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KSR Number: KSRA5322

Effectiveness of nonpharmacologic interventions in systemic sclerosis: a systematic review

Risk of Bias Assessment

Overall summary: High risk of bias in the review

Bottom Line

There is limited evidence on the effectiveness of non-pharmacologic interventions in patients with systemic sclerosis due to a wide variety of interventions and the low methodological quality of the evidence. A language restriction means that some eligible studies may have been missed. Only results for the three highest quality studies were reported in full, this may have biased the conclusions. Further research is required into the effectiveness of non-pharmacologic care for systemic sclerosis with an international consensus on treatment targets, interventions and outcome measures.

Risk of Bias Assessment

Overall summary High risk of bias in the review

High

A language restriction means that some eligible studies may have been missed. Only results for the three highest quality studies were reported in full and this was based on an arbitrary cut-off on the quality score. The other 20 studies were not included in the main synthesis, which may have biased the conclusions.

A. Did the interpretation of findings address all of the concerns identified in Domains 1 to 4? Probably no
B. Was the relevance of identified studies to the review's research question appropriately considered? Probably yes
C. Did the reviewers avoid emphasizing results on the basis of their statistical significance? Probably no
Risk of bias in the review High

Details of Review

Number of studies 23
Number of participants 722
Last search date March 2014
Review type Intervention
Objective To determine the effectiveness of non-pharmacologic interventions on physical functioning and psychological well-being in patients with systemic sclerosis
Population Patients (18 years of age or over) with systemic sclerosis.
Interventions Non-pharmacologic interventions (physical therapy, psychological therapy, occupational therapy, nursing, nutrition therapy, podiatry, education and oral/dental hygiene therapy, excluding medication, surgery or combined interventions with injections).
Comparator NR. Control treatments in the included studies included: usual care, a waiting list, exercises applied to the other hand, no treatment and treatments over a different time schedule.
Outcome Physical functioning including hand function, face function, skin problems, scleroderma visual analogue scale for Raynaud's phenomenon, limitations in activities and aerobic capacity. Psychological well-being including cognition, mood, coping and quality of life.
Study design Randomised clinical trials (RCTs), controlled clinical trials and observational studies.

Results

Nine RCTs, four controlled clinical trials and 10 observational studies were included. However, only the results for the three high-quality RCTs were reported. One study (n = 48) reported that a multifaceted oral health intervention had a large positive effect on mouth hygiene (Loe Silness gingival index) after six months compared with usual dental care (standardised mean difference [SMD] = 1.54, 95% confidence interval [CI], 0.79 to 2.22). However, there was no significant effect of orofacial exercise on maximal mouth opening after six months in patients with an oral aperture size less than 40 mm. The second study (n = 53) reported that compared to usual outpatient care a 12-week multidisciplinary team care programme resulted in significantly improved limitations in activities measured by the six-minute walking test (SMD = 0.75, 95% CI, 0.14 to 1.35, n = 53) and the Scleroderma Health Assessment Questionnaire (SMD 0.89, 95% CI 0.22 to 1.54)). It also had a medium effect on maximal mouth opening (SMD = 0.73, 95% CI, 0.12, 1.31), and large effect on grip strength (SMD = 1.08, 95% CI, 0.42 to 1.70). The third study (n = 35) reported one hour manual lymph drainage for five weeks showed significant improvements in hand function (hand volume, the Hand Mobility in Scleroderma test, visual analogue scale (VAS) hand oedema, and VAS hand pain), limitations in activities (Health Assessment Questionnaire, VAS interference oedema, and VAS interference hand pain) and quality of life (Short Form 36 physical component and mental component summary scores) compared to a waiting list group. Comprehensive rehabilitation for hands and/or face had a statistically significant effect on hand function or dexterity (two studies). Similar results were observed with aerobic training (one study) and stretching (one study), manual lymph drainage (one study) and biofeedback/ deep oscillation (one study). Transcutaneous electrical nerve stimulation (one study) and home total parenteral nutrition (one study) significantly reduced gastrointestinal problems.

Full Risk of Bias Assessment

The research objective was clearly stated and appropriate inclusion criteria were defined. Only studies published in English, German, French, Spanish or Dutch were included. Only studies with ≥10 participants were included.

1.1 Did the review adhere to pre-defined objectives and eligibility criteria? Yes
1.2 Were the eligibility criteria appropriate for the review question? Probably yes
1.3 Were eligibility criteria unambiguous? Probably yes
1.4 Were all restrictions in eligibility criteria based on study characteristics appropriate (e.g. date, sample size, study quality, outcomes measured)? Probably yes
1.5 Were any restrictions in eligibility criteria based on sources of information appropriate (e.g. publication status or format, language, availability of data)? Probably no
Concerns regarding specification of study eligibility criteria High

MEDLINE, EMBASE, CINAHL, PsycINFO, Cochrane Library and Web of Science were searched (from 1990 to March 2014). Reference lists of the relevant articles and review articles were handsearched for additional studies. The search strategy was provided and appeared to be adequate. The searches were restricted to studies published in English, French, German, Spanish and Dutch. Two reviewers were independently involved in the study selection process with disagreements resolved by discussion and consulting a third reviewer.

2.1 Did the search include an appropriate range of databases/electronic sources for published and unpublished reports? Yes
2.2 Were methods additional to database searching used to identify relevant reports? Yes
2.3 Were the terms and structure of the search strategy likely to retrieve as many eligible studies as possible? Probably yes
2.4 Were restrictions based on date, publication format, or language appropriate? Probably no
2.5 Were efforts made to minimise error in selection of studies? Yes
Concerns regarding methods used to identify and/or select studies High

One reviewer extracted the data. Sufficient study characteristics appear to have been extracted to allow interpretation of the results. Methodological quality of the included studies was assessed using the Downs and Black checklist by one reviewer and checked by a second, disagreements were resolved by discussion to reach a consensus. However, the numerical results were not reported for all studies, only for the three highest quality studies.

3.1 Were efforts made to minimise error in data collection? No
3.2 Were sufficient study characteristics considered for both review authors and readers to be able to interpret the results? Probably yes
3.3 Were all relevant study results collected for use in the synthesis? No
3.4 Was risk of bias (or methodological quality) formally assessed using appropriate criteria? Probably yes
3.5 Were efforts made to minimise error in risk of bias assessment? Probably yes
Concerns regarding methods used to collect data and appraise studies High

Only the three highest quality studies were included in the synthesis, the other 20 were excluded. This was based on a quality score cut-off chosen by the authors and may not have been appropriate. Results for the other studies were not fully reported. Meta-analysis was not feasible due to clinical and methodological heterogeneity, so a narrative synthesis was reported.

4.1 Did the synthesis include all studies that it should? No
4.2 Were all pre-defined analyses reported or departures explained? Probably yes
4.3 Was the synthesis appropriate given the degree of similarity in the research questions, study designs and outcomes across included studies? Probably yes
4.4 Was between-study variation minimal or addressed in the synthesis? Probably yes
4.5 Were the findings robust, e.g. as demonstrated through funnel plot or sensitivity analyses? Probably no
4.6 Were biases in primary studies minimal or addressed in the synthesis? Probably yes
Concerns regarding synthesis and findings High

Keywords

  • Exercise Therapy
  • Scleroderma, Systemic