6 research outputs found

    The Role of Endogenous Eicosapentaenoic Acid and Docosahexaenoic Acid-Derived Resolvins in Systemic Sclerosis

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    Resolvins, the member of specialized pro-resolving mediators, are produced from omega-3 polyunsaturated fatty acids as a response to an acute inflammatory process in that termination and resolution of inflammation. In the acute inflammation, these lipid mediators limit polymorphonuclear cells infiltration, proinflammatory cytokine production; promote efferocytosis, and regulate several cell types being important roles in innate and adaptive immunity. Any dysregulation or defect of the resolution phase result in prolonged, persistent inflammation and eventually fibrosis. Resolvins are implicated in the development of various chronic autoimmune diseases. Systemic sclerosis (SSc) is a very complicated, chronic autoimmune disorder proceeding with vasculopathy, inflammation, and fibrosis. Dysregulation of innate and adaptive immunity is another important contributing factor in the pathogenesis of SSc. In this review, we will focus on the different roles of this new family of lipid mediators, characterized by the ability to prevent the spread of inflammation and its chronicity in various ways and how they can control the development of fibrotic diseases like SSc

    Calcinosis in systemic sclerosis: An update on pathogenesis, related complications, and management: A heavy burden still waiting to be lifted off patients’ hands

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    In SSc, dystrophic calcinosis is one of the major clinical manifestations, characterized by the deposition of insoluble calcific substances in tissues, predominantly in the chemical form of calcium hydroxyapatite. Furthermore, calcinosis might lead to compressive neuropathies and severe pain. Current evidence suggests that tissue ischemia and repeated trauma are implicated in the development of calcinosis; however, there are still too many unknown areas that need to be investigated. Detection of calcinosis is commonly performed using X-ray or ultrasound. Moreover, quantification of calcinosis with X-ray and dual-energy computed tomography might be useful for the assessment of disease burden and monitoring of the disease. Despite its prevalence and clinical outcomes, there are no approved disease-modifying treatments for calcinosis in SSc. Debulking or surgical intervention might be preferred for calcinosis complicated with infection, compressive symptoms, or relief of pain. Therefore, innovative investigations and tailored therapeutic approaches are urgently needed to lift the burden of calcinosis from the hands of SSc patients

    Development and implementation of the AIDA international registry for patients with Schnitzler's syndrome

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    The present paper describes the design, development, and implementation of the AutoInflammatory Disease Alliance (AIDA) International Registry specifically dedicated to patients with Schnitzler's syndrome
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