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Reggio nell'Emilia, Italy

Salaffi F.,Marche Polytechnic University | Franchignoni F.,Unit of Occupational Rehabilitation and Ergonomics | Giordano A.,Unit of Bioengineering | Ciapetti A.,Marche Polytechnic University | And 2 more authors.
Clinical and Experimental Rheumatology

Objectives. The aim of this study was to perform a psychometric analysis of the Italian Fibromyalgia Impact Questionnaire Revised version (FIQR), using both classical test theory (CTT) and Rasch analysis (RA) in order to better analyse its construct validity and provide a rational basis for a possible improvement of its metric quality. Methods. The study involved 503 patients with fibromyalgia (FM) (423 women and 80 men) with mean age of 51.3±10.1 years (range 19-74) and mean duration of symptoms of 11.1±8.7 years (range 1-30). All patients completed the Italian FIQR during their clinical visit. The translation and cultural adaptation process of the Italian FIQR followed the published guidelines and no local adjustments were made except for a slight adaptation of item 13 related to "energy". Results. Factor Analysis revealed two salient dimensions: function (items 1-9) and symptoms (items 12-21). RA was thus performed on these two subscales. Rating scale diagnostics suggested collapsing the eleven rating categories of the scale into five. After combining these rating categories, RA showed that most items of each of the two subscales fitted the respective constructs to measure (MnSq 0.7-1.3). The reliability levels of the two subscales were higher than 0.80. Conclusion. This study provides psychometric evidence of the reliability, internal validity and two-dimensional structure of the FIQR in a FM population. Our results support the use of two separate subscales for "function" and "symptoms", and provide a useful starting point for further refinement of the scale. © Clinical and Experimental Rheumatology 2013. Source

Catanoso M.,Unita Operativa di Reumatologia
Internal and emergency medicine

Biological agents have originally been developed to treat refractory arthritis, but evidence has been accruing, supporting their use in vasculitis as well. In the large-vessel vasculitides giant cell arteritis and Takayasu arteritis, TNF-α inhibitors have shown some efficacy in patients with relapsing disease. In contrast, in patients with recent onset of giant cell arteritis, TNF-α inhibitors failed to provide a significant benefit over and above that conferred by glucocorticoids alone. More recent, preliminary data suggest a role for the interleukin-6 receptor antagonist tocilizumab in both resistant and treatment-naïve giant cell arteritis and Takayasu arteritis. Biological agents have also been proposed to treat difficult anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis. Uncontrolled observations suggest that the TNF-α inhibitor infliximab might be beneficial in resistant cases. On the contrary, a randomized controlled trial did not show superiority of the recombinant human soluble TNF-α p75 receptor fusion protein etanercept over placebo in maintaining remission in granulomatosis with polyangiitis. Two randomized controlled trials have demonstrated that the anti-CD20 monoclonal antibody rituximab was as effective as the standard-of-care agent cyclophosphamide in inducing remission. In addition, rituximab appeared to be superior to cyclophosphamide in inducing remission in the subset of patients with relapsing disease. These findings prove that biological therapy has a role in vasculitis. Research is investigating novel therapies as well as focusing on how to best use the available drugs. Source

Boiardi L.,Unita Operativa di Reumatologia
Rheumatology (Oxford, England)

