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. Author manuscript; available in PMC: 2022 Apr 1.
Published in final edited form as: Res Nurs Health. 2021 Mar 2;44(2):376–392. doi: 10.1002/nur.22118

Self-management interventions in systemic sclerosis: A systematic review

Robyn K Wojeck 1, Donald E Bailey 1, Tamara J Somers 2, Mitchell R Knisely 1
PMCID: PMC7971253  NIHMSID: NIHMS1676643  PMID: 33651396

Abstract

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 (PRISMA) guidelines, PubMed, Cumulative Index of Nursing and Allied Health Literature (CINAHL), and Embase were searched from the inception of each database to March 2020, yielding a total of 215 studies. Of these reports, sixteen 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

Systemic sclerosis (scleroderma) is a rare, chronic, multisystem autoimmune disease characterized by vasculopathy, autoantibody production, and progressive fibrosis of the skin and internal organs leading to skin thickening and multiorgan dysfunction (van den Hoogen et al., 2013). Prevalence rates vary worldwide, from 7 to 489 cases per million (Chifflot et al., 2008), with women disproportionately affected and often experiencing a disease onset between the ages of 30 to 50 years old (Mayes, 2003). Disease manifestations of systemic sclerosis such as skin thickening, finger ulcers, Raynaud’s phenomenon, gastroesophageal reflux, interstitial lung disease, and pulmonary arterial hypertension lead to significant morbidity and mortality (Ingegnoli et al., 2018).

Although systemic sclerosis is a clinically heterogenous disease, patients are often divided into two main subsets-- limited cutaneous systemic sclerosis and diffuse cutaneous systemic sclerosis-- based on the severity of their skin involvement (van den Hoogen et al., 2013). Skin thickening is localized to the fingers, distal extremities, and face in limited cutaneous systemic sclerosis, whereas skin thickening extends to the proximal extremities and trunk in diffuse cutaneous systemic sclerosis (Allanore et al., 2015). Additionally, patients with diffuse cutaneous systemic sclerosis experience rapid disease progression leading to more severe skin thickening and earlier development of organ complications in the gastrointestinal tract, lungs, heart, and kidneys (Allanore et al., 2015).

Patients with systemic sclerosis are challenged with managing debilitating physical and psychological symptoms such as pain, fatigue, sleep disturbance, body disfigurement, depression, anxiety, and social isolation (Almeida et al., 2015; Thombs et al., 2010). These symptoms lead to functional disability, reduced health-related quality of life, productivity losses, and high health care costs (Fischer et al., 2017), accounting for up to $1.9 billion in total costs per year in North America (Bernatsky et al., 2009). With no cure and no disease-modifying therapies, the emphasis of care is placed on reducing symptoms, minimizing functional disability, and improving one’s health-related quality of life (Kwakkenbos et al., 2017).

Thus, systemic sclerosis is a chronic condition requiring patients to self-manage their disease. Self-management is defined as the daily management of a chronic condition by an individual (Grady & Gough, 2018; Lorig & Holman, 2003) and is focused on equipping patients with the skills and resources to manage their chronic condition (Lorig & Holman, 2003). More specifically, self-management interventions are focused on improving one’s ability to improve their health status at any point along their disease trajectory (Grady & Gough, 2018). In a systematic review by Willems et al. (2015), variability in the content, intervention components, and outcomes of nonpharmacologic interventions for patients with systemic sclerosis was found. Research on nonpharmacologic, rehabilitative, and exercise interventions in systemic sclerosis has also addressed the poor methodological quality of studies, limiting the authors’ ability to synthesize the data and make recommendations for the use of these interventions in patients with systemic sclerosis (Liem et al., 2019; Mugii et al., 2018; Willems et al., 2015).

Given the lack of expert consensus on the use of psychosocial, rehabilitative, and educational interventions for treatment of systemic sclerosis, the European League Against Rheumatism (EULAR) and the EULAR Scleroderma Trials and Research group (EUSTAR) have been unable to make recommendations of their use in treatment guidelines (Kowal-Bielecka et al., 2017). As such, further exploration of self-management interventions and their outcomes is needed to inform treatment guidelines and clinical practice in this population.

To establish common outcomes and to facilitate comparison of data across self-management studies in chronic conditions, the National Institute of Nursing Research (NINR) recommended four common data elements (CDEs) for self-management research, including: patient activation, self-efficacy, self-regulation, and global health (Moore et al., 2016). These CDEs represent the core concepts in existing self-management frameworks and have been used in a variety of self-management studies in persons with chronic conditions (Moore et al., 2016).

Additionally, the NINR provided a definition of each construct and the recommended measure for each self-management outcome (Moore et al., 2016). Patient activation was defined as the knowledge, skills, and confidence for self-management of one’s chronic condition, as measured using the Patient Activation Measure (PAM; Hibbard et al., 2005; Hibbard et al., 2004). Self-efficacy was defined as one’s confidence to manage various aspects of his or her chronic condition as measured by the Self-Efficacy for Managing Chronic Disease (SEMCD) scale (Lorig et al., 2001). Self-regulation was defined as one’s effort and will to make behavioral changes as measured by the Index of Self-Regulation (ISR; Fleury, 1998; Yeom et al., 2011). Lastly, global health was defined as one’s general evaluation of his or her overall health, including both physical and mental health, as measured using the Patient Reported Outcomes Measurement Information System Global Health Short Form (PROMIS SF v1.1 Global; Hays et al., 2009). Global health is an indicator of health status (Hays et al., 2009) and includes components of global physical health, such as overall physical health, physical function, pain, and fatigue, as well as components of global mental health, such as quality of life, mental health, satisfaction with social activities, and emotional problems such as anxiety and depression (Hays et al., 2009). The use of CDEs in self-management research allows for comparisons of study findings across populations, improvements in the generalizability of findings, and acceleration of research into practice (Cohen et al., 2015).

Systematic evaluation of the essential intervention components and self-management outcomes, identified as CDEs, in patients with systematic sclerosis is necessary to assess the state of the science of self-management interventions and to guide future self-management research and intervention development. As such, the purpose of this systematic review was to identify and describe self-management interventions in systemic sclerosis and their impact on specific self-management outcomes (i.e., patient activation, self-efficacy, self-regulation, and global health).

METHODS

This systematic review was completed using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines (Liberati et al., 2009; Moher et al., 2009). In collaboration with a university librarian, a comprehensive search was conducted using PubMed, Cumulative Index to Nursing and Allied Health Literature (CINAHL), and Embase for studies published from the inception of each database to March 2020. PubMed and Embase have been identified as essential databases to be included in systematic reviews of randomized controlled trials, supporting their inclusion in this systematic review (Higgins et al., 2019). CINAHL was included as a subject-specific database to capture this systematic review’s nursing lens (Higgins et al., 2019). The searches included a combination of the following index terms and free text words using Boolean operators: systemic sclerosis, scleroderma, self-management, self-manage, and self-care (Table 1). Self-care was included as a search term given its often interchangeable use with self-management (Grady & Gough, 2018).

Table 1.

Search Strategy

PubMed ((“Scleroderma, Systemic”[Mesh] OR “Scleroderma, Localized”[Mesh] OR “Scleroderma, Limited”[Mesh] OR “Scleroderma, Diffuse”[Mesh] OR “systemic sclerosis” OR scleroderma OR “CREST syndrome”)) AND (“Self-Management”[Mesh] OR “self management” OR “self-management” OR “self manage” OR “self-manage” OR “self care”[MeSH Terms] OR “self care”[All Fields])
CINAHL ((MH “Scleroderma, Systemic+”) OR “systemic sclerosis” OR scleroderma) AND ((MH “Self-Management”) OR (MH “Self Care+”) OR “self-management” OR “self management” OR “self care”) 
Embase (‘systemic sclerosis’/exp OR ‘systemic sclerosis’ OR ‘systemic scleroderma’/exp OR ‘systemic scleroderma’ OR ‘scleroderma’/exp OR scleroderma) AND (‘self management’/exp OR ‘self management’ OR ‘self care’/exp OR ‘self care’)
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Inclusion criteria for studies in this review were: (a) at least one self-management intervention; (b) patient activation, self-efficacy, self-regulation, or global health as an outcome; (c) comprised of adults ≥ 18 years with a diagnosis of systemic sclerosis as the study sample; (d) published in English; and (e) in peer-reviewed journals available in full text. Studies were excluded if they were: (a) conference abstracts, editorial letters, comments, unpublished manuscripts, case reports, or literature reviews, (b) not published in English, (c) not available for full-text review, (d) focused on other chronic conditions, (e) comprised of only children or adolescents, or (f) not inclusive of a self-management intervention.

