Unmet Needs in Systemic Sclerosis Understanding and Treatment: the Knowledge Gaps from a Scientist’s, Clinician’s, and Patient’s Perspective

Systemic sclerosis (SSc) is a highly heterogeneous disease caused by a complex molecular circuitry. For decades, clinical and molecular research focused on understanding the primary process of fibrosis. More recently, the inflammatory, immunological and vascular components that precede the actual on...

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Bibliographic Details
Published in:	Clinical reviews in allergy & immunology 2018-12, Vol.55 (3), p.312-331
Main Authors:	Cossu, Marta, Beretta, Lorenzo, Mosterman, Petra, de Hair, Maria J. H., Radstake, Timothy R. D. J.
Format:	Article
Language:	eng
Subjects:	Allergology Care and treatment Complexity Design of experiments Development and progression Disease Management Disease Susceptibility Environmental factors Experimental design Fibrosis Health Knowledge, Attitudes, Practice Health Personnel Health Services Needs and Demand Humans Immune System - cytology Immune System - immunology Immune System - metabolism Immunology Inflammation Internal Medicine Medical research Medicine Medicine & Public Health Medicine, Experimental Patients Precision Medicine - methods Scientists Scleroderma Scleroderma (Disease) Scleroderma, Systemic - diagnosis Scleroderma, Systemic - epidemiology Scleroderma, Systemic - etiology Scleroderma, Systemic - therapy Systemic scleroderma Systemic sclerosis
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Summary:	Systemic sclerosis (SSc) is a highly heterogeneous disease caused by a complex molecular circuitry. For decades, clinical and molecular research focused on understanding the primary process of fibrosis. More recently, the inflammatory, immunological and vascular components that precede the actual onset of fibrosis, have become a matter of increasing scientific scrutiny. As a consequence, the field has started to realize that the early identification of this syndrome is crucial for optimal clinical care as well as for understanding its pathology. The cause of SSc cannot be appointed to a single molecular pathway but to a multitude of molecular aberrances in a spatial and temporal matter and on the backbone of the patient’s genetic predisposition. These alterations underlie the plethora of signs and symptoms which patients experience and clinicians look for, ultimately culminating in fibrotic features. To solve this complexity, a close interaction among the patient throughout its “journey,” the clinician through its clinical assessments and the researcher with its experimental design, seems to be required. In this review, we aimed to highlight the features of SSc through the eyes of these three professionals, all with their own expertise and opinions. With this unique setup, we underscore the importance of investigating the role of environmental factors in the onset and perpetuation of SSc, of focusing on the earliest signs and symptoms preceding fibrosis and on the application of holistic research approaches that include a multitude of potential molecular alterations in time in an unbiased fashion, in the search for a patient-tailored cure.
ISSN:	1080-0549 1559-0267