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Madrid, Spain

Vaglio A.,University of Parma | Palmisano A.,University of Parma | Alberici F.,University of Parma | Maggiore U.,University of Parma | And 6 more authors.
The Lancet

Glucocorticoids are the mainstay of treatment of idiopathic retroperitoneal fibrosis, but they often have substantial toxic effects. Several reports have suggested tamoxifen as an alternative to glucocorticoids. We compared the efficacy of prednisone with that of tamoxifen in maintainance of remission in patients with idiopathic retroperitoneal fibrosis. In this open-label, randomised controlled trial, we enrolled patients aged 18-85 years with newly diagnosed idiopathic retroperitoneal fibrosis at the Parma Hospital, Parma, Italy, between Oct 1, 2000, and June 30, 2006. After induction therapy with 1 mg/kg daily of prednisone for 1 month, the patients who achieved remission were randomly assigned to receive tapering prednisone (initial dose 0·5 mg/kg daily) for 8 months or tamoxifen (fixed dose 0·5 mg/kg daily) for 8 months. The sequence of randomisation (1:1), blocked in groups of two and four (with block size randomly selected), was generated by the trial statistician with a computer programme. After the end of treatment, the patients were followed up for an additional 18 months. Neither patients nor those giving interventions or analysing the data were masked to group assignment. The two radiologists who assessed CT and MRI scans were masked. The primary endpoint was the relapse rate by the end of treatment (month 8), which was analysed by intention to treat. This trial is registered with ClinicalTrials.gov, number NCT00440349. After induction therapy, 36 of the 40 enrolled patients achieved remission and were randomly assigned to treatment (18 per group). One patient (6) in the prednisone group and seven patients (39) in the tamoxifen group relapsed by the end of treatment (difference -33 [95 CI -58 to -8, p=0·0408]. The difference in relapse rate between the groups was sustained after the additional 18-month follow-up: the 26-month estimated cumulative relapse probability was 17 with prednisone and 50 with tamoxifen (difference -33 [-62 to -3, p=0·0372]). Cushingoid changes and grade 2 hypercholesterolaemia were more common in the prednisone group than in the tamoxifen group (p=0·0116 and p=0·0408, respectively). Prednisone is more effective in prevention of relapses than is tamoxifen in patients with idiopathic retroperitoneal fibrosis. Therefore, prednisone should be considered as first-line treatment for patients with newly diagnosed idiopathic retroperitoneal fibrosis. Parma University Hospital. © 2011 Elsevier Ltd. Source

Akikusa J.D.,Rheumatology Service | Akikusa J.D.,Murdoch Childrens Research Institute
Pediatric Clinics of North America

This article presents five clinical scenarios in which the initial manifestations of pediatric rheumatic diseases constitute life-threatening medical emergencies. It is intended as a problem-oriented guide for pediatricians to assist in the recognition of rheumatologic differentials in children presenting with critical illness and provides an approach to their initial investigation and management. © 2012 Elsevier Inc.. Source

Colina M.,Rheumatology Service | Trotta F.,University of Ferrara
Current Rheumatology Reviews

The peculiar bone involvement, represented by osteitis, is the common denominator of SAPHO syndrome. Hyperostosis and osteitis are chronic inflammatory reactions involving the cortical and trabecular bone respectively; both are characterised by increased sclerosis. Hyperostosis appears radiologically as chronic endosteal and periosteal thickening with narrowing of the medullary canal, but areas of ostelysis may also be present. Conversely, osteitis appears as increased osteosclerosis involving the trabecular infrastructure of cancellous bone. The occurrence of hyperostosis with little or no osteitis is not uncommon. SAPHO syndrome may have a prolonged course with phases of reacutization and remission; the long-term prognosis is usually fairly good, but sometimes a disabling course may occur. Our experience demonstrated that the majority of patients suffering from SAPHO syndrome experienced a chronic course, requiring continous treatment, whilst in a third of the cases the patients reported multiple remission and exacerbations of the disease with flares lasting till to 8 months. Only in a minority of cases the bone inflammation faded and never recurred. Female sex, peripheral arthritis, ACW involvement, the coexistence of more than one cutaneous symptoms, and high inflammatory indices are correlated with a chronic disease course and involvement of new osteoarticular sites. © 2013 Bentham Science Publishers. Source