For the purposes of this systematic review, a self-management intervention was defined as any program that focused on increasing one’s knowledge and/or skills to improve his or her own ability to manage systemic sclerosis on a daily basis. This definition was modified from Ryan and Sawin’s (2009) definition of self-management in their Individual and Family Self-Management Theory. The four self-management outcomes were selected because they have been identified as CDEs for self-management research by the NINR and represent the core concepts of self-management frameworks (Moore et al., 2016). The searches yielded 215 studies, with an additional seven studies identified from reference lists (Figure 1).

Figure 1.

Figure 1

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Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) Flow Diagram

Data Extraction

One author (R.W).reviewed the titles of the 222 studies in this sample to identify potentially relevant studies. After removing duplicates, the remaining 164 studies were screened by the same author. A total of 138 studies were excluded, resulting in 26 studies retained for full-text review. Of these 26 studies, 16 met the eligibility criteria. Figure 1 provides reasons for exclusion of full-text articles. Data were extracted using the Matrix Method, which provides a structure and process for reviewing the literature (Garrard, 2011). The steps of the Matrix Method include: (a) creating a paper trail of the search processes and results, (b) organizing the journal articles and other materials gathered for review, (c) abstracting data from each journal article, and (d) synthesizing abstracted information and writing the literature review (Garrard, 2011). The following information was abstracted and organized into tables: author, year of publication, country, study design, inclusion criteria, sample characteristics, intervention overview, CDE use and measures, and main findings. Two additional authors (D.B., M.K.) reviewed the extracted data. Disagreements were resolved through discussion until consensus was achieved.

Quality Assessment

The Downs and Black checklist, suitable for evaluating the quality of randomized and nonrandomized trials, was used to assess the quality of the included studies (Downs & Black, 1998). The Downs and Black (1998) checklist comprises 27 questions evaluating five subscales: study reporting, external validity, internal validity-bias, internal validity-confounding, and power. One question (question 27), evaluating the power of the study, was modified to a yes/no response (O’Connor et al., 2015; Willems et al., 2015). For each study, the scores for each question were added resulting in a total possible score of 28 (for question 5, 0=no, 1=partially, and 2=yes). The Downs and Black checklist does not provide specific score ranges to differentiate between excellent and poor study quality. As such, we defined the following study quality criteria a priori using previous publications as a guide for determining the quality scores: excellent (28–26), good (25–20), fair (19–15), and poor (≤14; Hooper et al., 2008; Silverman et al., 2012). Three authors (R.W., D.B., and M.K.)assessed the methodological quality of the final 16 studies, and disagreements were resolved through discussion until consensus was achieved.

RESULTS

An overview of the study characteristics of the 16 studies included in this review is provided in Table 2. Of the 16 studies, seven were randomized controlled trials. Studies were conducted in the United States (n = 5), Italy (n = 5), Netherlands (n = 2), Brazil, France, Japan, and Sweden (n = 1 each). Fewer than half the studies (n = 5) were published in the last five years. The sample sizes ranged from 6 to 267, with the majority of studies (n = 12) having a sample size of less than 50. Of the studies that reported gender, race/ethnicity, and marital status, the samples were predominately comprised of Caucasian, married females. Additionally, of the 11 studies that reported the systemic sclerosis subset, people with the limited cutaneous subtype were most represented. Among the fourteen studies where investigators reported the disease duration, all had samples with a mean or median disease duration of five years or greater.

Table 2.

Study Characteristics

Author (year) country Design Inclusion criteria N Control (Y/N) Sample characteristics
Age Female
N (%)		 White
N (%)		 Married
N (%)		 SSc subset
N (%)		 Disease duration
Rehabilitation programs focused on improving physical function
 Antonioli et al. (2009) Italy Case-control*

  1. SSc diagnosis according to LeRoy criteria

  2. Age 18–75 years

  3. Stable disease

  4. No change in antirheumatic treatment in the previous 3 mo.

 33 Y I: Median 66.5 (63–70.5)a
C: Median 57 (50–67)a 		I: 16 (100%)
C: 16 (94.1%)		 I: lcSSc: 12 (75%)
dcSSc: 4 (25%)
C: lcSSc: 11 (65%)
dcSSc: 6 (35%)		 I: Median 14.5 (10–21)a
C: Median 9 (5–13)a
 Maddali-Bongi et al. (2009) Italy RCT* 40 Y 57.8 (11.8)
I: 56.4 (10.2)
C: 58.1 (13.4)		 30 (75%)
I: 16 (80%)
C: 14 (70%)		 40 (100%) 9.0 (3.8)
I: 8.7 (3.5)
C: 9.4 (4.2)
 Maddali-Bongi et al. (2011) Italy RCT

  1. SSc diagnosis according to ACR criteria

  2. Face involvement with a Rodnan skin score ≥1

 40 Y 57.3 (11.3)
I: 57.2 (10.2)
C: 57.4 (12.6)		 34 (85%)
I: 18 (90%)
C: 16 (80%)		 9.4 (43)
I: 9.7 (42)
C: 9.1 (46)
 Mugii et al. (2006) Japan Pretest-posttest*

  1. SSc diagnosis according to ACR criteria

 45 N 48.6 (17.3) 39 (86.7%) lcSSc: 13 (28.9%)
dcSSc: 32 (71.1%)		 5.0 (7.6)
 Rannou et al. (2017) France RCT

  1. Diagnosis of SSc diagnosis according to ACR or LeRoy and Medsger criteria

  2. Age ≥ 18 years

  3. Disability rating of ≥0.5 on the HAQ-DI or symptoms of decreased mouth opening or limited range of motion of at least 1 joint

 218 Y I: 52.7 (14.8)
C: 53.1 (14.4)		 I: 95 (86.4%)
C: 86 (79.6%)		 I: lcSSc: 53 (48.2%)
dcSSc: 53 (48.2%)
Limited: 4 (3.6%)
C:
lcSSc: 50 (47.2%)
dcSSc: 54 (50.9%)
Limited: 2 (1.9%)		 I: 6.5 (6.5)
C: 6.7 (8.6)
 Stefanantoni et al. (2016) Italy RCT

  1. Diagnosis of SSc according ACR criteria

  2. Able to speak and read Italian

  3. Have hand involvement

  4. Have stable disease

  5. Never underwent an OT intervention

 31 Y I: 61.4
C: 60.5		 I: 15 (100%)
C: 15 (93%)		 I: lcSSc: 8 (53.3%)
dcSSc: 7 (46.6%)
C: lcSSc: 10 (62.5%)
dcSSc: 5 (31.2%)		 I: 10.8 (2–50)b
C: 14.1 (2–50)b
 Uras et al. (2019) Italy RCT

  1. Diagnosis of SSc according to ACR/EULAR criteria

  2. Age ≥ 18 years

  3. Ability to understand Italian language

  4. Subjective perception of problems with opening mouth as confirmed by a dermatologist

  5. Signed informed consent

 63 Y I: 54.6 (15.8)
C: 55.2 (13.3)		 I: 21 (65.6%)
C: 18 (58.1%)		 I: 8.7 (6.3)
C: 11.8 (8.4)
Self-management programs focused on improving disease-related knowledge
 Khanna et al. (2019) USA RCT

