Multifactorial pathways contributing to the development and impact of foot problems in systemic sclerosis (scleroderma)

Systemic sclerosis (SSc) is a heterogeneous connective tissue disease characterised by vasculopathy, immune activation and fibrosis [1-3]. The multisystem nature of the disease has a wide-ranging impact on the patient’s overall health on physical, psychological and psychosocial levels [4] [5-7]. While foot problems in patients with SSc have been previously described and that their presence is associated with disability [8-10], the impact of such problems and its major contributors has yet to be determined. The underpinning hypothesis of this thesis was that the development and impact of foot problems on the Quality of Life (QoL) of patients with SSc is multifactorial; involving a complex inter-relationship between disease, functional impairment, personal factors, environmental factors and psychosocial factors. In order to explore this hypothesis, a multiple methodological approach was employed. First, a literature review and a consultation with clinical experts was undertaken to identify the potential candidate factors that may contribute to foot problems. Second, a case-control, cross-sectional study of 121 patients with SSc and 51 healthy participants was undertaken in order to investigate the impact of factors that contribute to foot problems. Finally, the pathway by which the candidate factors that were identified as contributing to foot pathology impacted on the overall quality of life in people with SSc was explored. Using data from the same 121 patients with SSc, structural equation modelling (SEM) was used to explore the inter-relationships between multifactorial pathways associated with foot pathology and its impact on patients with SSc. The results from this thesis can be summarised as follows: i) patients with SSc have significant foot problems; ii) SSc has both a physical and psychological impact; iii) foot problems are affected by complex interrelations between multiple factors; and iv) foot problems are a significant contributor to the impact on the quality of life of patients with SSc

PSYCHOMETRIC CHARACTERISTICS OF A SPANISH TRANSLATION OF THE MANCHESTER FOOT PAIN AND DISABILITY INDEX: VALIDATION AND RASCH ANALYSIS

Background: The Manchester Foot Pain and Disability Index (MFPDI) is a self-assessment questionnaire developed in the UK to measure foot pain and disability in the general population1. It has been translated and validated in several languages 2,3 Objectives: The aim of this study was to conduct cross-cultural adaptation and validation of the MFPDI into Spanish Methods: The cross-cultural adaptation process was undertaken using International Society for Pharmacoeconomics and Outcomes Research (ISPOR)4 recommendations. This involved 8 stages: i) Forward translation, ii) Reconciliation, iii) Back translation, iv) Back translation review, v) Harmonisation, vi) Pilot, vii) Pilot review, and viii) Proofreading. In the validation phase, the MFPDI datasets from the UK (original) and Spain (adapted) were pooled and subjected to Rasch analysis. Fit to the Rasch model, unidimensionality, reliability and cross-cultural invariance is reported Results: The pooled dataset comprised 1015 patients (Spain n=333 and UK n=682) with characteristics summarised in Table 1. Rasch analysis confirmed three subscales for both the Spanish and UK datasets and fit to the Rasch model X2 (df) = 15.945 (12), p = 0.194 and 31.024 (21), p = 0.073, for Spain and UK . The reliability (Person Separation Index - PSI) was .85 and .82 for Spain and UK respectively. Significant cross-cultural non-invariance was present on the Functional and Personal appearance subscales. Adjustment for the bias was achieved by ‘splitting’ the affected subscales and creation of cultural-specific subscales for each country and cultural-general subscale. Fit to the Rasch model was satisfied following cross-cultural adjustment (Table 1). The MFPDI was calibrated into interval-level scales for Spain and UK to enable future data pooling or comparisons. Sampl e size Gender Age Item Fit Residual Person Fit Residual Chi Square Interaction PSI Analysis N Female (%) Mean (SD) Mean SD Mean SD Value (df) p Spain 333 248 (74.4) 51.6 (15.2) -0.164 3.07 - 0.364 0.93 15.95 (12) 0.19 0.85 UK 682 416 (61.0) 55.2 (16.7) -0.366 2.80 - 0.429 0.99 31.02 (21) 0.07 0.82 Pooled 1015 663 (65.4) 52.8 (15.8) -0.766 4.40 - 0.441 1.06 49.17 (27) 0.01 0.84 DIFAdjusted -0.420 2.98 - 0.415 0.98 57.94 (45) 0.09 0.84 Table 1: P = Χ2 interaction probability, (non-significant P = Fit to the model), PSI = Person separation index Conclusions: A gold standard translation process (ISPOR) has been used to develop a Spanish (for Spain) version of the MFPDI, a widely used foot-specific patient-reported outcome measure. Rasch analysis has confirmed that the MFPDI is a robust 3-subscale measure of foot pain, function and appearance in both its English and Spanish versions. Future work can make cross-cultural comparisons using the calibrated scale

