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Article Dans Une Revue Frontiers in Immunology Année : 2021

Editorial: Key Players in Systemic Sclerosis: The Immune System and Beyond


In these times of Covid-19 pandemic, during which medical research is mobilized in the fight against the coronavirus, patients continue to suffer from other acute and chronic diseases and research on them must continue. Systemic sclerosis (SSc) is one of these diseases, both chronic and complex, whose evolution can be fatal and whose clinical repercussions, variable from one patient to another, can result in a profound alteration of autonomy and quality of life. The current crisis of the coronavirus should not make us forget this reality. At this point, we must recall the well-known generalities about this disease (1). Systemic Sclerosis is a rare multifaceted disease, whose development is related to the interplay of three major pathophysiological mechanisms, i.e. vascular impairment, autoimmunity and uncontrolled fibrogenesis. These mechanisms lead to the development of the main disease-related manifestations including skin, lung, heart, and gastrointestinal tract involvement. Fibrosis primarily affects the skin dermis. However, the lungs, heart, and/or digestive tract may also be involved. Vascular impairment leads to the development of Raynaud's phenomenon, telangiectasia, digital ulcers, pulmonary arterial hypertension and/or vascular renal crisis. These features render SSc a very peculiar autoimmune disorder. Autoimmunity in SSc is mediated via the immune cell activation and production of autoantibodies (Abs). The latter are mainly directed against three target autoantigens, namely Topoisomerase 1 (anti-SCL70 Abs), CENP-A/B (anti-centromere Abs) or RNA-polymerase III. Sustained immune and inflammatory stimulation promote pathological fibroblast activation, which results in excessive extracellular matrix protein production and accumulation in tissues. The clinical presentation of the disease is heterogenous. SSc may present in three classical forms, including limited cutaneous, diffuse cutaneous, and 'sine scleroderma' subsets, and may associate with other connective tissue diseases. SSc has a poor prognosis, globally with a severe vital prognosis and potential long-term disability. Its treatment is mainly palliative and based on symptomatic therapies, vasodilators, immunosuppressants, targeted therapies and antifibrotic drugs. Therefore,
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hal-03647291 , version 1 (20-04-2022)





Philippe Guilpain, Danièle Noël, Jérôme Avouac. Editorial: Key Players in Systemic Sclerosis: The Immune System and Beyond. Frontiers in Immunology, 2021, 12, pp.770419. ⟨10.3389/fimmu.2021.770419⟩. ⟨hal-03647291⟩
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