  1. Diagnosis of SSc

  2. Age ≥18 years

  3. US resident

  4. Have basic computer literacy and access to a computer with internet and email

  5. Have English communication skills

  6. Be willing to complete the study protocol

 267 Y 53.7 (11.7)
I: 54.3 (10.1)
C: 52.9 (13.1)		 243 (91%)
I: 123 (91.8%)
C: 120 (90.2%)		 221 (82.8%)
I: 112 (83.6%)
C: 120 (90.2%)		 170 (63.7%)
I: 95 (70.9%)
C: 75 (56.4%)		 lcSSc/sine: 120 (44.9%)
dcSSc: 115 (43.1%)
Overlap: 31 (11.6%)
Unknown: 1 (0.4%)
I: lcSSc/sine: 57 (42.5%)
dcSSc: 57 (42.5%)
Overlap: 19 (14.1%)
Unknown: 1 (0.7%)
C: lcSSc/sine: 63 (47.4%)
dcSSc: 58 (43.6%)
Overlap: 12 (9%)
Unknown: 0		 8.97 (8.5)
I: 8.72 (7.8)
C: 9.23 (9.2)
 Landim et al. (2019) Brazil Quasi-exper.

  1. SSc diagnosis according to 2013 ACR/EULAR classification criteria

  2. Age ≥18 years

  3. Have hand involvement

  4. Stable drug therapy in the last 3 mo.

  5. Willingness to complete study protocol

 22 N 48.1 (11.7) 18 (85.71%) 13 (61.90%) 15 (66.67%) lcSSc: 16 (71.4%)
dcSSc: 6 (28.6%)		 11.19 (5.9)
 Poole et al. (2013) USA Pretest-posttest

  1. Diagnosis of SSc

  2. Age ≥ 21 years

  3. US resident

  4. Ability to communicate in English

  5. Willingness to complete study protocol

 49 N 53.9 (12.5) 45 (92%) 40 (82%) 36 (73%) 6.9 (7.1)
 Poole et al. (2014) USA Pretest-posttest, Pilot

  1. Diagnosis of SSc

  2. Age ≥18 years

  3. Resides in US

  4. Have basic computer literacy and access to computer with internet and email

  5. Ability to communicate in English

  6. Have moderate to severe pain (pain score of >3 on 0–10 VAS)

  7. Have poor self-efficacy pain scores (score of <7 on 0–10 VAS)

  8. Willing to complete study protocol

 16 N 52.2 (10.2) 87.5% 93.7% 68.8% lcSSc: 54.4%
dcSSc: 37.5%
Unknown: 6.2%		 7.8 (8.1)
Multidisciplinary team programs focused on improving disease-related knowledge and physical function
 Kwakkenbos et al. (2011) Netherlands Pretest-posttest

  1. SSc diagnosis for more than 1 yr.

 41 N 52.8 (12.2) Total: 83% Total: 73% lcSSc: 26 (63.4%)
dcSSc: 13 (31.7%)
Unknown: 2 (4.9%)		 9.5 (10.5)
 Samuelson et al. (2000) Sweden Pilot

  1. Expressed an interest in attending the education course

  2. Had a multisymptomatic medical history of SSc, including pain

 6 N 62 (47–74)b 6 (100%) 8 (4–11)b,c
 Schouffer et al. (2011) Netherlands RCT

  1. Diagnosis of SSc according to LeRoy criteria

  2. Age 18–75 years

  3. Able to cycle on bicycle ergometer

  4. Stable anti-inflammatory medication over the past 2 mo.

  5. Fluent in Dutch

 53 Y I: 53.9 (10.8)
C: 51.7 (10.8)		 I: 19 (67.9%)
C: 21 (84%)		 I: dcSSc: 15 (53.6%)
C: dcSSc: 15 (60%)		 I: Median 6.5 (IQR 8.2)
C: Median 8.2 (IQR 10.5)
 Other
 Hunnicutt et al. (2008) USA Cohort*

  1. SSc diagnosis according ACR criteria

  2. Disease duration <5 years

  3. Enrolled in GENISOS between 1997 – 2004

 36 Y I: 54.2 (8.9)
C: 48.7 (12.2)		 I: 17 (89%)
C: 13 (86%)		 I: 68%
C: 47%		 I: dcSSc: 9 (47%)
C: dcSSc: 5 (33%)
 Sallam et al. (2007) USA Explor.

  1. Diagnosis of SSc according to ACR criteria

  2. At least 1 upper or lower GI symptom

 17 Y 57.2 (1.9) 14 (82.4%) lcSSc: 8 (47.1%)
dcSSc: 9 (52.9%)
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Note. Mean (standard deviation) for age and disease duration, unless otherwise indicated; total values for the entire sample provided, unless specified by I (intervention group) and C (control group); areas left blank indicate information was not stated;

*

author interpretation due to unstated study design;

a

median (25th –75th percentile);

b

mean (range);

c

duration of SSc symptoms;

ACR, American College of Rheumatology; C, control; dcSSc, diffuse cutaneous systemic sclerosis; EULAR, European League Against Rheumatism; Explor., Exploratory; GENISCOS, Genetics versus Environment in Scleroderma Outcomes Study; GI, gastrointestinal; HAQ-DI, Health Assessment Questionnaire Disability Index; IQR, interquartile range; I, intervention; lcSSc, limited cutaneous systemic sclerosis; mo., months; OT, occupation therapy; Quasi-exper., Quasi-experimental; RCT, randomized controlled trial; SD, standard deviation; SSc, systemic sclerosis; VAS, visual analog scale; yr., year.

Overview of the Interventions

There was notable variability in the types of self-management interventions reported in the 16 studies (see Table 3). Self-management interventions focused on improving physical function through rehabilitation programs that included stretching, individualized exercise sessions, or physical/occupational therapy were the most common type of intervention (n = 7). The second most common type of self-management intervention was programs aimed at increasing participants’ knowledge of systemic sclerosis and improving patient-reported outcomes (n = 4), followed by multidisciplinary team educational programs (n = 3), complementary and alternative medicine therapies (n = 1) and transcutaneous electrical nerve stimulation (n = 1). Half of the interventions (n = 8) included a health care professional as the interventionist, of which occupational therapists were most common. The length of the self-management interventions ranged from six days (three weekends) to one year. The investigators of ten studies included an intervention control group, however, the type of control group (e.g., waitlist control, attention control) was not described in the majority of studies. Additionally, Samuelson and Ahlmén (2000) were the only investigators to describe the theory used to guide the development of their self-management intervention (i.e., the self-efficacy theory).

Table 3.