Common measure of quality of life for people with systemic sclerosis across seven European countries: A cross-sectional study

© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. Objectives the aim of this study was to adapt the Systemic Sclerosis Quality of Life Questionnaire (SScQoL) into six European cultures and validate it as a common measure of quality of life in systemic sclerosis (SSc). Methods this was a seven-country (Germany, France, Italy, Poland, Spain, Sweden and UK) cross-sectional study. A forward–backward translation process was used to adapt the English SScQoL into target languages. SScQoL was completed by patients with SSc, then data were validated against the rasch model. to correct local response dependency, items were grouped into the following subscales: function, emotion, sleep, social and pain and reanalysed for fit to the model, unidimensionality and cross-cultural equivalence. Results the adaptation of the SScQoL was seamless in all countries except Germany. Cross-cultural validation included 1080 patients with a mean age 58.0 years (Sd 13.9) and 87% were women. Local dependency was evident in individual country data. Grouping items into testlets corrected the local dependency in most country specific data. Fit to the model, reliability and unidimensionality was achieved in six-country data after cross-cultural adjustment for Italy in the social subscale. the SScQoL was then calibrated into an interval level scale. Conclusion the individual SScQoL items have translated well into five languages and overall, the scale maintained its construct validity, working well as a five-subscale questionnaire. Measures of quality of life in SSc can be directly compared across five countries (France, Poland Spain, Sweden and UK). data from Italy are also comparable with the other five countries although require an adjustment

Pressure and pain In Systemic sclerosis/Scleroderma - an evaluation of a simple intervention (PISCES): randomised controlled trial protocol

Background: foot problems associated with Systemic Sclerosis (SSc)/Scleroderma have been reported to be both common and disabling. There are only limited data describing specifically, the mechanical changes occurring in the foot in SSc. A pilot project conducted in preparation for this trial confirmed the previous reports of foot related impairment and reduced foot function in people with SSc and demonstrated a link to mechanical etiologies. To-date there have been no formal studies of interventions directed at the foot problems experienced by people with Systemic Sclerosis. The primary aim of this trial is to evaluate whether foot pain and foot-related health status in people with Systemic Sclerosis can be improved through the provision of a simple pressure-relieving insole. Methods: the proposed trial is a pragmatic, multicenter, randomised controlled clinical trial following a completed pilot study. In four participating centres, 140 consenting patients with SSc and plantar foot pain will be randomised to receive either a commercially available pressure relieving and thermally insulating insole, or a sham insole with no cushioning or thermal properties. The primary end point is a reduction in pain measured using the Foot Function Index Pain subscale, 12 weeks after the start of intervention. Participants will complete the primary outcome measure (Foot Function Index pain sub-scale) prior to randomisation and at 12 weeks post randomisation. Secondary outcomes include participant reported pain and disability as derived from the Manchester Foot Pain and Disability Questionnaire and plantar pressures with and without the insoles in situ. Discussion: this trial protocol proposes a rigorous and potentially significant evaluation of a simple and readily provided therapeutic approach which, if effective, could be of a great benefit for this group of patients

Management of systemic sclerosis: British Society for Rheumatology guideline scope

