Introduction: Reduced TAPSE/sPAP ratio has recently emerged as a predictive parameter risk factor for PH, however its role in SSc has been poorly investigated. The aim of the study was to investigate the prognostic value of the TAPSE/sPAP ratio for the prediction of mortality and cardiovascular events in patients with SSc complicated by PH. A comparison between SSc patients with PAH (SSc-PAH) and those with PH and significant ILD (SSc-PH) was also carried out.Materials and methods: A retrospective single-center study in which all patients having SSc—complicated by PH—referring to the Scleroderma-Unit of the AOU Policlinico of Modena, from October 2013 to October 2023 were evaluated. All SSc patients underwent recurrent clinical examination, routine blood chemistry analysis, functional, instrumental evaluation.Results: 61 SSc patients (F/M 52/9) were enrolled. During the follow-up, 60.1% of patients experienced at least one cardiovascular event and 62% died. The main causes of death were PH (39.4%) and other heart-related events (39.4%). The TAPSE/sPAP ratio was significantly lower in deceased patients compared to survivors (mm/mmHg 0.3 ± 0.12SD vs. 0.48 ± 0.17SD, p < 0.001). Compared to the SSc-PAH subgroup, the SSc-PH patients had lower survival rates (55.3 ± 31.2 SD months vs. 25 ± 19 SD, p = 0,05). At the multivariate analysis, TAPSE/sPAP ratio <0.32 mm/mmHg, male gender, and the presence of significant ILD were identified as independent predictors of mortality and cardiovascular events.Conclusion: Our work confirmed the predictive role of the TAPSE/sPAP ratio for mortality and cardiovascular events in patients with SSc complicated by PH.

Introduction Skin ulcers (SU) represent one of the most frequent manifestations of systemic sclerosis (SSc), occurring in almost 50% of scleroderma patients. SSc-SU are often particularly difficult to treat with conventional systemic and local therapies. In this study, a preliminary evaluation of the role and effectiveness of blue light photobiomodulation (PBM) therapy with EmoLED^® in the treatment of scleroderma skin ulcers (SSc-SU) was performed. Methods We retrospectively analyzed 12 consecutive SSc patients with a total of 15 SU on finger hands. All patients were treated with adequate systemic therapy and local treatment for SU; after a standard skin ulcer bed preparation with debridement of all lesions, EmoLED^® was performed. All patients were locally treated every week during 2 months of follow-up; SU data were collected after 4 weeks (T4) and 8 weeks (T8). Eight SSc patients with comparable SU were also evaluated as controls. Results The application of EmoLED^® in addition to debridement apparently produced faster healing of SU. Complete healing of SU was recorded in 41.6% cases during EmoLED^® treatment. Significant improvements in SU area, length, and width, wound bed, and related pain were observed in EmoLED^® patients from T0 to T8. Control subjects treated with standard systemic/local therapies merely showed an amelioration of SU area and width at the end of the follow-up. No procedural or post-procedural adverse events were reported. Conclusions The positive clinical results and the absence of side effects suggest that EmoLED^® could be a promising tool in the management of SSc-SU, with an interesting role to play in the healing process in addition to conventional systemic and local treatments.

(1) Background: Assessment of right ventricular (RV) function has become increasingly important in echocardiography due to its prognostic significance in cardiac and systemic diseases. (2) Objectives: Tricuspid Annular Plane Systolic Excursion (TAPSE) and Tissue Doppler Imaging (TDI S′) are commonly used methods to assess RV function. The aim of this study was to compare the reproducibility of TAPSE and TDI S′ among experienced operators. (3) Methods: A study was conducted on 100 consecutive patients with systemic sclerosis undergoing echocardiography evaluation screening for pulmonary hypertension. Standardized echocardiography images were acquired using a Philips EPIQ system (Philips Healthcare, Amsterdam, The Netherlands), measuring TAPSE and TDI S′. Five operators independently analyzed the images, with each blinded to the results. Exclusion criteria included pathological TAPSE or TDI S′ values, as well as indirect echocardiography signs of pulmonary hypertension. (4) Results: The study’s results indicate that there was superior inter-operator agreement for TDI S′ (ICC = 0.891) compared to TAPSE (ICC = 0.47). The mean TAPSE measurements ranged from 23.49 mm to 23.99 mm, while TDI S′ values ranged from 12.16 cm/s to 12.35 cm/s, with TDI S′ showing narrower confidence intervals. (5) Conclusions: Echocardiographic assessment of right ventricular (RV) function is crucial in clinical practice. Tissue Doppler imaging-derived systolic velocity (TDI S′) demonstrated superior reproducibility compared to tricuspid annular plane systolic excursion (TAPSE) in evaluating RV function. This enhanced reproducibility supports the routine incorporation of TDI S′ as a highly reproducible parameter within a multiparametric assessment framework.