Overview of the Interventions

Author (year) Intervention description Length Delivery Interventionist
Rehabilitation programs focused on improving physical function
 Antonioli et al. (2009) Rehabilitation program consisting of individualized exercise sessions and a home exercise program to improve disability, QOL, hand mobility, and physical activity 4 mo. In-person & at home
 Maddali-Bongi et al. (2009) Rehabilitation program consisting of connective tissue massage, Mc Mennell joint manipulation, and a home exercise program to improve hand function 9 wks. In person & at home Therapist
 Maddali-Bongi et al. (2011) Rehabilitation program consisting of Kabat’s technique, connective tissue massage, kinesitherapy, and a home exercise program to improve facial function 9 wks. In person & at home
 Mugii et al. (2006) Self-administered finger stretching program to improve finger joint motion 1 yr. In person & at home OT
 Rannou et al. (2017) Physical therapy program consisting of supervised and personalized sessions and a home exercise program to improve disability 12 mo. In-person & at home Physio.
OT
 Stefanantoni et al. (2016) Occupational therapy and self-administered stretching program for the hands to improve functional ability, health status, personal perception, and QOL 3 mo. In-person & at home OT
 Uras et al. (2019) Rehabilitation program including stretching exercises, educational materials (brochures & DVD), and face-to-face meetings to reduce microstomia 12 mo. In-person & at home Clinical & research nurse
Self-management programs focused on improving disease-related knowledge
 Khanna et al. (2019) Self-management program including online modules and a discussion board to improve self-efficacy and patient-reported outcomes 16 wks. Online
 Landim et al. (2019) Self-management program including workbook and DVD for home use to improve hand function 8 wks. At home
 Poole et al. (2013) Self-management program including workbook and exercise DVD for home use to improve self-efficacy, functional ability, pain, fatigue, depression, and health-related events 3–4 mo. At home
 Poole et al. (2014) Self-management program including online modules, an exercise video, worksheets, and a discussion board to improve self-efficacy, health efficacy, management of care, functional disability, pain, fatigue, and depression 10 wks. Online
Multidisciplinary team programs focused on improving disease-related knowledge and physical function
 Kwakkenbos et al. (2011) Multidisciplinary team psychoeducational program with educational modules and a group Thai Chi lesson to increase patients’ knowledge of SSc and its treatment, improve understanding of the multidisciplinary treatment options, and increase patient interaction 3 wknds. In-person SW
Rheum.
OT
PT
Psychol.
Nurse
 Samuelson et al. (2000) Multidisciplinary team education program to improve patients’ knowledge about SSc, reduce concerns related to SSc, facilitate daily activities, form realistic goals, and connect with other patients with SSc 5 wks. In-person OT (primary)
Rheum.
PT
Dietician
SW
Nurse
 Schouffer et al. (2011) Multidisciplinary team care program including group exercises, group education, individual treatments, and home-based exercise program to improve measures of health status 12 wks. In-person & at home Rheum.
OT
PT
SW
Nurse
Other
 Hunnicut et al. (2008) Complementary and alternative medicine therapies for symptom management
 Sallam et al. (2007) Transcutaneous electrical nerve stimulation on GI symptoms, QOL, and heart rate variability 14 days In-person & at home
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Note. Areas left blank indicate information was not stated; length refers to the length of the intervention; delivery refers to whether the intervention included an in-person component (e.g., group sessions) and/or was delivered at home (by self); interventionalist refers to members of the healthcare team involved in the intervention study; GI, gastrointestinal; invn, intervention; mo., months; OT, occupational therapist; PT, physical therapist; physio., physiotherapist; psychol., psychologist; QOL, quality of life; rheum., rheumatologist; SW, social worker; SSc, systemic sclerosis; wknds., weekends; wks., weeks.

Self-management Outcomes

Among the 16 studies, only three of the four self-management outcomes (i.e., patient activation, self-efficacy, and global health) were measured, and there was significant variability in the measures used to assess these outcomes (see Table 4). Patient activation was the only self-management outcome measured using a NINR-recommended measure (i.e., the Patient Activation Measure). Self-regulation was not measured as an outcome in any of the studies.

Table 4.

Intervention Efficacy and Findings

Author (year) CDE Self-mgmt. outcomes General outcomes
PA SE SR GH
Antonioli et al. (2009) SF-36
SGRQ
HAQ-DI

  • Improvement in the physical and mental health components of the SF-36 at 4 months in 69% and 62% of patients who received the intervention

  • Improvement in the perceived QOL in airway disease (SGRQ) in 67% of patients

  • Progressive improvement in disability (HAQ-DI) over time for patients in the intervention group, but did not reach statistical significance

  • Improvement in exercise tolerance and hand mobility (HAMIS)
Hunnicut et al. (2008) SF-36

  • Higher mean physical and mental health component scores of the SF-36 at year 2 in CAM users (compared to non-CAM users)

  • CAM users had significantly higher SF-36 scores for the physical component, role physical, bodily pain, and vitality at year 2

  • CAM therapies were used for symptoms such as arthritis, pain, GI dysfunction, and fatigue

  • Approximately 70% used a CAM therapy for more than 1 year
Khanna et al. (2019) PAM PROMIS Self-Efficacy PROMIS-29v2
EQ-5D
QALYs
PHQ-8

  • No significant difference in PAM, PROMIS Self-Efficacy, PROMIS-29v2, and PHQ-8 scores between the intervention and control groups

  • Significant change in EQ-5D index scores from baseline to follow up

  • No significant difference in Brief-SWAP scores between the intervention and control group
Kwakkenbos et al. (2011) HAQ-DI
SHAQ
IRGL
Pain VAS
Fatigue VAS

  • No significant change in depressive mood and physical functioning (HAQ-DI, pain, fatigue) after the intervention

  • Patients had lower levels of helplessness and higher acceptance of limitations post-intervention and after 6 months

  • No significant change in coping
Landim et al. (2019) SF-36
HAQ-DI
SHAQ
Pain VAS

  • Significant improvement in physical and mental health components of SF-36 (role physical, physical functioning, social functioning, bodily pain, vitality, and mental health) after the intervention

  • Significant improvement in HAQ and SHAQ scores after the intervention

  • Significant improvement in finger motion, hand strength, moisturizing and cold avoidance habits, hand pain, CHFS, and impact of Raynaud’s phenomenon after the intervention
Maddali-Bongi et al. (2009) SF-36
HAQ

  • In intervention group, significant improvement in the physical and mental health components of the SF-36 and HAQ scores

  • In intervention group, significant improvement in fist closure, HAMIS test, and CHFS after the intervention

  • Control group which included only at home daily exercises showed improvement in fist closure only
Maddali-Bongi et al. (2011) SF-36
HAQ-DI

  • No significant change in SF-36 and HAQ scores in intervention group at the end of treatment (T1) and after 9 weeks of follow up (T2)

  • Mouth opening improved for the intervention and control group at the end of treatment, but only stayed significant after 9 weeks of follow up for the intervention group

  • Significant decrease in facial skin score after the end of treatment and at 9 weeks; no change in the control group

  • MHISS scores improved significantly in the intervention group at the end of treatment; no change in the control group
Mugii et al. (2006) HAQ-DI

  • After 1 year of the intervention, there was no significant change in the mean HAQ-DI score

  • When the individual components of the HAQ-DI were explored, only eating and gripping significantly improved after one year; components requiring lower extremity function did not change (i.e., dressing, arising, walking, hygiene, reaching, activities)

  • Total passive range of motion improved in each finger after 1 month of the intervention and was maintained 1 year after
Poole et al. (2013) ASES HAQ
SFAQ
CES-D
Pain VAS
MAF

  • Self-efficacy for controlling pain and self-efficacy “other” improved after the intervention, but only self-efficacy for pain had a statistically significant change

  • Depression, pain, and fatigue decreased after the intervention, but were not statistically significant

  • Participants felt they could better manage their pain after the intervention

  • No significant change in the number of doctor or emergency department visits, and number of hospital night stays in the past 6 months
Poole et al. (2014) PAM CDSES HAQ-DI
CES-D
Pain VAS
Fatigue VAS

  • Significant improvements in patient activation scores after the intervention

  • Self-efficacy improved after the intervention, but not significantly

  • No significant change in functional ability or pain

  • Significant changes in depression and fatigue scores

  • Significant improvements in health efficacy and ability to manage care

  • No significant changes in self-reported health
Rannou et al. (2017) SF-36
HAQ-DI
SHAQ
MACTAR
Pain VAS

  • No significant between-group difference in the primary outcome, disability (HAQ-DI), after the intervention was delivered (at 12 months)

  • HAQ-DI, SHAQ, and MACTAR scores improved at 1 month in the intervention group

  • Microstomia improved at 1,6, and 12 months in the intervention group

  • Reduction in microstomia, improvement in global hand mobility, and reduction in hand disability at 1 month in the intervention group
Sallam et al. (2007) SF-36

  • Prolonged TENS to GI acupoints resulted in improvement in physical functioning scores of the SF-36; no significant change in other domains of SF-36