This guideline will provide a practical roadmap for management of SSc that builds upon the previous treatment guideline to incorporate advances in evidence-based treatment and increased knowledge about assessment, classification and management. General approaches to management as well as treatment of specific complications will be covered, including lung, cardiac, renal and gastrointestinal tract disease, as well as RP, digital vasculopathy, skin manifestations, calcinosis and impact on quality of life. It will include guidance related to emerging approved therapies for interstitial lung disease and account for National Health Service England prescribing policies and national guidance relevant to SSc. The guideline will be developed using the methods and processes outlined in Creating Clinical Guidelines: Our Protocol. This development process to produce guidance, advice and recommendations for practice has National Institute for Health and Care Excellence accreditation

A survey of foot orthoses prescription habits amongst podiatrists in the UK, Australia and New Zealand

Background: Foot orthoses are frequently used but little is known about which types are used in contemporary practice. This study aimed to explore the types of foot orthoses currently used by podiatrists and the prescription variations in a range of conditions. Methods: A web-based, cross-sectional survey was distributed through professional bodies in the United Kingdom (UK), Australia, and New Zealand. Questions focussed on foot orthosis prescription habits in relation to 26 conditions affecting the back and lower limb. Results: Two hundred and sixty-four podiatrists practising in 19 different countries completed the survey; the majority practised in the UK (47%, n=124), Australia (30%, n=79) and New Zealand (12%, n=32). Respondents qualified between 1968 and 2016, and 147 (56%) were female. Respondents worked in different healthcare sectors and this varied between countries: 42 (34%) respondents in the UK worked solely in the public sector, compared to 3 (4%) in Australia and 2 (6%) in New Zealand. Forty-four (35%) respondents in the UK worked solely in private practice, compared to 64 (81%) in Australia and 14 (44%) in New Zealand. UK respondents prescribed more prefabricated orthoses per week (mean 5.5 pairs) than simple insole-type devices (±2.7) and customised devices (±2.9). Similarly, respondents in New Zealand prescribed more prefabricated orthoses per week (±7.7) than simple (±1.4) and customised (±2.8) devices. In contrast, those in Australia prescribed more customised orthoses per week (±4.4) than simple (±0.8) and prefabricated (±1.9) orthoses. Differences in the types of orthoses prescribed were observed between country of practice, working sector, and the condition targeted. Generally, prefabricated orthoses were commonly prescribed for the 26 highlighted conditions in the UK and New Zealand. Australian podiatrists prescribed far fewer devices overall, but when they did prescribe, they were more likely to prescribe custom devices. Respondents in all three countries were more likely to prescribe customised orthoses for people with diabetes complicated by peripheral neuropathy than for diabetes without this complication. Conclusions: Foot orthosis prescription habits vary between countries. Prefabricated orthoses were frequently prescribed in the UK and New Zealand, and customised orthoses in Australia. Prescriptions for people with diabetes differed depending on the presence of neuropathy, despite a lack of robust evidence supporting these decisions. This study provides new insight into contemporary practice

Medial longitudinal arch development of school children : The College of Podiatry Annual Conference 2015: meeting abstracts

Background Foot structure is often classified into flat foot, neutral and high arch type based on the variability of the Medial Longitudinal Arch (MLA). To date, the literature provided contrasting evidence on the age when MLA development stabilises in children. The influence of footwear on MLA development is also unknown. Aim This study aims to (i) clarify whether the MLA is still changing in children from age 7 to 9 years old and (ii) explore the relationship between footwear usage and MLA development, using a longitudinal approach. Methods We evaluated the MLA of 111 healthy school children [age = 6.9 (0.3) years] using three parameters [arch index (AI), midfoot peak pressure (PP) and maximum force (MF: % of body weight)] extracted from dynamic foot loading measurements at baseline, 10-month and 22-month follow-up. Information on the type of footwear worn was collected using survey question. Linear mixed modelling was used to test for differences in the MLA over time. Results Insignificant changes in all MLA parameters were observed over time [AI: P = .15; PP: P = .84; MF: P = .91]. When gender was considered, the AI of boys decreased with age [P = .02]. Boys also displayed a flatter MLA than girls at age 6.9 years [AI: mean difference = 0.02 (0.01, 0.04); P = .02]. At baseline, subjects who wore close-toe shoes displayed the lowest MLA overall [AI/PP/MF: P < .05]. Subjects who used slippers when commencing footwear use experienced higher PP than those who wore sandals [mean difference = 31.60 (1.44, 61.75) kPa; post-hoc P = .04]. Discussion and conclusion Our findings suggested that the MLA of children remained stable from 7 to 9 years old, while gender and the type of footwear worn during childhood may influence MLA development. Clinicians may choose to commence therapy when a child presents with painful flexible flat foot at age 7 years, and may discourage younger children from wearing slippers when they commence using footwear