Chronic periaortitis (CP) is a rare disease characterized by a fibro-inflammatory tissue surrounding the abdominal aorta, and includes non-aneurysmal [idiopathic retroperitoneal fibrosis (IRF)] and aneurysmal forms [inflammatory abdominal aortic aneurysm (IAAA)]. We investigated whether CC chemokine receptor 5 (CCR5)Δ32 polymorphism confers susceptibility to CP. One hundred CP patients and 180 healthy controls were genotyped for CCR5Δ32 polymorphism by molecular methods. The patients were subgrouped according to the type of CP (IRF or IAAA) and the presence of established atherosclerotic disease (ischaemic heart disease, cerebrovascular disease and peripheral arterial disease). The distribution of the CCR5Δ32 genotype differed between CP patients and controls (P = 0.01). The CCR5Δ32 allele was more frequent in CP patients than in controls [P = 0.02, odds ratio (OR) 2.8 (95% CI 1.2, 6.4)]. The distribution of the CCR5Δ32 genotype did not differ significantly between IRF patients and controls, whereas the CCR5Δ32 allele was more frequent in IAAA patients than in controls [P = 0.0001, OR 10.0 (95% CI 3.7, 27.3)]. Furthermore, the CCR5Δ32 allele occurred more frequently in IAAA than in IRF patients [P = 0.001, OR 6.4 (95% CI 2.1, 19.1)]. The CCR5Δ32 allele frequency was higher in IAAA patients without established atherosclerotic disease compared with controls [66.7 vs 5.6%, P = 0.00001, OR 34.0 (95% CI 7.4, 156.3)], but not in IAAA patients with atherosclerotic disease and IRF patients with or without atherosclerotic disease. The CCR5Δ32 polymorphism might be associated with an increased risk of developing the aneurysmal form of CP, IAAA, particularly in patients without established atherosclerotic disease. Chemokines may have a role in the pathophysiology of CP. Source

Salvarani C.,Unita Operativa di Reumatologia | Hunder G.G.,Mayo Medical School | Morris J.M.,Mayo Medical School | Brown Jr. R.D.,Mayo Medical School | And 2 more authors.

Objective: To analyze the clinical findings, response to therapy, and outcomes of patients with cerebral vascular amyloid-b (Ab) deposition with and without inflammatory vascular infiltration. Methods: We report 78 consecutive patients with cerebral vascular Ab deposition examined at Mayo Clinic Rochester over 25 years (1987 through 2011). Specimens reviewed by a neuropathologist showed 40 with vascular Ab peptide without inflammation (cerebral amyloid angiopathy [CAA]), 28 with granulomatous vasculitis (Ab-related angiitis or ABRA), and 10 with perivascular CAA-related inflammation. We also matched findings in 118 consecutive patients with primary CNS vasculitis (PCNSV) without Ab seen over 25 years (1983 through 2007). Results: Compared to the 40 with CAA, the 28 with ABRA were younger at diagnosis (p = 0.05), had less altered cognition (p = 0.02), fewer neurologic deficits (p = 0.02), and fewer intracranial hemorrhages (<0.001), but increased gadolinium leptomeningeal enhancement (p = 0.01) at presentation, and less mortality and disability at last follow-up (p < 0.001). Compared with PCNSV, the 28 patients with ABRAwere older at diagnosis (p < 0.001), had a higher frequency of altered cognition (p = 0.05), seizures/spells (p = 0.006), gadolinium leptomeningeal enhancement (p < 0.001), and intracerebral hemorrhage (p = 0.02), lower frequency of hemiparesis (p = 0.01), visual symptoms (p = 0.04), and MRI evidence of cerebral infarction (p = 0.003), but higher CSF protein levels (p = 0.03). Results of treatment and outcomes in ABRA and PCNSV were similar. Conclusions: ABRA appears to represent a distinct subset of PCNSV. © 2013 American Academy of Neurology. Source

Salvarani C.,Unita Operativa di Reumatologia | Brown R.D.,Mayo Medical School | Morris J.M.,Mayo Medical School | Huston J.,Mayo Medical School | Hunder G.G.,Mayo Medical School
Clinical and Experimental Rheumatology

Primary central nervous system vasculitis (PCNSV) is an uncommon condition that affects the brain and the spinal cord. It is heterogeneous in presenting characteristics and outcomes. We report a patient with a catastrophic rapidly progressive course refractory to intensive treatment with pulses of methylprednisolone and iv cyclophosphamide. The condition rapidly deteriorated and the patient died 6 weeks after presentation. Rapidly progressive PCNSV represents the worst end of the clinical spectrum of PCNSV. These patients are characterised by bilateral, multiple, large cerebral vessel lesions on angiograms and multiple bilateral cerebral infarctions. © Clinical and experimental rheumatology 2014. Source

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