Alvarez-Nemegyei J.,Medical Research Unit | Pelaez-Ballestas I.,Hospital General de Mexico | Sanin L.H.,Autonomous University of Chihuahua | Cardiel M.H.,Research Unit Dr Mario Alvizouri Munoz | And 2 more authors.
Journal of Rheumatology

To assess the prevalence of musculoskeletal (MSK) pain and rheumatic diseases in the southeastern Mexican state of Yucatán. Methods: Using the Community Oriented Program in the Rheumatic Diseases (COPCORD) methodology, we performed a door-to-door, cross-sectional study generated through a multistage, stratified, randomized method on 3915 adult residents (age 42.7 ± 17.1 yrs; women 61.8%; urban setting 45.7%) of the Mexican state of Yucatán. We used universally accepted criteria for the diagnosis or classification of rheumatoid arthritis (RA), osteoarthritis (OA; knee and hand), fibromyalgia, systemic lupus erythematosus (SLE), gout, ankylosing spondylitis, regional rheumatic pain syndromes, and inflammatory back pain. Results: Nontraumatic MSK pain in the last 7 days was present in 766 (19.6%; 95% CI 18.3-20.8) individuals. MSK pain was more prevalent in women (26.6%) versus men (12.2%; p < 0.01). Self-reported MSK disability occurred in 1.7%. Most MSK pain-related variables were consistently more prevalent in the urban setting. The prevalence of rheumatic disease was: OA 6.8% (95% CI 6.0-7.6); back pain 3.8% (95% CI 3.2-4.4); RA 2.8% (95% CI 2.2-3.3); rheumatic regional pain syndromes 2.3% (95% CI 1.9-2.8); inflammatory back pain 0.7% (95% CI 0.5-1.0); fibromyalgia 0.2% (95% CI 0.1-0.4); gout 0.1% (95% CI 0.07-0.3); and SLE 0.07% (95% CI 0.01-0.2). Conclusion: The prevalence of MSK pain was 19.6%. MSK pain was more prevalent in women and in the urban setting. A remarkably high prevalence of RA was found in this population, which suggests a role for geographic factors. Source

Kanterewicz E.,Rheumatology Unit | Puigoriol E.,Epidemiology Unit | Garcia-Barrionuevo J.,Radiology Unit | Del Rio L.,Densitometry Unit | And 2 more authors.
Osteoporosis International

Summary: Population-based studies performed with vertebral fracture assessment (VFA) morphometric technology are lacking in postmenopausal osteoporosis. In this study, we show a lower than expected prevalence of vertebral fractures, a high prevalence of minor vertebral deformities, and a clear association with clinical and densitometric parameters indicating the usefulness of this approach. Introduction: Adequate epidemiological data on the prevalence of vertebral fractures (VF) is essential in studies of postmenopausal osteoporosis. Routine DXA-assisted VFA may be useful to determine the presence of VF. However, population-based studies performed with this technology are lacking. We aimed to assess the prevalence of VF and minor deformities in 2,968 postmenopausal women aged 59-70 years from a population-based cohort. Methods: VFA and bone mineral density (BMD) measurements were conducted, and McCloskey criteria (vertebral heights under 3 SD from reference values) confirmed with the Genant method were used to define VF. Additionally, minor vertebral deformities (vertebral heights between -2 and -2.99 SD) were evaluated. Results: The prevalence of VF was 4.3 %, and 17 % of the participants had minor vertebral deformities. Low BMD was frequently observed in women with VF, with 48 %, and 42 % of participants showing osteoporosis and osteopenia. Minor vertebral deformities were observed in nearly 40 % of women with VF. Multivariate logistic regression analysis showed that age, history of previous fracture, osteoporotic BMD, receiving anti-osteoporotic treatment, and current use of glucocorticoids were significantly associated with VF. Conclusions: Although the VFA approach showed a lower than expected prevalence of VF in our cohort, its association with clinical and densitometric parameters may be useful to identify women at risk for developing fragility fractures and may therefore justify its use in longitudinal studies. The high prevalence of minor vertebral deformities detected in patients with VF indicates the need to evaluate this type of deformity as a risk factor for further skeletal fractures. © 2014 International Osteoporosis Foundation and National Osteoporosis Foundation. Source

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