Osteitis Deformans: Paget's Disease of the Bone

Osteitis deformans was described by Sir James Paget (1) in 1876 and now bears his name. Very little has been added to his explicit observations on the advanced clinical form of the disease. Biochemical, pathologic, and roentgenologic investigations have made it possible, however, to segregate osteitis deformans in its preclinical stage from a large group of forms of benign osteitis. At the Mayo Clinic prior to January 1938, a diagnosis of osteitis deformans was made in 367 cases. The analysis of the findings in that group will be given in this paper. It is not a new observation that osteitis deformans manifests itself roentgenologically in various forms. In this study, however, we shall attempt to correlate the clinical manifestations of the disease with the roentgenologic evidence in a series of 200 cases. The criteria for the roentgenographic classification will be discussed later. Historical Review Both Wrany, in 1867, and Wilks (2), in 1869, as quoted by Paget, deserve credit for recognizing the clinical form of osteitis deformans. The two cases described by those men were among the five recorded in Paget's original paper. Paget was inclined to believe that the disease was of recent origin, but paleopathologic evidence based on old museum specimens is an indication of its presence lone before modern times. Denninger (3) described gross morphologic and roentgenologic evidence of osteitis deformans in the skeletons of five American Indians, excavated from the Illinois River valley. Fisher (4) reported additional paleopathologic evidence of the condition in two tibiae; the disease is well described and well illustrated in his paper. Following the examination of a number of skulls in the South Kensington Natural History Museum, Butlin (5) expressed the opinion that the Neanderthal skull had the appearance of a specimen representing osteitis deformans. Etiology As the term osteitis deformans implies, Paget (1) believed the disease to be a chronic inflammatory process. Fairly frequently, local heat may be demonstrated in the involved bones, particularly in those which are easily palpable, as for example the tibia and even occasionally the ilium. DaCosta et al. (6) referred to the work of Morpure, Archangeli and Fiocca,, who claimed to have found a diplococcus in the bones of patients suffering from osteitis deformans and osteomalacia. DaCosta and his associates unsuccessfully attempted to prepare a vaccine, but cultures and animal inoculation gave negative results. Knaggs (7 and 8) expressed the belief that a toxin was responsible for osteitis deformans. In his opinion the proliferative changes were more prominent than the vascular disturbances, and evidence of leukocytic migration was minimal. Such changes, when not to be explained on a bacterial basis, may be the result of repeated slight mechanical injuries, or more often the effects of perverted metabolism and continued slight intoxication, affecting particularly certain tissues.