BackgroundNailfold videocapillaroscopy (NVC) is an imaging technique representing a reliable tool for the classification, diagnosis and monitoring of systemic sclerosis (SSc) patients. The capillaroscopic skin ulcer index (CSURI) was suggested to identify patients at risk of developing digital ulcers (DU) [1].ObjectivesThis study aims (1) to describe the practicality of the CSURI in clinical practice, (2) to describe the change of CSURI during follow-up, and (3) to assess associations between the change in CSURI and demographic and disease characteristics.MethodsThis Swiss multicentre, prospective, observational study analysed SSc patients with a history of active DU. Demographic characteristics and routine clinical data were recorded and patients underwent NVC at baseline and at the follow-up visit.The centres' investigators were trained to perform NVC and, the nailfolds of 8 fingers (digit 2–5 on both hands) were examined if possible. All images were stored centrally and separately analysed by two extensively trained and experienced reviewers (OD & UAW) separately.In each NVC image the total number of capillaries in the distal row, the number of megacapillaries and the maximum diameter of the megacapillaries were assessed. Based on these 3 characteristics, the CSURI was calculated according to Sebastiani et al. [1] for patients with at least 1 megacapillary present. Data were analysed descriptively.ResultsBetween 2011 and 2015, 61 patients fulfilled the eligibility criteria and had at least one follow-up visit (median observation time 1.0 years, interquartile range [IQR] 1.0–1.1). Of these patients, more than a third (reviewer 1: n=24 patients, 39%; reviewer 2: n=26 patients, 43%) had no megacapillaries present on NVC on any assessed finger and hence the CSURI could not be calculated for those either at baseline or follow-up. Therefore, this analysis is based on the remaining 34 patients who had at least one megacapillary present on NVC at baseline and at follow-up by both reviewers (26% male; median age 57 years, IQR 48–65 years).The median baseline CSURI scores according to reviewer 1 was 5.3 (IQR 2.6–16.3) increasing to a median of 5.9 (1.3–12.0) at follow-up. The CSURI as evaluated by reviewer 2 reduced from baseline (median 6.4, IQR 2.4–12.5) to follow-up (5.0, IQR 1.7–10.0).None of the assessed demographic or disease characteristics (Box 1) were associated with the changes in the CSURI between baseline and follow-up for the scores obtained by reviewer 1 and reviewer 2 at the same time; although the limited sample size should be kept in mind interpreting this lack of association.ConclusionsIn this study, around 40% of patients could not be evaluated with the CSURI due to the absence of megacapillaries on NVC. Clinical decisions based on the CSURI in routine practice should be made with caution, as it can vary greatly between assessors even if they are extensively trained.References Sebastiani M, et al. Predictive role of capillaroscopic skin ulcer risk index in systemic sclerosis: a multicentre validation s...
BackgroundCalcinosis is a disabling, rarely discussed manifestation of SSc for which the natural history and management is poorly understood. This investigation is the 1st phase of a multi-tiered project to understand calcinosis from patients' perspectives creating the groundwork for a SSc-calcinosis PROM.MethodsFive focus groups and individual interviews in the US and UK were recorded and transcribed verbatim. Pathophysiologic and life impact were elicited with: 1. Since developing calcinosis how has your life changed over time? 2. How has calcinosis changed over time? A final probe was a request for questions that a clinician could ask to understand if calcinosis was better, worse or same.Transcripts were analysed by hand (highest method) by an iterative inductive process (content drives coding) by at least 5 independent analysts including at least one patient research partner. Concepts were triangulated until a comprehensive set of concepts emerged. Occurrence was quantified per participant.ResultsTwenty-three patients (29/31 female, 27/31 white, with mean disease duration 18.1 years) were consented and interviewed. Responses spanned broadly to include concepts of self-management strategies and recurrent hypotheses relating calcinosis development to trauma, Raynaud's and cold exposure (tables 1 & 2).Cold exposure and Raynaud's were a perceived association to calcinosis severity - “when they are cold mine always open back up”.A majority of patients engage in strategies to extrude calcinosis with either pressure +/- soaking or at home surgical techniques. “I actually have homemade surgical tools to get these out.”The following anchors were consistently suggested for physicians to assess calcinosis severity: pain level, size, frequency, number and functional impairment. A 2-step question was suggested to help differentiate ulcer, infection and calcinosis symptoms: 1st regarding predomiant wound character and then target the related calcinosis.ConclusionsThese results provide the groundwork for and conclude the 1st steps (item collection) in PROM development. As suggested by patients, a composite of scales anchored in pain, size, frequency, number and related impairment may reasonably serve as an interim instrument for SSc calcinosis. Next steps are validating content with a large subject base and questionniare development with subsequent validation.Very importantly, patients' observations and self-management behavior provide opportunities to learn from and to preemptively educate physicians and patients. Patients are eager for self-management guidance. An essential deliverable of this work will be a patient-physician education guide on calcinosis management.AcknowledgementsIn memory of Anne Mawdsley, founder: Raynaud's & Scleroderma Association UK – tireless engine for education and advocacy raising >£10 million for SSc research.Disclosure of InterestA. Christensen: None declared, S. Khalique: None declared, S. Cenac: None declared, K. Fligelstone: None declared, V. Jaeger: None declared, A. Mawdsley: None declare...
