Search

Paget's disease of bone (PDB) manifests fairly late in life and currently affects up to 3% of individuals older than 55 years of age. An about 60% decline in the prevalence of PDB between 1970 and 1990 was documented in England, particularly in Lancashire, where the disease was initially most common. Although wide regional variations exist, the overall prevalence is decreasing, as confirmed by a recent meta-analysis. This secular decline is associated with a decrease in mortality due to the disappearance of sarcomatous transformation of PDB lesions. Another reported change is a decrease in the severity of the clinical PDB phenotype, with a gradual increase in the age at diagnosis of 4 years per decade. In familial forms related to an SQSTM1 mutation, the first manifestations are detected about 10 years later from one generation to the next, and the disease is less severe, with smaller elevations in serum alkaline phosphatase levels and less extensive lesions. Although incompletely understood, the reasons for these changes might involve environmental factors such as the correction of childhood deficiencies in calcium and/or vitamin D, a less rural lifestyle, and decreased contact with domestic animals and consumption of bovine organ meats during childhood. Childhood exposure to industrial waste and products of combustion has also been incriminated. Finally, although classical, the role for paramyxoviruses such as the measles virus in the pathogenesis of PDB remains debated in the literature., (Copyright © 2016 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.)

Purpose: The radiologist plays a critical role at all steps of the management of patients with fibrous dysplasia (FD) and McCune-Albright syndrome (MAS). The development of a standardized approach to the management of FD/MAS is crucial given the low incidence and multiple clinical presentations of these conditions. Our aim was to develop recommendations for bone imaging in FD/MAS management., Materials and Methods: The establishment of National Reference Centers in France as part of a Health Ministry program for orphan diseases has triggered the development of recommendations for the clinical management of FD/MAS. We used a well-established robust methodological approach involving an extensive literature review by a multidisciplinary working group (20 healthcare professionals) and scoring by a peer-review group (20 healthcare professionals different from the 20 previous ones). There were four phases: a systematic literature review, drafting of initial recommendations, peer-review of this initial draft, and drafting of the final recommendations., Results: Fifty-seven specific recommendations are provided as key points for the diagnosis, prognosis, and follow-up of patients with FD/MAS. Issues of special interest are highlighted in the discussion, and areas in which future research is needed are identified., Conclusion: We believe the dissemination of these recommendations within the radiology community may facilitate communication between radiologists and other healthcare providers, thereby substantially improving the management of patients with these rare but potentially disabling conditions., (Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.)

Gout is a common disease. Although effective treatments are available for gout, there is some disagreement as to how they should be used. To study prescription patterns in gout, we conducted a questionnaire survey among 2520 rheumatologists. Seven hundred and fifty completed questionnaires were returned over a two-month period. Among respondents, 35.4% worked in a private office, 21% in a hospital and 43.6% in both. The most widely prescribed treatments in acute gout attack were colchicine alone (63%), colchicine with a nonsteroidal antiinflammatory drug (NSAID) (31.7%) and NSAID alone (5.2%), with significant variations according to the type of practice. Mean duration of treatment in acute gout was 18 +/- 16.8 days (range, 3-180 days). Mean time interval between the attack and initiation of therapy with a xanthine oxidase inhibitor was 21.6 +/- 17.2 days (range 0-180); here also, significant variations were seen according to the type of practice. Concomitant symptomatic therapy was prescribed in 97.3% of cases, for a mean duration of 54 +/- 55.4 days (range 2-365). Thirty per cent of responders never prescribed uricosuric agents. The estimated rate of occurrence of treatment-induced attacks increased with the reported interval between the attack and initiation of urate-lowering therapy. Our data demonstrate that French rheumatologists have widely diverging views on how to treat gout. Whether a waiting period is needed between an acute attack and initiation of urate-lowering therapy, and how long this period should be, are unsettled issues that deserve to be studied.