  • TENS application significantly improved sympathetic and vagal activities versus baseline in the acute study phase

  • Prolonged TENS resulted in significant decreases in GI symptom scores and normalized sympathetic balance
Samuelson et al. (2000) ASES HAQ
GH VAS
PDWB
Pain VAS

  • Improvement in the self-efficacy pain subscale in 3 out of 5 patients after the intervention

  • The self-efficacy function subscale remained stable

  • Improvement in self-efficacy to cope with physical and psychological symptoms

  • The professional medical information met the participants’ needs

  • Participants were satisfied with the program and felt they had adequate opportunities to connect with and share their SSc experiences

  • Goal setting was an unfamiliar task for participants
Schouffer et al. (2011) SF-36
SHAQ

  • Significant improvement in SHAQ scores in the intervention group at 12 weeks

  • No significant improvement in SF-36 scores between those in intervention group and control group

  • Significant improvement in grip strength, MMO, 6MWD in the intervention group at 12 weeks

  • Significant improvement in grip strength persisted at 24 months
Stefanantoni et al. (2016) SF-36
HAQ
COPM

  • At 3 months, HAQ and mental health component of the SF-36 improved in the intervention group

  • In the control group, significant improvement in the HAQ scores and mental health component of SF-36 at 1 month and 3 months

  • Significant improvement in COPM Satisfaction and COPM Performance scores in the intervention group after 1 and 3 months

  • No significant improvement in hand function (DHI) scores over time in the intervention or control group
Uras et al. (2019) Skindex-17
GHQ-12
SySQ

  • No significant difference in QOL between the intervention and control group

  • Significant increase in mouth opening in the intervention group at 12-month follow up

  • The difference in mouth opening between the intervention and control group was not significant, but reached significance in per-protocol analysis
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Note. Global Health included both physical and mental health, such as physical function, quality of life, and psychological measures; location-specific disability scales (i.e., hand function, mouth function) were not included in global health; self-management outcomes refer to study findings related to the four common data elements; general outcomes refer to other study findings; areas left blank indicate information was not stated; 6MWD, 6-minute walk distance; ASES, Arthritis Self-Efficacy Scale; Brief-SWAP, Brief Satisfaction with Appearance Scale; CAM, complementary and alternative medicine; CDE, common data element; CDSES, Chronic Disease Self-Efficacy Scale; CES-D, Center for Epidemiologic Studies Depression Scale; CHFS, Cochin Hand Function Scale; COPM, Canadian Occupational Performance Measure; DHI, Duruoz Hand Index; EQ-5D, EuroQol 5D; GH, global health; GH, global health; GHQ-12, General Health Questionnaire-12; GI, gastrointestinal; HAMIS, Hand Mobility in Scleroderma; HAQ-DI, Health Assessment Questionnaire Disability Index; IRGL, Impact of Rheumatic Diseases on General Health and Lifestyle; MACTAR, McMaster Toronto Arthritis Patient Preference Disability Questionnaire; MAF, Multidimensional Assessment of Fatigue; MHISS, Mouth Handicap in Systemic Sclerosis; MMO, maximal mouth opening; PA, patient activation; PAM, Patient Activation Measure; PDWB, Psychological General Well-Being index; PHQ-8, Patient Health Questionnaire 8; PROMIS-29v2; Patient-Reported Outcomes Measurement Information System-29 Profile version 2.0; PROMIS Self-Efficacy, Patient-Reported Outcomes Measurement Information System Self-Efficacy for Managing Chronic Conditions; QOL, quality of life; QALYs, quality-adjusted life years; SE, self-efficacy; self-mgmt., self-management; SF-36, 36-Item Short Form Survey; SFAQ, Scleroderma Functional Assessment Questionnaire; SGRQ, Saint George’s Respiratory Questionnaire; SHAQ, Scleroderma Health Assessment Questionnaire; SR, self-regulation; SySQ, Systemic Sclerosis Questionnaire; TENS, transcutaneous electrical nerve stimulation; VAS, visual analog scale.

Patient activation.

As shown in Table 4, Khanna et al. (2019) and Poole et al. (2014) evaluated patient activation before and after an online self-management program. Both studies used the NINR-recommended measure for patient activation in self-management research (i.e., the Patient Activation Measure) to assess this outcome. In the study by Poole et al. (2014), the investigators found significant improvements in patient activation scores and moderate effect sizes post-intervention among 16 participants. In a randomized controlled trial consisting of 267 participants with relatively high levels of patient activation, Khanna et al. (2019) found no statistically significant difference in patient activation scores between participants randomized to an internet-based self-management program and those that received a patient-focused educational book.

Self-efficacy.

Self-efficacy was included as an outcome in four studies. Khanna et al. (2019) and Poole et al. (2013; 2014) evaluated the effectiveness of a self-management program on patient-reported measures including self-efficacy. Samuelson and Ahlmén (2000) evaluated the effectiveness of a multidisciplinary team education program on self-efficacy. Different measures were used to assess self-efficacy, including the Arthritis Self-Efficacy Scale (ASES; Poole et al., 2013; Samuelson & Ahlmén, 2000), the Chronic Disease Self-Efficacy Scale (CDSES; Poole et al., 2014), and the Patient Reported Outcomes Measurement Information System Self-Efficacy for Managing Chronic Conditions measure (PROMIS Self-Efficacy; Khanna et al., 2019).

The interventions used in these studies demonstrated differing effects on self-efficacy. For example, Poole et al. (2013) found that self-efficacy for controlling pain improved after participation in a mail-delivered self-management program. Similarly, Samuelson and Ahlmén (2000) found that self-efficacy for controlling pain and other disease-related symptoms improved after a multidisciplinary team education program, however, the sample only had six participants. For the two studies in which investigators evaluated participation in an online self-management program, no statistically significant improvements in self-efficacy were found (Poole et al., 2014, Khanna et al., 2019).

Global health.

Global health, another self-management CDE which reflects one’s overall health, was described through measures of physical and/or mental health. Physical health was most frequently assessed using a functional disability measure (n = 12) and mental health was most frequently measured through quality of life (n = 9). The investigators of nearly half the studies (n = 7) evaluated both functional disability and quality of life, of which the Health Assessment Questionnaire and the 36-Item Short Form Survey were most commonly used. No studies, however, used the NINR-recommended outcome measure for global health (i.e., PROMIS SF v1.1 Global).

The investigators reported improvements in global health (i.e., physical and/or mental health) after the intervention in half the studies (n = 8). Among the different types of interventions, rehabilitation programs consisting of stretching and/or at home exercises were most commonly associated with improved global health (Antonioli et al., 2009; Maddali Bongi et al., 2009; Stefanantoni et al., 2016). Next most common were self-management programs that focused on increasing participants’ disease-related knowledge (Landim et al., 2019; Poole et al., 2014) and multidisciplinary team education programs that included educational sessions and exercise information (Schouffoer et al., 2011). Other self-management interventions such as complementary and alternative medicine therapies (Hunnicutt et al., 2008) and transcutaneous nerve stimulation (Sallam et al., 2007) also led to improvements in global health.

Self-management Intervention Efficacy

Effect sizes (Cohen’s d) were reported in three studies (Kwakkenbos et al., 2011; Landim et al., 2019; Poole et al., 2014). Effect sizes below .50 were considered small, above .50 but below .80 were considered medium, and above .80 were considered large. Kwakkenbos and colleagues (2011) evaluated a multidisciplinary team psychoeducational program and found no improvement in self-management outcomes as demonstrated by effect sizes (physical functioning: d = −0.06 and −0.09; pain: d = 0.10 and 0.13; fatigue: d = 0.02 and 0.05). Poole et al. (2014) found small effect sizes for self-efficacy (d = 0.46) and pain (d = −0.31), and medium effect sizes for patient activation (d = 0.62), depression (d = −0.71), and fatigue (d = −0.55). In a self-management program focused on improving hand function, Landim et al. (2019) reported medium and large effect sizes for components of global health including role physical (d = 0.61), social function (d = 0.64), bodily pain (d = 0.58), and mental health (d = 0.84) of the 36-Item Short Form Survey. Medium effect sizes were reported for functional disability (d = 0.53) using the Health Assessment Questionnaire Disability Index, whereas large effect sizes were found using a more disease-specific measure (i.e., the Scleroderma Health Assessment Questionnaire) for functional disability (d = 1.01; Landim et al., 2019).