Oral abstracts 3: RA Treatment and outcomesO13. Validation of jadas in all subtypes of juvenile idiopathic arthritis in a clinical setting

Background: Juvenile Arthritis Disease Activity Score (JADAS) is a 4 variable composite disease activity (DA) score for JIA (including active 10, 27 or 71 joint count (AJC), physician global (PGA), parent/child global (PGE) and ESR). The validity of JADAS for all ILAR subtypes in the routine clinical setting is unknown. We investigated the construct validity of JADAS in the clinical setting in all subtypes of JIA through application to a prospective inception cohort of UK children presenting with new onset inflammatory arthritis. Methods: JADAS 10, 27 and 71 were determined for all children in the Childhood Arthritis Prospective Study (CAPS) with complete data available at baseline. Correlation of JADAS 10, 27 and 71 with single DA markers was determined for all subtypes. All correlations were calculated using Spearman's rank statistic. Results: 262/1238 visits had sufficient data for calculation of JADAS (1028 (83%) AJC, 744 (60%) PGA, 843 (68%) PGE and 459 (37%) ESR). Median age at disease onset was 6.0 years (IQR 2.6-10.4) and 64% were female. Correlation between JADAS 10, 27 and 71 approached 1 for all subtypes. Median JADAS 71 was 5.3 (IQR 2.2-10.1) with a significant difference between median JADAS scores between subtypes (p < 0.01). Correlation of JADAS 71 with each single marker of DA was moderate to high in the total cohort (see Table 1). Overall, correlation with AJC, PGA and PGE was moderate to high and correlation with ESR, limited JC, parental pain and CHAQ was low to moderate in the individual subtypes. Correlation coefficients in the extended oligoarticular, rheumatoid factor negative and enthesitis related subtypes were interpreted with caution in view of low numbers. Conclusions: This study adds to the body of evidence supporting the construct validity of JADAS. JADAS correlates with other measures of DA in all ILAR subtypes in the routine clinical setting. Given the high frequency of missing ESR data, it would be useful to assess the validity of JADAS without inclusion of the ESR. Disclosure statement: All authors have declared no conflicts of interest. Table 1Spearman's correlation between JADAS 71 and single markers DA by ILAR subtype ILAR Subtype Systemic onset JIA Persistent oligo JIA Extended oligo JIA Rheumatoid factor neg JIA Rheumatoid factor pos JIA Enthesitis related JIA Psoriatic JIA Undifferentiated JIA Unknown subtype Total cohort Number of children 23 111 12 57 7 9 19 7 17 262 AJC 0.54 0.67 0.53 0.75 0.53 0.34 0.59 0.81 0.37 0.59 PGA 0.63 0.69 0.25 0.73 0.14 0.05 0.50 0.83 0.56 0.64 PGE 0.51 0.68 0.83 0.61 0.41 0.69 0.71 0.9 0.48 0.61 ESR 0.28 0.31 0.35 0.4 0.6 0.85 0.43 0.7 0.5 0.53 Limited 71 JC 0.29 0.51 0.23 0.37 0.14 -0.12 0.4 0.81 0.45 0.41 Parental pain 0.23 0.62 0.03 0.57 0.41 0.69 0.7 0.79 0.42 0.53 Childhood health assessment questionnaire 0.25 0.57 -0.07 0.36 -0.47 0.84 0.37 0.8 0.66 0.4