BackgroundSystemic sclerosis (SSc) is a rare, multisystem autoimmune disorder. It is characterised by generalized microangiopathy, in which hypoxia and oxidative stress have been implicated in its pathogenesis. Tobacco inhalation increases free radicals and strongly promotes vascular damage. So far, data available with regards to a role of tobacco exposure with SSc severity and progression are scarce.ObjectivesWe aimed to assess the effects of smoking on the speed of worsening of organ manifestations, namely lung involvement (forced vital capacity, FVC; forced expiratory volume, FEV1/FVC ratio; diffusing capacity for carbon monoxide corrected for alveolar volume, DLCO/VA), skin involvement (modified Rodnan skin score; mRSS), and digital ulcers (DU) in the European scleroderma trials and research (EUSTAR) database.MethodsAdult SSc patients from the EUSTAR cohort with a follow-up visit 12±4 months after their baseline visit and available data on their smoking habits were included.The associations of smoking behaviour (never smokers vs ex-smokers vs current smokers) with the disease manifestations at follow up were assessed after adjusting for potentially confounding covariates using multivariable linear or logistic regression analyses.Missing data were imputed using multiple imputations.ResultsOf the 3,023 patients included (mean age 57 years, SD 13; 85% female), 66% stated that they never smoked, 23% were ex-smokers and 11% were current smokers. On average, ex-smokers had smoked for 19.5 years (SD 12) while current smokers smoked for 29.1 years (SD 13). Ex-smokers had smoked on average 17.3 pack-years (SD 20) and current smokers 29.3 pack-years (SD 36). The mean time since smoking cessation in ex-smokers was 15.8 years (SD13).The FEV1/FVC ratio changed from 96.3 (SD 14) at baseline to 96.2 (SD 13) at follow up. Taking into account the effect of age, sex, autoantibody status, disease duration and SSc subset as well as the baseline values of the outcome, the FEV1/FVC ratio decreased faster in currently smoking SSc patients than in never smokers (β=-2.8, p=0.001). Similarly, the DLCO/VA diminished faster in current smokers (β=-3.7, p=0.006) while on average the DLCO/VA decreased from 78.1 (SD 18) to 76.5 (SD 17). Changes in FVC were however not associated with smoking behaviour (p=0.13).The mRSS changed between baseline and follow-up from an average of 7.6 (SD 7) to 7.3 (SD 7); on average, in smokers the mRSS improved faster than in never smokers (β=-0.65, p=0.015). The presence of DUs at follow-up was not associated with current smoking (p=0.9).Having previously smoked was not significantly associated with the progression of any of the assessed outcomes.ConclusionsAlthough the adverse effects of smoking on bronchial airways that is known in the general population could be replicated in the SSc population, the lack of a measurable adverse effect of smoking on SSc organ manifestations argues against a major role of tobacco associated free radicals and vasoconstriction in the pathogenesis of SSc vasculopathy and fibrosi...
