title: Patients' Perspectives and Experiences Living with Systemic Sclerosis: A Systematic Review and Thematic Synthesis of Qualitative Studies.
abstract: Systemic sclerosis (SSc) is a chronic, progressive autoimmune disease with major end-organ involvement. Much attention has been focused on the management of physical and clinical manifestations; however, the effect of the disease and treatment on the patient's identity, relationships, functioning, and mental well-being are less known. We aimed to describe the patients' perspectives and experiences of living with SSc.
  
  
  
  Electronic databases were searched to October 2014. Thematic synthesis was used to analyze the findings.
  
  
  
  We included 26 studies involving 463 patients. Six key themes were identified: distressing appearance transformation (disturbing facial changes, stigmatizing sickness, unrecognizable self), palpable physical limitations (bodily restrictions, frustrating mind-body disconnect, pervasive fatigue, disabling pain), social impairment (breaking intimacy, struggling to fulfill family responsibilities, maintaining work, losing independence), navigating uncertainty (diagnostic ambiguity, medically fending for oneself, unpredictable course of illness), alone and misunderstood (fearful avoidance of fellow patients, invisible suffering), and gradual acceptance and relative optimism (adapting to change and accepting limitations, taking a positive spin, cautious hoping, empowering relationships, valuing medical support).
  
  
  