Objective: COVID-19 pandemic represents a serious health emergency that severely compromised our Public Health system, resulting in a rapid and forced reorganization and involved the management of chronic diseases too. The Scleroderma Unit of Modena and Reggio Emilia follows more than 600 patients suffering from systemic sclerosis (SSc) and recently became the referral center (HUB) in Emilia-Romagna for this rare connective tissue disease. The aim of the present study was to evaluate the extent by which the lockdown and the pandemic has impacted the activity of admissions to Scleroderma Unit of Modena and Reggio Emilia. Methods: Our daily clinical activity is characterized by outpatient visits, videocapillaroscopy exam, ulcers treatment, therapeutic infusions in day hospital regimen, multidisciplinary visits following our dedicated SSc care pathway, and clinical trials. Our activity has been quickly rescheduled to ensure the proper assistance to our SSc patients during the COVID-19 pressure. Results: The use of telemedicine has certainly assured a robust continuity of health care. Furthermore, telephone pre-triage, nurse/medical triage, proper physical distancing and use of PPE/DPI allowed us to re-organize and continue SSc daily activity. Specifically, therapeutic infusions in day hospital regimen and outpatient visits, including ulcers treatment, was guaranteed and maximized. Conclusion: The management of scleroderma patients by an expert specialist reference center is crucial in order to ensure continuity of care and pursue the best SSc practice.

Objectives: MMF is a mainstay for the treatment of SSc. The occurrence and implications of MMF-related adverse events (AEs) on drug retention rates in real life remain poorly defined. We aimed to determine the MMF retention rate and to investigate the causes and patterns of discontinuation, AEs and treatment options used after discontinuation. Methods: SSc patients who started MMF treatment underwent a retrospective longitudinal assessment for up to 5 years. We documented the incidence, predictors and impacts of MMF treatment on gastrointestinal intolerance, infections, laboratory abnormalities and cancer. Rescue strategies implemented after MMF discontinuation were recorded. Results: The 5-year MMF retention rate of 554 patients stood at 70.7%, and 19.6% of them stopped MMF due to AEs. One out of every four patients experienced a dose reduction or discontinuation of MMF due to AEs, with gastrointestinal intolerance being the predominant cause. The 5-year cumulative incidence rates for gastrointestinal intolerance, cancer, severe infections and laboratory toxicity leading to MMF discontinuation were 6.4%, 4.1%, 3.1% and 2.1%, respectively. Lower respiratory tract was the most affected, with bacteria being the predominant causative agent. Intestinal and pulmonary circulation involvement were tied to elevated AE rates and MMF discontinuation. The most common approaches post-MMF cessation were ‘watch and wait’ and switch to rituximab. Conclusions: : MMF use in SSc appears to be limited by the occurrence of AEs, both in terms of persistence and dosing of the drug. Rescue options after MMF discontinuation are limited and many patients remain without immunosuppressant.

Systemic sclerosis is a progressive fibrotic autoimmune disease which mainly affects young women causing fibrosis of the skin and internal organs. In systemic sclerosis patients, facial and hand alterations are significant as these may have an impact on body image. In fact, systemic sclerosis could cause disfigurement of the most visible parts of the body, hindering self-confidence and quality of life. Therefore, the assessment of body image in systemic sclerosis is crucial in order to optimize a tailored care of the patient. In the near future, the creation of a pathway involving dedicated specialists (psychologists, physiotherapists, dermatologists, and plastic surgeons) should become an integral part of the disease management.