1,721,138 research outputs found
Very early and early diagnosis of systemic sclerosis
No full textSystemic sclerosis (SSc) is easily diagnosed when the disease is evolved to skin fibrosis with obliterative vasculopathy and internal organs involvement. In the very early/early phase of SSc, the diagnosis remains very difficult because of the lack of validated diagnostic criteria. Actually, the American College of Rheumatology (ACR) and LeRoy classification criteria are not sufficiently sensitive to enable very early/early diagnosis of the disease, limiting the possibility of a precocious treatment. Therefore, in many cases, treatment is delayed for several years following the onset of Raynaud‘s phenomenon (RP) and even after the onset of the first non-RP symptom. For this reason, the very early/early diagnosis of SSc is today of pivotal importance. Recently, RP, puffy fingers turning into sclerodactyly and antinuclear antibody (ANA) positivity are considered the three red flags for the suspicion of a very early SSc. Further signs such as positivity of other specific autoantibodies and/or videocapillaroscopy pattern may allow very early diagnosis of SSc. This may allow follow-up of the patient and start the appropriate therapeutic regimen to arrest the disease progression when an organ involvement is detected. The time gap between the onset of signs and the diagnosis can be considered the ‘window of opportunity‘ where the disease may be stopped before skin and internal organs are irreversibly damaged. Therefore, the main topic discussed in this chapter will be ‘why we need to distinguish between a very early and an early diagnosis of SSc‘
Avascular bone necrosis: An underestimated complication of systemic sclerosis
No full textAvascular bone necrosis: An underestimated complication of systemic sclerosi
One year in review 2017: idiopathic inflammatory myopathies
No full textEvery year new concepts about pathogenesis, serology, diagnosis and treatment in inflammatory myopathies (IIMs) have been provided. The purpose of this manuscript is to summarise the most relevant literature contributions published over the last year about these complex and rare diseases
Prediction and primary prevention of major vascular complications in systemic sclerosis
No full textObjective: In Systemic Sclerosis (SSc), vasculopathy is the background of major vascular complications (MVCs), like digital ulcers (DUs), pulmonary arterial hypertension (PAH) and scleroderma renal crisis (SRC). We aimed to identify the predictors and to test the primary preventive effect of vasoactive/vasodilating drugs (VVD) for the development of MVCs in SSc MVCs-na & iuml;ve patients.Methods: patients fulfilling the ACR/EULAR 2013 classification criteria for SSc without history of MVCs were eligible. Data about clinical manifestations, laboratory and instrumental assessments and treatments were retrospectively collected at baseline and latest available follow-up.Results: 134 SSc patients were enrolled (mean age 56.5 years +/- 14.2, females 88.1%, limited subset 61.9%, ACA positivity 60.4%). In a mean of 43 +/- 19 months of follow-up 12 (9.0%) patients developed at least 1 MVC (10 DU, 2 PAH and 1 SRC). Dyspnoea and arthritis at baseline were independent predictors for MVCs development (p = 0.012, and p = 0.002 respectively). No primary preventive effect of VVD on MVCs development was found. However, sildenafil reduced the renal resistive index increase (p = 0.042) and alprostadil slowed the DLco decline (p = 0.029). Both iloprost and angiotensin-receptor blockers (ARBs) delayed MVCs development, while angiotensin converting enzyme inhibitors (ACEi) determined an earlier onset of such MCVs.Conclusions: in SSc patients, our data confirm the role of arthritis and dyspnea as independent predictors of major vascular complications, in particular in MVCs-na & iuml;ve patients. Prostanoids, sildenafil and ARBs, even in absence of a primary preventive action, might help in slowing disease progression and postponing the onset of MVCs
Use of biologics and other novel therapies for the treatment of systemic sclerosis
No full textINTRODUCTION Systemic sclerosis (SSc) is a systemic autoimmune disease characterized by vasculopathy, inflammation and fibrosis. These three main disease-determining pathways are the target of the currently available treatments used to possibly modify the progression of disease-related manifestations, although this synergy has not been fully applied on SSc joint, skin or lung involvement yet. Areas covered: we describe the current status of SSc treatment/therapy performing a literature search in MEDLINE/Pubmed and Thomson Reuter's Web of Science for articles published until March 2016. Moreover, ongoing registered clinical trials (RCTs) on SSc were searched through clinicaltrials.gov website. Expert commentary: presently, promising drugs are under evaluation to target the different pathogenic pathways of systemic sclerosis: Tocilizumab and Abatacept for skin and lung fibrosis; Riociguat and Selexipag are approved for pulmonary arterial hypertension but promising anti-fibrotic effects are now being studied. Finally, several anti-fibrotic molecules are currently involved in RCTs, such as Nintedanib, IVA-337, Terguride
POLYESTER BLENDS NANOCOMPOSITES AND COMPOSITES WITH INCREASED THERMAL STABILITY OR FLAME ESTINGUISHING PROPERTIES
No full textThe dispersion of phyllosilicates into polymer or blends at nanometer scale [1] allows improving the properties of the polymer matrix [2] including thermal stability and flame retardancy .Indeed the dispersion of nanoclays favors char formation on the burning surface [3].
Poly(ethylene terephthalate) (PET), which is one of the most diffused polymers, is used in a wide variety of applications. PET-based nanocomposites which contain phyllosilicates are considered superior for providing improved gas barrier properties, due mainly to the strong effect of confinement as the result of a high surface: volume ratio (i.e., reducing chain mobility and permeability [4]), as well as to the enhancement of tortuosity [5] of the path required for small molecules to permeate through the polymer film due to the presence of silicate lamellae. Flame retardancy and gas barrier functions are much-required properties in the area of textiles and civil engineering, the main aim being to develop fabrics or security panels and interior decorations with flame self-extinguishing properties [6].
PET based micro-composites can also show flame estinguishing properties when filled with phosphorous derivatives which are quite effective for imparting flame estinguishig properties to PET blends [7].
In the present contribution some insights on the preparation of PET nanocomposites with modified nanosilicates (dellite) and ipophosphite based microcomposites are reported
Hydroxychloroquine and joint involvement in systemic sclerosis: Preliminary beneficial results from a retrospective case-control series of an EUSTAR center
No full textHydroxychloroquine and joint involvement in systemic sclerosis: Preliminary beneficial results from a retrospective case-control series of an EUSTAR cente
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