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This article discusses points to consider when undertaking a clinical trial to test therapy for renal involvement in SSc, not including scleroderma renal crisis. Double-blind, randomized controlled trials vs placebo or standard background therapy should be strongly considered. Inclusion criteria should consider a prespecified range of renal functions or stratification of renal function. Gender and age limitations are probably not necessary. Concomitant medications including vasodilators, immunosuppressants and endothelin receptor antagonists and confounding illnesses such as diabetes, kidney stones, hypertension and heart failure need to be considered. A measure of renal function should be strongly considered, while time to dialysis, mortality, prevention of scleroderma renal crisis and progression of renal disease can also be considered, although they remain to be validated. Detailed, pre-planned analysis should be strongly considered and should include accounting for missing data. [ABSTRACT FROM AUTHOR]

This article discusses points to consider when undertaking a clinical trial to test therapy for skin ulcers in SSc. A validated definition of skin ulcers should be used if available. Defining a uniform SSc patient population, including consideration of disease duration, history of digital ulcers and capillaroscopic patterns, is important. Excluding confounding factors such as infection, calcinosis and trauma should be strongly considered, or at least accounted for, in defining patients. Outcome measures such as time to healing, prevention of new ulcers, function, pain and objective measures such as US, laser Doppler and thermography can be considered as outcome measures, although their validation has not yet been achieved and efforts may be needed to validate them before use. Likewise, biomarkers should be considered or consideration should be given to storing serum, plasma or cells for possible future analysis. A pre-planned analysis is important and should include consideration of missing data. [ABSTRACT FROM AUTHOR]

SSc is a multisystem disease characterized by an unpredictable course, high mortality and resistance to therapy. The complexity and severity of SSc is a growing burden on the health-care systems. As a result, researchers are seeking new therapeutic strategies for effectively managing these patients. Disease registries are used to support care management efforts for groups of patients with chronic diseases and are meaningful to capture and track key patient information to assist the physicians in managing patients. For these reasons, SSc surveys, research associations and consortiums are pivotal to conduct ongoing research and data collection to enhance disease knowledge and support research projects. Currently, there are several national SSc registries in the UK, Germany, USA, Canada, Brazil and Australia. There is also an international registry established by the European League Against Rheumatism scleroderma trial and research (EUSTAR) called minimal essential data set (MEDS) Online, which collects data from over 8000 patients from 92 centres worldwide, including 21 European centres and 9 centres outside Europe. By collecting, analysing and disseminating data on disease progression and patient responses to long-term disease management strategies, registries help to improve understanding of the disease and keep medical professionals up to date on the latest advances. [ABSTRACT FROM AUTHOR]

Objective. To evaluate in SSc, the frequency of digital lesions and the morphology, characteristics, natural course and time to healing of 1614 digital ulcers (DUs). [ABSTRACT FROM PUBLISHER]

Digital ulcers are a very frequent complication of systemic sclerosis affecting about half of the SSc patients, and about 75% of the affected patients have their first DU episode within 5 years from their first non-Raynaud symptom. The lack of adequate classification criteria as well as the lack of knowledge of the development of DU have contributed to the opening of specific registries to better understand the natural history of these lesions. For these reason, specific disease registries play a fundamental role in this field of research. Thanks to the systematic collection of data and their subsequent analysis and comparison between different cohorts, it is possible to improve understanding of the underlying trigger mechanisms of DU development and to determine temporal trends. In the future, the development of recommendations for the management of DU remains of pivotal importance to prevent DU development and obtain rapid healing as well as reduction of pain and disability. [ABSTRACT FROM AUTHOR]

Rehabilitation may contribute to the management of systemic sclerosis (SSc) dealing with disabilities due to hand involvement. The aim of this study is to evaluate the efficacy of a rehabilitation programme based on the combination of connective tissue massage and Mc Mennell joint manipulation specifically conceived for SSc patients’ hands. Forty SSc patients were enrolled: 20 (interventional group) were treated for a 9-week period (twice a week, 1 h per session) with a combination of connective tissue massage, Mc Mennell joint manipulation and home exercise programme, and 20 (control group) were assigned only to home exercise programme. Patients of both groups were assessed at baseline (T0), after 9 week (T1) and at a 9 weeks follow-up (T2). They were evaluated for quality of life by SF-36 and Health Assessment Questionnaire (HAQ), hands involvement by Hand Mobility in Scleroderma (HAMIS) test, Cochin hand functional disability scale and the measurements of ROM. In the interventional group, fist closure, HAMIS test and Cochin hand functional disability scale improved at the end of the treatment ( p < 0.0001) as well as HAQ, Physical Synthetic Index (PSI) and Mental Synthetic Index (MSI) of SF-36 scores (HAQ and PSI, p < 0.0001; MSI, p < 0.001). In the control group, the programme of home daily exercises improved only fist closure at the end of the treatment ( p < 0.0001). The combination of connective tissue massage, Mc Mennell joint manipulation and home exercise programme is effective in the rehabilitative treatment of SSc hands. This combined treatment may lead to an improvement of hand function and quality of life. [ABSTRACT FROM AUTHOR]

Abstract: Systemic sclerosis (SSc) is characterized by wide-spread fibrosis, activation of immune system with production of autoantibodies and extensive vascular damage. Raynaud''s phenomenon (RP) and digital ulcers (DU) represent two faces of the same coin in SSc vasculopathy. RP, the earliest manifestation of the vascular involvement, is due to an excessive vasospasm of digital arteries, precapillary arterioles and cutaneous arteriovenous shunts, usually in response to cold exposure or other stimuli. DU are a severe complication of microvessel involvement and also of the persistent vasospasm of RP. Thus, the management of RP and DU requires a multimodal approach using a combination of pharmacological, non-pharmacological, and surgical treatments. Currently, the treatment of these complications represents a great challenge for all physicians. [Copyright &y& Elsevier]