Systemic sclerosis is a rare and incurable autoimmune disease in which patients are challenged with self-managing their disease. Systematic evaluation of the essential self-management intervention components and self-management outcomes is necessary to assess the state of the science of self-management for patients with systemic sclerosis. As such, the purpose of this systematic review was to identify and describe self-management interventions and their impact on self-management outcomes in adults with systemic sclerosis. Studies were included if they contained a self-management intervention, incorporated at least one self-management outcome identified as a common data element by the National Institute of Nursing Research (i.e., patient activation, self-efficacy, self-regulation, global health), and were conducted in adults with systemic sclerosis. Using the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines, PubMed, Cumulative Index of Nursing and Allied Health Literature, and Embase were searched from the inception of each database to March 2020, yielding a total of 215 studies. Of these reports, 16 met the inclusion criteria for this review. There was notable variability in the types of self-management interventions and their effects on key self-management outcomes. Self-management interventions focused on improving physical function through rehabilitation programs were the most common interventions (n = 7). The poor methodological quality of the studies included in this systematic review, however, limited the synthesis of and recommendations for self-management interventions in adults with systemic sclerosis. Future research in systemic sclerosis should include key self-management outcomes in larger, more rigorously designed studies to allow for comparisons across studies and to advance the science of self-management.
Keywords: common data element; scleroderma; self-management; systematic review; systemic sclerosis.
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