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Villajoyosa, Spain

Ramirez Huaranga M.A.,Reumatologia | Ramos Rodriguez C.C.,Daniel Alcides Carrion National University | Bellido Pastrana D.,Servicio de Medicina Interna
Reumatologia Clinica | Year: 2012

Cryoglobulinemia is a rare disease characterized by the production of monoclonal or polyclonal immunoglobulins that precipitate in cold temperature. While this phenomenon can be observed in a large number of disorders, it has been associated with hepatitis C virus infection in more than 90% of cases. The remaining 10%, called essential cryoglobulinemia, has been characterized by a more severe course and a failure to respond to conventional treatment. This article describes the case of a patient with essential cryoglobulinemia presenting with acronecrosis with a poor outcome, despite treatment, leading to amputation. © 2011 Elsevier España, S.L. Source

Azagra R.,Autonomous University of Barcelona | Azagra R.,International University of Catalonia | Roca G.,Autonomous University of Barcelona | Martin-Sanchez J.C.,International University of Catalonia | And 6 more authors.
Medicina Clinica | Year: 2015

Background and objective To detect FRAX® threshold levels that identify groups of the population that are at high/low risk of osteoporotic fracture in the Spanish female population using a cost-effective assessment.Patients and methods This is a cohort study. Eight hundred and sixteen women 40-90 years old selected from the FRIDEX cohort with densitometry and risk factors for fracture at baseline who received no treatment for osteoporosis during the 10 year follow-up period and were stratified into 3 groups/levels of fracture risk (low < 10%, 10-20% intermediate and high > 20%) according to the real fracture incidence.Results The thresholds of FRAX® baseline for major osteoporotic fracture were: low risk < 5; intermediate ≥ 5 to < 7.5 and high ≥ 7.5. The incidence of fracture with these values was: low risk (3.6%; 95% CI 2.2-5.9), intermediate risk (13.7%; 95% CI 7.1-24.2) and high risk (21.4%; 95% CI12.9-33.2). The most cost-effective option was to refer to dual energy X-ray absorptiometry (DXA-scan) for FRAX® ≥ 5 (Intermediate and high risk) to reclassify by FRAX® with DXA-scan at high/low risk. These thresholds select 17.5% of women for DXA-scan and 10% for treatment. With these thresholds of FRAX®, compared with the strategy of opportunistic case finding isolated risk factors, would improve the predictive parameters and reduce 82.5% the DXA-scan, 35.4% osteoporosis prescriptions and 28.7% cost to detect the same number of women who suffer fractures.Conclusions The use of FRAX ® thresholds identified as high/low risk of osteoporotic fracture in this calibration (FRIDEX model) improve predictive parameters in Spanish women and in a more cost-effective than the traditional model based on the T-score ≤ -2.5 of DXA scan. © 2013 Elsevier España, S.L.U. All rights reserved. Source

Morales-Angulo C.,Hospital Universitario Marques Of Valdecilla | Garcia-Zornoza R.,Hospital Universitario Marques Of Valdecilla | Obeso-Aguera S.,Hospital Universitario Marques Of Valdecilla | Calvo-Alen J.,Reumatologia | Gonzalez-Gay M.A.,Hospital Universitario Marques Of Valdecilla
Acta Otorrinolaringologica Espanola | Year: 2012

Introduction and objectives: Granulomatosis with polyangiitis (GPA), previously called Wegener's granulomatosis, is a small vessel vasculitis often associated with clinical head and neck manifestations, which are sometimes the presenting symptoms of the disease. The aim of our study was to identify ear, nose and throat (ENT) manifestations associated with GPA and propose a work-up for the management and diagnosis for patients with suspicion or confirmed diagnosis of this ENT pathology. Patients and methods: Retrospective review of the medical records of all patients diagnosed with GPA who were seen at the Department of Otolaryngology from a tertiary public hospital in Cantabria (Spain) over a 20-year period. Clinical and laboratory data, in particular those concerning ENT manifestations, were retrieved from the patients' medical records. Results: Twenty-five patients (age range: 30-81 years) were included in the study. Of these, 88% had ENT manifestations at some point in the course of the disease. In 28% of the cases, ENT features were the presenting manifestations. The most frequent ENT manifestations were sinonasal symptoms (52%), followed by otological manifestations (32%). Conclusions: Patients with GPA often present with clinical ENT manifestations. Consequently, routine ENT physical examination must be performed in patients with suspected vasculitis to establish a diagnosis of GPA or to better determine the degree of organ system involvement in patients with GPA. © 2011 Elsevier España, S.L. Source