Study Quality

The methodological quality of the sixteen studies ranged from poor to good (see Table 5). Almost half the studies were classified as having poor methodological quality (n = 7; quality score ≤ 14). No studies had excellent methodological quality. The most common limitations were related to power and external validity—the majority of studies did not include a power analysis (n = 12) and nearly half (n = 7) did not meet any of the criteria for external validity (e.g., representativeness of the participants to their source population, representativeness of where treatment was received by the majority of patients).

Table 5.

Quality Assessment

Author (year) Study quality
(11)* 		External validity
(3)* 		Internal validity-bias
(7)* 		Internal validity-confounding
(6)* 		Power
(1)* 		Total score+
(28)
Antonioli et al. (2009) 8 1 4 2 0 15
Hunnicut et al. (2008) 6 0 3 2 0 11
Khanna et al. (2019) 9 1 5 5 1 21
Kwakkenbos et al. (2011) 8 0 2 1 0 11
Landim et al. (2019) 9 1 3 3 0 16
Maddali-Bongi et al. (2009) 8 1 4 3 0 16
Maddali-Bongi et al. (2011) 8 1 4 3 0 16
Mugii et al. (2006) 5 0 4 0 0 9
Poole et al. (2013) 7 0 4 1 0 12
Poole et al. (2014) 6 0 4 1 0 11
Rannou et al. (2017) 11 1 6 6 1 25
Sallam et al. (2007) 6 0 4 0 0 10
Samuelson et al. (2000) 3 1 3 0 0 7
Schouffer et al. (2011) 10 0 6 6 1 23
Stefanantoni et al. (2016) 8 2 6 4 0 20
Uras et al. (2019) 9 1 5 5 1 21
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Note.

*

maximum score in each subscale;

+

interpretation of total score: excellent (28–26), good (25–20), fair (19–15), and poor (≤14).

DISCUSSION

In this systematic review, we evaluated the state of the science of self-management interventions and their impact on self-management outcomes in adults with systemic sclerosis. The significant variability in the types of self-management interventions, their content, and measured outcomes posed limitations in interpreting and recommending the use of self-management interventions in adults with systemic sclerosis. Additionally, fewer than half the studies included in this systematic review (n = 5) were conducted in the last five years, over which time treatment for systemic sclerosis has changed (Barsotti et al., 2019).

Findings from this systematic review highlight the poor methodological quality of studies on self-management interventions in systemic sclerosis and underscore the need for larger and more rigorously designed self-management studies, such as randomized controlled trials, in this population. The studies included in this systematic review had predominately small samples, contributing to their poor methodological quality due to inadequate power and external validity. For example, only four studies were adequately powered (Khanna et al., 2019; Rannou et al., 2017; Schouffoer et al., 2011; Uras et al., 2019), and only three studies calculated effect sizes for the outcomes after intervention delivery (Kwakkenbos et al., 2011; Landim et al., 2019; Poole et al., 2014).

Our findings are similar to those found in a systematic review exploring nonpharmacologic interventions in systemic sclerosis in which only three high-quality randomized controlled trials reported medium to large effect sizes out of 23 total studies (Willems et al., 2015). An obstacle to conducting well-powered randomized controlled trials in this population is the small number of patients at a single study center (Willems et al., 2015). As such, international collaborations have been established to conduct large, adequately powered, multi-site randomized controlled trials to improve patient-centered care (Kwakkenbos et al., 2013).

Patient-activation, self-efficacy, self-regulation, and global health were included as self-management outcomes in this systemic review. Many studies, however, did not assess all four outcomes. Self-regulation was not evaluated as an outcome in any of the studies. Instead, many interventions included self-regulation activities such as goal setting, planning and action, and self-evaluation. Global health was most commonly evaluated through a variety of patient-reported outcomes including physical function, pain, fatigue, depression, and quality of life. No studies, however, used the NINR-recommended outcome measures for self-management research (i.e., Patient Activation Measure, Self-Efficacy for Managing Chronic Disease scale, Index of Self-Regulation, and PROMIS Global Health scale). Only one of the recommended measures, the Self-Efficacy for Managing Chronic Disease scale, has been validated in systemic sclerosis (Riehm et al., 2016).

Large systemic sclerosis databases such as the Scleroderma Patient-centered Intervention Network (SPIN), the Canadian Scleroderma Research Group (CSRG), and the European Scleroderma Trials and Research group (EUSTAR) should consider the inclusion of key self-management outcomes and their recommended measures. Such an approach would support evaluating the unique constructs of self-management and comparing findings across studies and populations to advance the science of self-management.

Limitations

This systematic review had several limitations. Only studies published in English were included, potentially leading to missed studies and a limited ability to assess all self-management interventions. Only studies that evaluated the effect of a self-management intervention on one or more of the self-management outcomes were included. The studies included in this systematic review did not report treatment fidelity, thereby limiting the ability to determine if the intervention had a true effect or if bias was potentially introduced. Additionally, the majority of studies included in this systematic review were inadequately powered. The studies included in this systematic review were also limited by homogenous samples, thereby reducing generalizability of their findings.

CONCLUSION

In this systematic review we assessed the components of self-management interventions and their efficacy on key self-management outcomes in adults with systemic sclerosis. A variety of self-management interventions were included but lacked consistency in their effects on self-management outcomes. The low methodological quality of the studies included in this systematic review, however, limited synthesis of and recommendations for self-management interventions in adults with systemic sclerosis. Larger and more rigorously designed self-management studies are needed to support their use in future treatment recommendations. Future research should consider the use of key self-management outcomes, identified as CDEs, in systemic sclerosis to allow for comparisons across studies, improve generalizability of study findings, and advance the science of self-management in systemic sclerosis.

Funding information

National Institute of Nursing Research, Grant/Award Number: F31NR019007; Jonas Center for Nursing Excellence, Grant/Award Number: Jonas Nurse Scholar

