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Juvenile-onset systemic sclerosis (jSSc) is a rare condition, having unique characteristic features compared to adult-onset SSc. Although cardiac involvement (CI) is known as a leading cause of mortality overall in SSc, the importance of CI in jSSc has not been emphasized. Here we present a 13-year-old female with jSSc overlapped with dermatomyositis (DM) complicated CI. She developed skin thickness and induration, Raynaud’s phenomenon, digital pitting scars in fingertips, and skeletal myositis. Oral prednisolone and pulse methotrexate treatment led to the improvement of skin findings; however two weeks after the initiation she suddenly presented with muscle pain and dyspnea within a few days. Cardiac investigations then showed pericardiac effusion and diastolic dysfunction due to significant biventricular hypertrophy causing heart failure. As pericardiac effusion and exacerbation of skeletal myositis were evident, steroid pulse therapy was initiated. Unexpectedly, not only the myositis but also the CI including diastolic dysfunction was improved. She thereafter followed a favorable clinical course without reactivation of the CI or cardiac fibrosis. As a conclusion, close attention to CI must be paid in jSSc patients, especially when skeletal muscle involvement is evident and immunosuppressive therapy may be effective for CI in jSSc in cases where it occurs abruptly.

Additional file 2. Supplemental figures for the pain intensity data (residual plots, Pâ P plots and Cookâ s distance against leverage/(1-leverage)).

Background and Aims: With the recent development of capecitabine and oxaliplatin (XELOX) therapy, implantation of a Central Venous (CV) port can be now avoided. However, vascular pain occasionally requires switching of the drip infusion route. Some investigators reported that addition of steroids to oxaliplatin drip infusion is useful in controlling vascular pain. However, the pharmacological use of steroids can make oxaliplatin unstable due to the elevation of pH; further, the effectiveness of steroid in this therapy is unknown. This study was undertaken to evaluate the effectiveness of dexamethasone (DEX) for controlling vascular pain caused by the administration of oxaliplatin via the peripheral vein. Study design: Retrospective study. Place and duration of the Study: Department of Gastroenterological Surgery, Fukuoka University Faculty of Medicine, between April 2010 and November 2011. Methodology: The study included 69 patients who received XELOX + bevacizumab therapy for advanced or recurrent colorectal cancer. In all the patients, oxaliplatin (130 mg/m2) was administered in combination with DEX (6.6 mg) via the peripheral vein. Results: Vascular pain developed in 47 patients (68.1%), but it was transient. No patients required CV port implantation. Grade 3 or higher hemotoxicity was noted in 14.5% of the patients, and grade 3 or higher nonhematological toxicity was noted in 20.3% of the patients. The response rate was 59.4%. One patient experienced hypersensitivity reactions to oxaliplatin. Conclusions: The recorded response rate combined with the use of DEX suggests that DEX probably does not exert adverse effects on the therapy, ie, it does not affect the stability of oxaliplatin by elevating the pH. DEX may be useful not only for controlling vascular pain caused by the administration of oxaliplatin via the peripheral vein but also for controlling oxaliplatin-induced hypersensitivity reactions.