  SSc is a rare and unpredictable illness that undermines patients' sense of certainty and control and impairs their self-image, identity, and daily functioning. Patient-centered care that encompasses strategies to promote self-esteem, resilience, and self-efficacy may help to improve treatment satisfaction and health and quality of life outcomes for patients with SSc.
authors.affiliation: From the Centre for Kidney Research, and Department of Rheumatology, The Children's Hospital at Westmead; Sydney School of Public Health, and Discipline of Paediatrics and Child Health, University of Sydney; School of Maternal and Child Health, University of New South Wales; Department of Rheumatology, Liverpool Hospital, Sydney; Department of Rheumatology, Canberra Hospital, Canberra; School of Medicine, University of Western Sydney, Campbelltown; South West Sydney Clinical School, University of New South Wales, Liverpool, Australia.A. Nakayama, MBBS, Centre for Kidney Research, The Children's Hospital at Westmead, and Department of Rheumatology, Canberra Hospital; D.J. Tunnicliffe, MIPH, Centre for Kidney Research, The Children's Hospital at Westmead, and Sydney School of Public Health, University of Sydney; V. Thakkar, MD, Department of Rheumatology, Liverpool Hospital, and School of Medicine, University of Western Sydney, and South West Sydney Clinical School, University of New South Wales; D. Singh-Grewal, PhD, Associate Professor, Department of Rheumatology, The Children's Hospital at Westmead, and Discipline of Paediatrics and Child Health, University of Sydney, and School of Maternal and Child Health, University of New South Wales, and Department of Rheumatology, Liverpool Hospital; S. O'Neill, PhD, Professor, Department of Rheumatology, Liverpool Hospital, and South West Sydney Clinical School, University of New South Wales; J.C. Craig, PhD, Associate Professor, Centre for Kidney Research, The Children's Hospital at Westmead, and Sydney School of Public Health, University of Sydney; A. Tong, PhD, Centre for Kidney Research, The Children's Hospital at Westmead, and Sydney School of Public Health, University of Sydney. Ayano.Nakayama@act.gov.au.
authors.forename: Ayano
authors.initials: A
authors.lastname: Nakayama
authors.affiliation: From the Centre for Kidney Research, and Department of Rheumatology, The Children's Hospital at Westmead; Sydney School of Public Health, and Discipline of Paediatrics and Child Health, University of Sydney; School of Maternal and Child Health, University of New South Wales; Department of Rheumatology, Liverpool Hospital, Sydney; Department of Rheumatology, Canberra Hospital, Canberra; School of Medicine, University of Western Sydney, Campbelltown; South West Sydney Clinical School, University of New South Wales, Liverpool, Australia.A. Nakayama, MBBS, Centre for Kidney Research, The Children's Hospital at Westmead, and Department of Rheumatology, Canberra Hospital; D.J. Tunnicliffe, MIPH, Centre for Kidney Research, The Children's Hospital at Westmead, and Sydney School of Public Health, University of Sydney; V. Thakkar, MD, Department of Rheumatology, Liverpool Hospital, and School of Medicine, University of Western Sydney, and South West Sydney Clinical School, University of New South Wales; D. Singh-Grewal, PhD, Associate Professor, Department of Rheumatology, The Children's Hospital at Westmead, and Discipline of Paediatrics and Child Health, University of Sydney, and School of Maternal and Child Health, University of New South Wales, and Department of Rheumatology, Liverpool Hospital; S. O'Neill, PhD, Professor, Department of Rheumatology, Liverpool Hospital, and South West Sydney Clinical School, University of New South Wales; J.C. Craig, PhD, Associate Professor, Centre for Kidney Research, The Children's Hospital at Westmead, and Sydney School of Public Health, University of Sydney; A. Tong, PhD, Centre for Kidney Research, The Children's Hospital at Westmead, and Sydney School of Public Health, University of Sydney.
authors.forename: David J
authors.initials: DJ
authors.lastname: Tunnicliffe
authors.affiliation: From the Centre for Kidney Research, and Department of Rheumatology, The Children's Hospital at Westmead; Sydney School of Public Health, and Discipline of Paediatrics and Child Health, University of Sydney; School of Maternal and Child Health, University of New South Wales; Department of Rheumatology, Liverpool Hospital, Sydney; Department of Rheumatology, Canberra Hospital, Canberra; School of Medicine, University of Western Sydney, Campbelltown; South West Sydney Clinical School, University of New South Wales, Liverpool, Australia.A. Nakayama, MBBS, Centre for Kidney Research, The Children's Hospital at Westmead, and Department of Rheumatology, Canberra Hospital; D.J. Tunnicliffe, MIPH, Centre for Kidney Research, The Children's Hospital at Westmead, and Sydney School of Public Health, University of Sydney; V. Thakkar, MD, Department of Rheumatology, Liverpool Hospital, and School of Medicine, University of Western Sydney, and South West Sydney Clinical School, University of New South Wales; D. Singh-Grewal, PhD, Associate Professor, Department of Rheumatology, The Children's Hospital at Westmead, and Discipline of Paediatrics and Child Health, University of Sydney, and School of Maternal and Child Health, University of New South Wales, and Department of Rheumatology, Liverpool Hospital; S. O'Neill, PhD, Professor, Department of Rheumatology, Liverpool Hospital, and South West Sydney Clinical School, University of New South Wales; J.C. Craig, PhD, Associate Professor, Centre for Kidney Research, The Children's Hospital at Westmead, and Sydney School of Public Health, University of Sydney; A. Tong, PhD, Centre for Kidney Research, The Children's Hospital at Westmead, and Sydney School of Public Health, University of Sydney.
authors.forename: Vivek
authors.initials: V
authors.lastname: Thakkar
authors.affiliation: From the Centre for Kidney Research, and Department of Rheumatology, The Children's Hospital at Westmead; Sydney School of Public Health, and Discipline of Paediatrics and Child Health, University of Sydney; School of Maternal and Child Health, University of New South Wales; Department of Rheumatology, Liverpool Hospital, Sydney; Department of Rheumatology, Canberra Hospital, Canberra; School of Medicine, University of Western Sydney, Campbelltown; South West Sydney Clinical School, University of New South Wales, Liverpool, Australia.A. Nakayama, MBBS, Centre for Kidney Research, The Children's Hospital at Westmead, and Department of Rheumatology, Canberra Hospital; D.J. Tunnicliffe, MIPH, Centre for Kidney Research, The Children's Hospital at Westmead, and Sydney School of Public Health, University of Sydney; V. Thakkar, MD, Department of Rheumatology, Liverpool Hospital, and School of Medicine, University of Western Sydney, and South West Sydney Clinical School, University of New South Wales; D. Singh-Grewal, PhD, Associate Professor, Department of Rheumatology, The Children's Hospital at Westmead, and Discipline of Paediatrics and Child Health, University of Sydney, and School of Maternal and Child Health, University of New South Wales, and Department of Rheumatology, Liverpool Hospital; S. O'Neill, PhD, Professor, Department of Rheumatology, Liverpool Hospital, and South West Sydney Clinical School, University of New South Wales; J.C. Craig, PhD, Associate Professor, Centre for Kidney Research, The Children's Hospital at Westmead, and Sydney School of Public Health, University of Sydney; A. Tong, PhD, Centre for Kidney Research, The Children's Hospital at Westmead, and Sydney School of Public Health, University of Sydney.