References

  1. Allanore Y, Simms R, Distler O, Trojanowska M, Pope J, Denton CP, & Varga J (2015). Systemic sclerosis. Nature Reviews Disease Primers, 1, 1–21. 10.1038/nrdp.2015.2 [DOI] [PubMed] [Google Scholar]
  2. Almeida C, Almeida I, & Vasconcelos C (2015). Quality of life in systemic sclerosis. Autoimmunity Reviews, 14, 1087–1096. 10.1016/j.autrev.2015.07.012 [DOI] [PubMed] [Google Scholar]
  3. Antonioli CM, Bua G, Frigè A, Prandini K, Radici S, Scarsi M, … Airo P (2009). An individualized rehabilitation program in patients with systemic sclerosis may improve quality of life and hand mobility. Clinical Rheumatology, 28, 159–165. 10.1007/s10067-008-1006-x [DOI] [PubMed] [Google Scholar]
  4. Barlow J, Wright C, Sheasby J, Turner A, & Hainsworth J (2002). Self-management approaches for people with chronic conditions: A review. Patient Education and Counseling, 48, 177–187. 10.1016/S0738-3991(02)00032-0 [DOI] [PubMed] [Google Scholar]
  5. Barsotti S, Orlandi M, Codullo V, Di Battista M, Lepri G, Rossa A. Della, & Guiducci S (2019). One year in review 2019: Systemic sclerosis. Clinical and Experimental Rheumatology, 37(4), 3–14. [PubMed] [Google Scholar]
  6. Bernatsky S, Hudson M, Panopalis P, Clarke AE, Pope J, LeClercq S, … Ligier S (2009). The cost of systemic sclerosis. Arthritis Care and Research, 61(1), 119–123. 10.1002/art.24086 [DOI] [PubMed] [Google Scholar]
  7. Chifflot H, Fautrel B, Sordet C, Chatelus E, & Sibilia J (2008). Incidence and Prevalence of Systemic Sclerosis: A Systematic Literature Review. Seminars in Arthritis and Rheumatism, 37(4), 223–235. 10.1016/j.semarthrit.2007.05.003 [DOI] [PubMed] [Google Scholar]
  8. Cohen MZ, Thompson CB, Yates B, Zimmerman L, & Pullen CH (2015). Implementing common data elements across studies to advance research. Nursing Outlook, 63(2), 181–188. 10.1016/j.outlook.2014.11.006 [DOI] [PMC free article] [PubMed] [Google Scholar]
  9. Downs SH, & Black N (1998). The feasibility of creating a checklist for the assessment of the methodological quality both of randomised and non-randomised studies of health care interventions. Journal of Epidemiology and Community Health, 52(6), 377–384. 10.1136/jech.52.6.377 [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. Du S, & Yuan C (2010). Evaluation of patient self-management outcomes in health care: a systematic review. International Nursing Review, 57(2), 159–167. 10.1111/j.1466-7657.2009.00794.x [DOI] [PubMed] [Google Scholar]
  11. Fischer A, Zimovetz E, Ling C, Esser D, & Schoof N (2017). Humanistic and cost burden of systemic sclerosis: A review of the literature. Autoimmunity Reviews, 16(11), 1147–1154. 10.1016/j.autrev.2017.09.010 [DOI] [PubMed] [Google Scholar]
  12. Fleury J (1998). The Index of Self-Regulation: Development and Psychometric Analysis. Journal of Nursing Measurement, 6(1), 3–17. 10.1891/1061-3749.6.1.3 [DOI] [PubMed] [Google Scholar]
  13. Garrard J (2011). Health Sciences Literature Review Made Easy. Retrieved from https://books.google.com/books?id=RjDDeEEO6qUC
  14. Grady PA, & Gough LL (2018). Self-management: A comprehensive approach to management of chronic conditions. American Journal of Public Health, 104(8), e25–e31. 10.2105/AJPH.2014.302041 [DOI] [PMC free article] [PubMed] [Google Scholar]
  15. Grey M, Knafl K, & McCorkle R (2006). A framework for the study of self- and family management of chronic conditions. Nursing Outlook, 54(5), 278–286. 10.1016/j.outlook.2006.06.004 [DOI] [PubMed] [Google Scholar]
  16. Hays RD, Bjorner JB, Revicki DA, Spritzer KL, & Cella D (2009). Development of physical and mental health summary scores from the patient-reported outcomes measurement information system (PROMIS) global items. Quality of Life Research, 18(7), 873–880. 10.1007/s11136-009-9496-9 [DOI] [PMC free article] [PubMed] [Google Scholar]
  17. Hibbard JH, Mahoney ER, Stockard J, & Tusler M (2005). Development and testing of a short form of the patient activation measure. Health Services Research, 40(6), 1918–1930. 10.1111/j.1475-6773.2005.00438.x [DOI] [PMC free article] [PubMed] [Google Scholar]
  18. Hibbard JH, Stockard J, Mahoney ER, & Tusler M (2004). Development of the Patient Activation Measure (PAM): Conceptualizing and Measuring Activation in Patients and Consumers. Health Services Research, 39(4), 1005–1026. 10.1111/j.1475-6773.2004.00269.x [DOI] [PMC free article] [PubMed] [Google Scholar]
  19. Higgins JP, Thomas J, Chandler J, Cumpston M, Li T, Page MJ, & Welch VA (2019). Cochrane Handbook for Systematic Reviews of Interventions. In Cochrane Handbook for Systematic Reviews of Interventions. 10.1002/9781119536604 [DOI] [PMC free article] [PubMed] [Google Scholar]
  20. Hooper P, Jutai JW, Strong G, & Russell-Minda E (2008). Age-related macular degeneration and low-vision rehabilitation: A systematic review. Canadian Journal of Ophthalmology, 43(2), 180–187. 10.3129/I08-001 [DOI] [PubMed] [Google Scholar]
  21. Hunnicutt SE, Grady J, & McNearney TA (2008). Complementary and Alternative Medicine Use Was Associated With Higher Perceived Physical and Mental Functioning in Early Systemic Sclerosis. Explore: The Journal of Science and Healing. 10.1016/j.explore.2008.04.004 [DOI] [PMC free article] [PubMed] [Google Scholar]
  22. Ingegnoli F, Ughi N, & Mihai C (2018). Update on the epidemiology, risk factors, and disease outcomes of systemic sclerosis. Best Practice and Research: Clinical Rheumatology, 32(2), 223–240. 10.1016/j.berh.2018.08.005 [DOI] [PubMed] [Google Scholar]
  23. Khanna D, Serrano J, Berrocal VJ, Silver RM, Cuencas P, Newbill SL, … Poole JL (2019). Randomized Controlled Trial to Evaluate an Internet-Based Self-Management Program in Systemic Sclerosis. Arthritis Care and Research, 71(3), 435–447. 10.1002/acr.23595 [DOI] [PMC free article] [PubMed] [Google Scholar]
  24. Kowal-Bielecka O, Fransen J, Avouac J, Becker M, Kulak A, Allanore Y, … Imbert B (2017). Update of EULAR recommendations for the treatment of systemic sclerosis. Annals of the Rheumatic Diseases, 76(8), 1327–1339. 10.1136/annrheumdis-2016-209909 [DOI] [PubMed] [Google Scholar]
  25. Kwakkenbos L, Bluyssen SJM, Vonk MC, Van Helmond AF, Van Den Ende CHM, Van Den Hoogen FHJ, & Van Lankveld WGJM (2011). Addressing patient health care demands in systemic sclerosis: Pre-post assessment of a psycho-educational group programme. Clinical and Experimental Rheumatology, 29, S60–S65. [PubMed] [Google Scholar]
  26. Kwakkenbos L, Jewett LR, Baron M, Bartlett SJ, Furst D, Gottesman K, … Thombs BD (2013). The Scleroderma Patient-centered Intervention Network (SPIN) Cohort: Protocol for a cohort multiple randomised controlled trial (cmRCT) design to support trials of psychosocial and rehabilitation interventions in a rare disease context. BMJ Open, 3(8), e003563. 10.1136/bmjopen-2013-003563 [DOI] [PMC free article] [PubMed] [Google Scholar]
  27. Kwakkenbos L, Thombs BD, Khanna D, Carrier ME, Baron M, Furst DE, … Bartlett SJ (2017). Performance of the Patient-Reported Outcomes Measurement Information System-29 in scleroderma: A Scleroderma Patient-centered Intervention Network Cohort Study. Rheumatology (Oxford), 56(8), 1302–1311. 10.1093/rheumatology/kex055 [DOI] [PMC free article] [PubMed] [Google Scholar]