BackgroundSSc is a complex, diffuse, devastating health condition of vascular injury, inflammation and fibrosis resulting in multiple organ involvement with high impact on survival and quality of life.Demonstrated research activity tends to define SSc Centers of Excellence (SCoE) certification. However, SSc complications require coordinated high-level multi-specialty expert care. The Scleroderma Foundation, Scleroderma Australia and Scleroderma Society UK engaged SSc patients and SSc health providers (HPs) in a multi-tiered process to assess priorities in recognition of SCoEs.MethodsA mixed methods design ensured comprehensive item collection in addressing “important qualities and services in a certified SSc Center of Excellence”. A core of 35 patients & SSc HPs from 8 countries initiated the study through an iterative process using nominal group technique with rounds of item revision until saturation and satisfaction of proposed survey content was achieved and subsequently field-tested with a 5 point scale (critical to low importance).Participation was screened and “gate-controlled” with online survey access through a unique one-time link. Telephone interview was offered. Responses from SSc patients and HPs were compared by Pearson's χ2 or Fisher's exact tests as appropriate.ResultsInitial phases yielded a 54 item survey and field-tested in 15 SSc patients & HPs. 400 patients and SSc HPs received surveys of which 299 from 19 countries (75% response rate) were completers. Expert care superseded research as a priority of “critical importance” by HPs & patients respectively at 69% & 48% (p=0.02) and by 94% & 89% (p=0.8) when “critical to very important” were collapsed. 3 questions provided internal cross-validation of this query. “SCoEs should engage in research” received 57% of patients and 48% of HPs (p=0.37) as being critical. Further, education, rehabilitative services and support networks were consistently highly rated items with topics stratified by ratings (tables 1 & 2). Discrepant areas of importance between patients and HPs are highlighted in tables.ConclusionsParticipation was robust in all project stages emphasizing the perceived global importance of this effort. Though research is of clear importance, quality expert care incorporating rehabilitative and educational provisions is a SCoE operational priority. These findings signal the need to redefine SCoE certification standards and provide a roadmap to SCoE development.AcknowledgementsDedicated to the memory of Anne Mawdsley.Disclosure of InterestV. Jaeger: None declared, A. Aubin: None declared, N. Baldwin: None declared, K. Fligelstone: None declared, R. Sims: None declared, J. Welling: None declared, R. Burrill: None declared, K. Connolly: None declared, J. Gordon: None declared, T. Frech: None declared, T. Ngcozana: None declared, M. Kowalczyk: None declared, M. Lammi: None declared, J. Lasky: None declared, U. Walker: None declared, L. A. Saketkoo Grant/research support from: ACR/EULAR Exchange Awardee
BackgroundThe management of patients with rheumatic diseases is guided in part by asking patients about their medical history at each clinic visit. Patients often find it difficult to accurately remember the course of their symptoms between these appointments as they are often months apart. Regular app-based patients' self-monitoring of disease activity (with our without feedback to the rheumatologist) between clinic visits might provide a possible solution for this.The COmPASS I study [1] demonstrated that RA patients' self-assessments of disease activity via App correlate strongly with rheumatologists' assessments. Following up on this, the Swiss based COmPASS II study is embedded in the Swiss rheumatology registry (SCQM) and hence allows the linkage of data obtained via the COmPASS II App from the patients with routine clinical data collected in the registry.The main aims of the COmPASS II study are to assess if continuous self-monitoring of the disease by patients optimises disease management and outcome in rheumatic diseases, and to assess the fluctuation of disease activity between clinic visits.ObjectivesThe objectives of this abstract are to describe the set-up and the recruitment of the COmPASS II study in the first 11 months.MethodsThe COmPASS II App questionnaire consists of the RAPID3 score, a validated, commonly used PRO to self-assess disease activity. Additionally, patients are asked about their therapy compliance and cortisone dose.At inclusion, interested patients with RA, axSpA and PsA are electronically randomized into 3 study arms (Figure 1). In arm 1 patients and rheumatologist are displayed the self-assessed disease activity over time, the patient directly via the App and the rheumatologist via the SCQM registry. In arm 2 only the patients are displayed their disease activity chart and in study arm 3 neither sees the recorded data. Patients are encouraged to fill in the App weekly.ResultsThe COmPASS II App went online on the 15/02/2016. In the first 11 months of COmPASS II, 272 patients were enrolled by their rheumatologist. 64% of patients used the WebApp (32% in arm 1, 38% in arm 2 and 30% in arm 3); 82% of patients filled in the questionnaires for longer than a months, the longest follow-up was 11 months. On average patients use the App every 2 weeks.Patients found the App easy to use “The COmPASS II WebApp is so easy to use. It doesn't even take me 2 min.” and received feedback included “Now my rheumatologist sees how I was since the last appointment instead of me trying to remember how I was half a year ago.”.ConclusionsThe COmPASS II study will validate the utility of app-based patients' self-assessments in enhancing disease control in a treat to target approach and deliver numerous additional scientific data.References Mueller R et al. Patient's Self-Monitoring Via Smartphone: The Compass Study - COrrelation between PAtient self-ASSessment of rheumatoid arthritis disease activity via smartphone technology and physicians' validated scores [abstract]. Arthritis Rheumatol. 2014;66(S10). A...
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