title: Systemic sclerosis: The need for structured care.
abstract: Autoimmune connective tissue diseases (CTDs) have a propensity to affect multiple organ systems as well as physical function, quality of life, and survival. Their clinical heterogeneity, multisystem involvement, and low worldwide prevalence present challenges for researchers to establish a study design to help better understand the course and outcomes of CTDs. Systemic sclerosis (SSc) is a notable example of a CTD, wherein longitudinal cohort studies (LCS) have enabled us to elucidate disease manifestations, disease course, and risk and prognostic factors for clinically important outcomes, by embedding research in clinical practice. Nevertheless, further efforts are needed to better understand SSc especially with regard to recognizing organ involvement early, developing new therapies, optimizing the use of existing therapies, and defining treatment targets. The heterogeneous multi-organ nature of SSc would lend itself well to a structured model of care, wherein step-up treatment algorithms are used with the goal of attaining a prespecified treatment target. In this chapter, we discuss the rationale for a structured treatment approach in SSc and propose possible treatment algorithms for three of the more common disease manifestations, namely skin involvement, digital ulcers and gastrointestinal tract involvement. We discuss possible strategies for evaluating and implementing these algorithms in the setting of LCS. We conclude by presenting a research agenda for the development of structured models of care in SSc.
authors.affiliation: Department of Medicine, The University of Melbourne at St. Vincent's Hospital, Melbourne, VIC, Australia; Department of Rheumatology, The University of Melbourne at St. Vincent's Hospital, Melbourne, VIC, Australia.
authors.forename: Kathleen
authors.initials: K
authors.lastname: Morrisroe
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, University of Utah, Salt Lake City, UT, USA; Salt Lake Regional Veterans Affair Medical Center, Salt Lake City, UT, USA.
authors.forename: Tracy
authors.initials: T
authors.lastname: Frech
authors.affiliation: Division of Rheumatology, University Hospital Zurich, Zurich, Switzerland.
authors.forename: Janine
authors.initials: J
authors.lastname: Schniering
authors.affiliation: Division of Rheumatology, University Hospital Zurich, Zurich, Switzerland.
authors.forename: Britta
authors.initials: B
authors.lastname: Maurer
authors.affiliation: Department of Medicine, The University of Melbourne at St. Vincent's Hospital, Melbourne, VIC, Australia; Department of Rheumatology, The University of Melbourne at St. Vincent's Hospital, Melbourne, VIC, Australia. Electronic address: m.nikpour@unimelb.edu.au.
authors.forename: Mandana
authors.initials: M
authors.lastname: Nikpour
mesh_terms: D003240:Connective Tissue Diseases / Q000628:therapy*; D018450:Disease Progression; D006801:Humans; D015995:Prevalence; D011788:Quality of Life; D012595:Scleroderma, Systemic / Q000628:therapy*; D012883:Skin Ulcer / Q000628:therapy
keywords: Digital ulcers; Model of care; Scleroderma; Systemic sclerosis; Treatment algorithm