Levy R.,Paediatric Rheumatology | Gerard L.,Hopital Saint Louis | Kuemmerle-Deschner J.,University of Tubingen | Lachmann H.J.,University College London | And 21 more authors.
Annals of the Rheumatic Diseases | Year: 2014

Objective To evaluate genetic, demographic and clinical features in patients with cryopyrin-associated periodic syndrome (CAPS) from the Eurofever Registry, with a focus on genotype-phenotype correlations and predictive disease severity markers. Methods A web-based registry retrospectively collected data on patients with CAPS. Experts in the disease independently validated all cases. Patients carrying NLRP3 variants and germline-mutation-negative patients were included. Results 136 patients were analysed. The median age at disease onset was 9 months, and the median duration of follow-up was 15 years. Skin rash, musculoskeletal involvement and fever were the most prevalent features. Neurological involvement (including severe complications) was noted in 40% and 12% of the patients, respectively, with ophthalmological involvement in 71%, and neurosensory hearing loss in 42%. 133 patients carried a heterozygous, germline mutation, and 3 patients were mutation-negative (despite complete NLRP3 gene screening). Thirty-one different NLRP3 mutations were recorded; 7 accounted for 78% of the patients, whereas 24 rare variants were found in 27 cases. The latter were significantly associated with early disease onset, neurological complications (including severe complications) and severe musculoskeletal involvement. The T348M variant was associated with early disease onset, chronic course and hearing loss. Neurological involvement was less strongly associated with V198M, E311 K and A439 V alleles. Early onset was predictive of severe neurological complications and hearing loss. Conclusions Patients carrying rare NLRP3 variants are at risk of severe CAPS; onset before the age of 6 months is associated with more severe neurological involvement and hearing loss. These findings may have an impact on treatment decisions. Source

Garcia M.A.,Reumatologia | Alarcon G.S.,University of Alabama at Birmingham | Boggio G.,National University of Rosario | Hachue L.,National University of Rosario | And 16 more authors.
Rheumatology (United Kingdom) | Year: 2014

Objectives. The aim of this study was to assess the cumulative incidence, risk and protective factors and impact on mortality of primary cardiac disease in SLE patients (disease duration ≥2 years) from a multiethnic, international, longitudinal inception cohort (34 centres, 9 Latin American countries). Methods. Risk and protective factors of primary cardiac disease (pericarditis, myocarditis, endocarditis, arrhythmias and/or valvular abnormalities) were evaluated. Results. Of 1437 patients, 202 (14.1%) developed one or more manifestations: 164 pericarditis, 35 valvulopathy, 23 arrhythmias, 7 myocarditis and 1 endocarditis at follow-up; 77 of these patients also had an episode of primary cardiac disease at or before recruitment. In the multivariable parsimonious model, African/Latin American ethnicity [odds ratio (OR) 1.80, 95% CI 1.13, 2.86], primary cardiac disease at or before recruitment (OR 6.56, 95% CI 4.56, 9.43) and first SLICC/ACR Damage Index for SLE assessment (OR 1.31, 95% CI 1.14, 1.50) were risk factors for the subsequent occurrence of primary cardiac disease. CNS involvement (OR 0.44, 95% CI 0.25, 0.75) and antimalarial treatment (OR 0.62, 95% CI 0.44, 0.89) at or before recruitment were negatively associated with the occurrence of primary cardiac disease risk. Primary cardiac disease was not independently associated with mortality. Conclusion. Primary cardiac disease occurred in 14.1% of SLE patients of the Grupo Latino Americano de Estudio de Lupus cohort and pericarditis was its most frequent manifestation. African origin and lupus damage were found to be risk factors, while CNS involvement at or before recruitment and antimalarial treatment were protective. Primary cardiac disease had no impact on mortality. © The Author 2014. Published by Oxford University Press. All rights reserved. Source

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