Systemic sclerosis: The need for structured care.

Morrisroe K; Frech T; Schniering J; Maurer B; Nikpour M

doi:10.1016/j.berh.2016.04.004

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Journal article

Systemic sclerosis: The need for structured care.

Morrisroe K 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.
Frech T 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.
Schniering J Division of Rheumatology, University Hospital Zurich, Zurich, Switzerland.
Maurer B Division of Rheumatology, University Hospital Zurich, Zurich, Switzerland.
Nikpour M 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.

2016-07-17

Published in:

Best practice & research. Clinical rheumatology. - 2016

English 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.

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