  28. Landim SF, Bertolo MB, Marcatto de Abreu MF, Del Rio AP, Mazon CC, Marques-Neto JF, … de Paiva Magalhães E (2019). The evaluation of a home-based program for hands in patients with systemic sclerosis. Journal of Hand Therapy, 32, 313–321. 10.1016/j.jht.2017.10.013 [DOI] [PubMed] [Google Scholar]
  29. Liberati A, Altman DG, Tetzlaff J, Mulrow C, Gøtzsche PC, Ioannidis JPA, … Moher D (2009). The PRISMA statement for reporting systematic reviews and meta-analyses of studies that evaluate healthcare interventions: explanation and elaboration. PLoS Medicine, 6(7), e1000100. 10.1136/bmj.b2700 [DOI] [PMC free article] [PubMed] [Google Scholar]
  30. Liem SIE, Vliet Vlieland TPM, Schoones JW, & de Vries-Bouwstra JK (2019). The effect and safety of exercise therapy in patients with systemic sclerosis: a systematic review. Rheumatology Advances in Practice, 3(2), rkz044. 10.1093/rap/rkz044 [DOI] [PMC free article] [PubMed] [Google Scholar]
  31. Lorig KR, Sobel DS, Ritter PL, Laurent D, & Hobbs M (2001). Effect of a self-management program on patients with chronic disease. Effective Clinical Practice, 4(6), 256–262. [PubMed] [Google Scholar]
  32. Lorig KR, & Holman HR (2003). Self Management Education: History, Definition, Outcomes and Mechanisms. Annals of Behavioral Medicine, 26(1), 1–7. [DOI] [PubMed] [Google Scholar]
  33. Maddali Bongi S, Del Rosso A, Galluccio F, Sigismondi F, Miniati I, Conforti ML, … Matucci Cerinic M (2009). Efficacy of connective tissue massage and Mc Mennell joint manipulation in the rehabilitative treatment of the hands in systemic sclerosis. Clinical Rheumatology, 28, 1167–1173. 10.1007/s10067-009-1216-x [DOI] [PubMed] [Google Scholar]
  34. Mayes MD (2003). Scleroderma epidemiology. Rheumatic Disease Clinics of North America, 29(2), 239–254. 10.1016/S0889-857X(03)00022-X [DOI] [PubMed] [Google Scholar]
  35. Moher D, Liberati A, Tetzlaff J, Altman DG, Altman D, Antes G, … Tugwell P (2009). Preferred reporting items for systematic reviews and meta-analyses: The PRISMA statement. PLoS Medicine, 6(7), e1000097. 10.1371/journal.pmed.1000097 [DOI] [PMC free article] [PubMed] [Google Scholar]
  36. Moore SM, Schiffman R, Waldrop-Valverde D, Redeker NS, McCloskey DJ, Kim MT, … Grady P (2016). Recommendations of Common Data Elements to Advance the Science of Self-Management of Chronic Conditions. Journal of Nursing Scholarship, 48(5), 437–447. 10.1111/jnu.12233 [DOI] [PMC free article] [PubMed] [Google Scholar]
  37. Mugii N, Hamaguchi Y, & Maddali-Bongi S (2018). Clinical significance and usefulness of rehabilitation for systemic sclerosis. Journal of Scleroderma and Related Disorders, 3(1), 71–80. 10.1177/2397198317750043 [DOI] [PMC free article] [PubMed] [Google Scholar]
  38. O’Connor SR, Tully MA, Ryan B, Bradley JM, Baxter GD, & McDonough SM (2015). Failure of a numerical quality assessment scale to identify potential risk of bias in a systematic review: A comparison study. BMC Research Notes, 8(1), 1–7. 10.1186/s13104-015-1181-1 [DOI] [PMC free article] [PubMed] [Google Scholar]
  39. Poole JL, Mendelson C, Skipper B, & Khanna D (2014). Taking charge of systemic sclerosis: A pilot study to assess the effectiveness of an internet self-management program. Arthritis Care and Research, 66(5), 778–782. 10.1002/acr.22192 [DOI] [PubMed] [Google Scholar]
  40. Poole JL, Skipper B, & Mendelson C (2013). Evaluation of a mail-delivered, print-format, self-management program for persons with systemic sclerosis. Clinical Rheumatology, 32, 1393–1398. 10.1007/s10067-013-2282-7 [DOI] [PubMed] [Google Scholar]
  41. Rannou F, Boutron I, Mouthon L, Sanchez K, Tiffreau V, Hachulla E, … Poiraudeau S (2017). Personalized Physical Therapy Versus Usual Care for Patients With Systemic Sclerosis: A Randomized Controlled Trial. Arthritis Care and Research, 69(7), 1050–1059. 10.1002/acr.23098 [DOI] [PubMed] [Google Scholar]
  42. Riehm KE, Kwakkenbos L, Carrier ME, Bartlett SJ, Malcarne VL, Mouthon L, … Mills SD (2016). Validation of the Self-Efficacy for Managing Chronic Disease Scale: A Scleroderma Patient-Centered Intervention Network Cohort Study. Arthritis Care and Research. 10.1002/acr.22807 [DOI] [PubMed] [Google Scholar]
  43. Ryan P, & Sawin KJ (2009). The Individual and Family Self-Management Theory: Background and perspectives on context, process, and outcomes. Nursing Outlook, 57(4), 217–225. 10.1016/j.outlook.2008.10.004 [DOI] [PMC free article] [PubMed] [Google Scholar]
  44. Sallam H, McNearney TA, Doshi D, & Chen JDZ (2007). Transcutaneous electrical nerve stimulation (TENS) improves upper GI symptoms and balances the sympathovagal activity in scleroderma patients. Digestive Diseases and Sciences. 10.1007/s10620-006-9257-3 [DOI] [PubMed] [Google Scholar]
  45. Samuelson UK, & Ahlmén EM (2000). Development and evaluation of a patient education program for persons with systemic sclerosis (scleroderma). Arthritis & Rheumatism, 13(3), 141–148. [DOI] [PubMed] [Google Scholar]
  46. Schouffoer AA, Ninaber MK, Beaart-Van De Voorde LJJ, Van Der Giesen FJ, De Jong Z, Stolk J, … Vliet Vlieland TPM (2011). Randomized comparison of a multidisciplinary team care program with usual care in patients with systemic sclerosis. Arthritis Care and Research, 63(6), 909–917. 10.1002/acr.20448 [DOI] [PubMed] [Google Scholar]
  47. Silverman SR, Schertz LA, Yuen HK, Lowman JD, & Bickel CS (2012). Systematic review of the methodological quality and outcome measures utilized in exercise interventions for adults with spinal cord injury. Spinal Cord, 50(10), 718–727. 10.1038/sc.2012.78 [DOI] [PubMed] [Google Scholar]
  48. Stefanantoni K, Sciarra I, Iannace N, Vasile M, Caucci M, Sili Scavalli A, … Riccieri V (2016). Occupational therapy integrated with a self-administered stretching programme on systemic sclerosis patients with hand involvement. Clinical and Experimental Rheumatology, 34, S157–S161. [PubMed] [Google Scholar]
  49. Thombs BD, Van Lankveld W, Bassel M, Baron M, Buzza R, Haslam S, … Worron-Sauve M (2010). Psychological health and well-being in systemic sclerosis: State of the science and consensus research agenda. Arthritis Care and Research, 62(8), 1181–1189. 10.1002/acr.20187 [DOI] [PubMed] [Google Scholar]
  50. Uras C, Mastroeni S, Tabolli S, Masini C, Pallotta S, Teofoli P, … Abeni D (2019). A comparison between two educational methods in the rehabilitation of the microstomia in systemic sclerosis: a randomized controlled trial. Clinical Rehabilitation, 33(11), 1747–1756. 10.1177/0269215519858395 [DOI] [PubMed] [Google Scholar]
  51. van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, … Pope JE (2013). 2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative. Annals of the Rheumatic Diseases, 72(11), 1747–1755. 10.1136/annrheumdis-2013-204424 [DOI] [PubMed] [Google Scholar]
  52. Willems LM, Vriezekolk JE, Schouffoer AA, Poole JL, Stamm TA, Boström C, … Van Den Ende CHM (2015). Effectiveness of Nonpharmacologic Interventions in Systemic Sclerosis: A Systematic Review. Arthritis Care and Research, 67(10), 1426–1439. 10.1002/acr.22595 [DOI] [PubMed] [Google Scholar]
  53. Yeom HA, Choi M, Belyea M, & Fleury J (2011). Psychometric evaluation of the index of self-regulation. Western Journal of Nursing Research, 33(2), 268–285. 10.1177/0193945910378854 [DOI] [PubMed] [Google Scholar]

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