title: Systemic sclerosis: Recent insights.
abstract: Systemic sclerosis is an orphan connective tissue disease characterized by alterations of the microvasculature, disturbances of the immune system and massive deposition of collagen and other matrix substances in the skin and internal organs. A major achievement of the recent years has been the validation of new classification criteria, allowing earlier diagnosis and earlier treatment of systemic sclerosis, before irreversible fibrosis and organ damage appeared ("window of opportunity"). Raynaud's phenomenon is usually the first sign of the disease and is considered as the main sentinel sign for the identification of very early systemic sclerosis. Systemic sclerosis is clinically heterogeneous and disease course remains unpredictable. Its prognosis depends on cardiopulmonary involvement and recent studies aim to identify serum or genetic biomarkers predictive of severe organ involvement. Moreover, the prospective follow-up of large cohorts has provided and will offer critical material to identify strong prognostic factors. Whereas the outcomes of vascular manifestations of the disease has been recently improved due to targeted therapy, recent data have highlighted that mortality has not changed over the past 40 years. This reflects the absence of efficacy of current available drugs to counteract the fibrotic process. Nevertheless, several targeted immunity therapies, commonly with proven efficacy in other immune diseases, are about to be investigated in systemic sclerosis. Indeed, promising results in small and open studies have been reported. This article deals with recent insights into classification criteria, pathogenesis, organ involvements, outcome and current and possible future therapeutic options in systemic sclerosis.
authors.affiliation: Paris Descartes University, Sorbonne Paris Cité, Rheumatology A department, Cochin Hospital, 27, rue du Faubourg-Saint-Jacques 75014 Paris, France; Inserm U1016, Cochin Hospital, 27, rue du Faubourg-Saint-Jacques 75014 Paris, France.
authors.forename: Muriel
authors.initials: M
authors.lastname: Elhai
authors.affiliation: Paris Descartes University, Sorbonne Paris Cité, Rheumatology A department, Cochin Hospital, 27, rue du Faubourg-Saint-Jacques 75014 Paris, France; Inserm U1016, Cochin Hospital, 27, rue du Faubourg-Saint-Jacques 75014 Paris, France.
authors.forename: Jérôme
authors.initials: J
authors.lastname: Avouac
authors.affiliation: Paris Descartes University, Sorbonne Paris Cité, Rheumatology A department, Cochin Hospital, 27, rue du Faubourg-Saint-Jacques 75014 Paris, France.
authors.forename: André
authors.initials: A
authors.lastname: Kahan
authors.affiliation: Paris Descartes University, Sorbonne Paris Cité, Rheumatology A department, Cochin Hospital, 27, rue du Faubourg-Saint-Jacques 75014 Paris, France; Inserm U1016, Cochin Hospital, 27, rue du Faubourg-Saint-Jacques 75014 Paris, France. Electronic address: yannick.allanore@cch.aphp.fr.
authors.forename: Yannick
authors.initials: Y
authors.lastname: Allanore
mesh_terms: D018501:Antirheumatic Agents / Q000627:therapeutic use; D042241:Early Diagnosis; D005355:Fibrosis; D006801:Humans; D011379:Prognosis; D011928:Raynaud Disease / Q000209:etiology; D012595:Scleroderma, Systemic / Q000145:classification / Q000150:complications / Q000503:physiopathology / Q000628:therapy
chemical_list: D018501:Antirheumatic Agents
keywords: Fibrosis; Pathogenesis; Raynaud's phenomenon; Systemic sclerosis; Treatment