authors.forename: Davinder
authors.initials: D
authors.lastname: Singh-Grewal
authors.affiliation: From the Centre for Kidney Research, and Department of Rheumatology, The Children's Hospital at Westmead; Sydney School of Public Health, and Discipline of Paediatrics and Child Health, University of Sydney; School of Maternal and Child Health, University of New South Wales; Department of Rheumatology, Liverpool Hospital, Sydney; Department of Rheumatology, Canberra Hospital, Canberra; School of Medicine, University of Western Sydney, Campbelltown; South West Sydney Clinical School, University of New South Wales, Liverpool, Australia.A. Nakayama, MBBS, Centre for Kidney Research, The Children's Hospital at Westmead, and Department of Rheumatology, Canberra Hospital; D.J. Tunnicliffe, MIPH, Centre for Kidney Research, The Children's Hospital at Westmead, and Sydney School of Public Health, University of Sydney; V. Thakkar, MD, Department of Rheumatology, Liverpool Hospital, and School of Medicine, University of Western Sydney, and South West Sydney Clinical School, University of New South Wales; D. Singh-Grewal, PhD, Associate Professor, Department of Rheumatology, The Children's Hospital at Westmead, and Discipline of Paediatrics and Child Health, University of Sydney, and School of Maternal and Child Health, University of New South Wales, and Department of Rheumatology, Liverpool Hospital; S. O'Neill, PhD, Professor, Department of Rheumatology, Liverpool Hospital, and South West Sydney Clinical School, University of New South Wales; J.C. Craig, PhD, Associate Professor, Centre for Kidney Research, The Children's Hospital at Westmead, and Sydney School of Public Health, University of Sydney; A. Tong, PhD, Centre for Kidney Research, The Children's Hospital at Westmead, and Sydney School of Public Health, University of Sydney.
authors.forename: Sean
authors.initials: S
authors.lastname: O'Neill
authors.affiliation: From the Centre for Kidney Research, and Department of Rheumatology, The Children's Hospital at Westmead; Sydney School of Public Health, and Discipline of Paediatrics and Child Health, University of Sydney; School of Maternal and Child Health, University of New South Wales; Department of Rheumatology, Liverpool Hospital, Sydney; Department of Rheumatology, Canberra Hospital, Canberra; School of Medicine, University of Western Sydney, Campbelltown; South West Sydney Clinical School, University of New South Wales, Liverpool, Australia.A. Nakayama, MBBS, Centre for Kidney Research, The Children's Hospital at Westmead, and Department of Rheumatology, Canberra Hospital; D.J. Tunnicliffe, MIPH, Centre for Kidney Research, The Children's Hospital at Westmead, and Sydney School of Public Health, University of Sydney; V. Thakkar, MD, Department of Rheumatology, Liverpool Hospital, and School of Medicine, University of Western Sydney, and South West Sydney Clinical School, University of New South Wales; D. Singh-Grewal, PhD, Associate Professor, Department of Rheumatology, The Children's Hospital at Westmead, and Discipline of Paediatrics and Child Health, University of Sydney, and School of Maternal and Child Health, University of New South Wales, and Department of Rheumatology, Liverpool Hospital; S. O'Neill, PhD, Professor, Department of Rheumatology, Liverpool Hospital, and South West Sydney Clinical School, University of New South Wales; J.C. Craig, PhD, Associate Professor, Centre for Kidney Research, The Children's Hospital at Westmead, and Sydney School of Public Health, University of Sydney; A. Tong, PhD, Centre for Kidney Research, The Children's Hospital at Westmead, and Sydney School of Public Health, University of Sydney.
authors.forename: Jonathan C
authors.initials: JC
authors.lastname: Craig
authors.affiliation: From the Centre for Kidney Research, and Department of Rheumatology, The Children's Hospital at Westmead; Sydney School of Public Health, and Discipline of Paediatrics and Child Health, University of Sydney; School of Maternal and Child Health, University of New South Wales; Department of Rheumatology, Liverpool Hospital, Sydney; Department of Rheumatology, Canberra Hospital, Canberra; School of Medicine, University of Western Sydney, Campbelltown; South West Sydney Clinical School, University of New South Wales, Liverpool, Australia.A. Nakayama, MBBS, Centre for Kidney Research, The Children's Hospital at Westmead, and Department of Rheumatology, Canberra Hospital; D.J. Tunnicliffe, MIPH, Centre for Kidney Research, The Children's Hospital at Westmead, and Sydney School of Public Health, University of Sydney; V. Thakkar, MD, Department of Rheumatology, Liverpool Hospital, and School of Medicine, University of Western Sydney, and South West Sydney Clinical School, University of New South Wales; D. Singh-Grewal, PhD, Associate Professor, Department of Rheumatology, The Children's Hospital at Westmead, and Discipline of Paediatrics and Child Health, University of Sydney, and School of Maternal and Child Health, University of New South Wales, and Department of Rheumatology, Liverpool Hospital; S. O'Neill, PhD, Professor, Department of Rheumatology, Liverpool Hospital, and South West Sydney Clinical School, University of New South Wales; J.C. Craig, PhD, Associate Professor, Centre for Kidney Research, The Children's Hospital at Westmead, and Sydney School of Public Health, University of Sydney; A. Tong, PhD, Centre for Kidney Research, The Children's Hospital at Westmead, and Sydney School of Public Health, University of Sydney.
authors.forename: Allison
authors.initials: A
authors.lastname: Tong
mesh_terms: D000223:Adaptation, Psychological; D001294:Attitude to Health; D017281:Cost of Illness; D006801:Humans; D007398:Interpersonal Relations; D036301:Qualitative Research; D011788:Quality of Life / Q000523:psychology*; D012595:Scleroderma, Systemic / Q000523:psychology*; D012649:Self Concept; D020377:Self Efficacy; D057545:Social Stigma
keywords: HEALTH BEHAVIOR; QUALITATIVE RESEARCH; QUALITY OF LIFE/PSYCHOLOGY; REVIEW; SOCIAL SUPPORT; SYSTEMIC SCLEROSIS