Objective. To refine the previously developed scleroderma (systemic sclerosis [SSc]) gastrointestinal tract (GIT) instrument (SSC-GIT 1.0).Methods. We administered the SSC-GIT 1.0 and the Short Form 36 to 152 patients with SSc; 1 item was added to the SSC-GIT 1.0 to assess rectal incontinence. In addition, subjects completed a rating of the severity of their GIT involvement (from very mild to very severe). Evaluation of psychometric properties included internal consistency reliability, testretest reliability (mean time interval 1.1 weeks), and multitrait scaling analysis. Results. Study participants were mostly women (84%) and white (81%); 55% had diffuse SSc. Self-rated severity of GIT involvement ranged from no symptoms to very mild (39%), mild (21%), moderate (31%), and severe/very severe (9%). Of an initial 53 items in the SSC-GIT 1.0, 19 items were excluded, leaving a 34-item revised instrument (the University of California, Los Angeles Scleroderma Clinical Trial Consortium GIT 2.0 [UCLA SCTC GIT 2.0]). Analyses supported 7 multi-item scales: reflux, distention/bloating, diarrhea, fecal soilage, constipation, emotional well-being, and social functioning. Test-retest reliability estimates were >0.68 and coefficient alphas were >0.67. Participants who rated their GIT disease as mild had lower scores on a 0 -3 scale on all 7 scales. Symptom scales were also able to discriminate subjects with corresponding clinical GIT diagnoses. The Total GIT Score, developed by averaging 6 of 7 scales (excluding constipation), was reliable and provided greater discrimination between mild, moderate, and severe self-rated GIT involvement than individual scales. Conclusion. This study provides support for the reliability and validity of the UCLA SCTC GIT 2.0, an improvement over the SSC-GIT 1.0, and supports a Total GIT Score in SSc patients with GIT.

IntroductionPsychosocial and rehabilitation interventions are increasingly used to attenuate disability and improve health-related quality of life (HRQL) in chronic diseases, but are typically not available for patients with rare diseases. Conducting rigorous, adequately powered trials of these interventions for patients with rare diseases is difficult. The Scleroderma Patient-centered Intervention Network (SPIN) is an international collaboration of patient organisations, clinicians and researchers. The aim of SPIN is to develop a research infrastructure to test accessible, low-cost self-guided online interventions to reduce disability and improve HRQL for people living with the rare disease systemic sclerosis (SSc or scleroderma). Once tested, effective interventions will be made accessible through patient organisations partnering with SPIN.Methods and analysisSPIN will employ the cohort multiple randomised controlled trial (cmRCT) design, in which patients consent to participate in a cohort for ongoing data collection. The aim is to recruit 1500–2000 patients from centres across the world within a period of 5 years (2013–2018). Eligible participants are persons ≥18 years of age with a diagnosis of SSc. In addition to baseline medical data, participants will complete patient-reported outcome measures every 3 months. Upon enrolment in the cohort, patients will consent to be contacted in the future to participate in intervention research and to allow their data to be used for comparison purposes for interventions tested with other cohort participants. Once interventions are developed, patients from the cohort will be randomly selected and offered interventions as part of pragmatic RCTs. Outcomes from patients offered interventions will be compared with outcomes from trial-eligible patients who are not offered the interventions.Ethics and disseminationThe use of the cmRCT design, the development of self-guided online interventions and partnerships with patient organisations will allow SPIN to develop, rigourously test and effectively disseminate psychosocial and rehabilitation interventions for people with SSc.

BackgroundShort-term medical missions (STMMs) represent a grass-roots form of aid, transferring medical services rather than funds or equipment. The objective of this paper is to review empirical studies on social, economic and diplomatic aspects of STMMs.MethodsA systematic literature review was conducted by searching PubMed and EBSCOhost for articles published from 1947–2014 about medical missions to lower and middle income countries (LMICs). Publications focused on military, disaster and dental service trips were excluded. A data extraction process was used to identify publications relevant to our objective stated above.ResultsPubMed and EBSCOhost searches provided 4138 and 3262 articles respectively for review. Most articles that provide useful information have appeared in the current millennium and are found in focused surgical journals. Little attention is paid to aspects of volunteerism, altruism and philanthropy related to STMM activity in the literature reviewed (1 article). Evidence of professionalization remains scarce, although elements including guidelines and tactical instructions have been emerging (27 articles). Information on costs (10 articles) and commentary on the relevance of market forces (1 article) are limited. Analyses of spill-over effects, i.e., changing attitudes of physicians or their communities towards aid, and characterizations of STMMs as meaningful foreign aid or strategic diplomacy are few (4 articles).ConclusionsThe literature on key social, economic and diplomatic aspects of STMMs and their consequences is sparse. Guidelines, tactical instructions and attempts at outcome measures are emerging that may better professionalize the otherwise unregulated activity. A broader discussion of these key aspects may lead to improved accountability and intercultural professionalism to accompany medical professionalism in STMM activity.Electronic supplementary materialThe online version of this article (doi:10.1186/s12913-015-0980-3) contains supplementary material, which is available to authorized users.

Objective. Few studies exist on sexual activity and functioning in female patients with systemic sclerosis (SSc, scleroderma). We studied the patientreported impact of SSc on sexual functioning among female patients. Methods. 101 SSc patients completed the Short Form-36 (SF-36), the Female Sexual Functioning Index (FSFI) and the Female Sexual Function in Scleroderma (FSFS) questionnaires. Results. Sixty patients reported being sexually active (59.4%). Reasons for sexual inactivity included lack of a partner (36.6%), personal choice (31.7%), and health status of the respondent's partner (19.5%). Only 7 subjects (17%) listed scleroderma as the primary reason for sexual inactivity. The mean FSFI score in the sexually active population was 24.9 (SD=6.7, range = 4.5-34.8) which is significantly lower than the mean score of 30.5 reported for the general population. Sexual functioning was significantly correlated with the Mental Component Score of the SF-36 (r=0.54, p<0.001) but surprisingly not with the Physical Component Score of the SF-36, age, and disease classification or duration. Several scleroderma-related problems including fatigue, body pain, vaginal dryness, and vaginal discomfort were cited as contributing to sexual difficulties. Conclusion. Women with scleroderma do remain sexually active overall in spite of several disease-related physical and psychological difficulties. Many of their problems are amenable to health interventions and should be addressed during health care visits.