EVIDENCE FOR SECULAR CHANGE IN PAGETS-DISEASE

Death certification data has shown that death rates due to Paget's dis ease of bone and osteosarcoma in older people (assumed to be attributa ble to Paget's) declined in the latter part of the 19th and in the ear ly 20th century, suggesting that there may be a secular trend toward l ess severe disease. We have reviewed a 21 year experience in a clinic specializing in Paget's disease. Data from all 1041 patients attending the clinic in this period were reviewed. Despite an increase in the s usceptible population and an increased rate of referral to the clinic over this time (p = 0.012), there was a fall in the absolute numbers o f patients referred with severe disease, as judged by the initial plas ma alkaline phosphatase activity at presentation. In the years 1973-19 78 the initial plasma alkaline phosphatase was >500 U/L in an average of 22 new patients per year and >1000 U/L in 12 per year. In the years 1988-1993, the figures were 12 and 3 per year, respectively. During t his period, there were no other facilities offering scintigraphy or in travenous treatment for Paget's disease in the Auckland region, making it unlikely that patients with severe disease were being seen and tre ated elsewhere. The average age of newly referred patients rose steadi ly from a mean 62 years, in 1971-1973 to 71 years in 1991-1993 (p < 0. 001), 534 subjects had scintiscans (52%) from which the extent of skel etal involvement was calculated. Skeletal involvement showed a signifi cant negative correlation with year of birth (p < 0.01) but not with a ge or year of presentation. The proportion of patients with >20% skele tal involvement had fallen by a third in the cohort born after 1926, c ompared to the cohort born before 1915. Our data demonstrate that, on average, newly referred patients with Paget's disease have less severe disease and are significantly older at diagnosis than was the case tw o decades ago. (C) 1997 by Elsevier Science Inc.