upper end far more commonly than from the lower: the same is true of the femur. Changes may begin at both ends of a bone, but this is excessively uncommon. The distribution appears to be almost entirely erratic, and shows little if any tendency to be symmetrical. When, however, the pelvis is involved there is a greater chance than otherwise of finding the upper end of one or both femora and the sacrum and lower spine also showing changes.