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.
In 2019, the novel SARS-CoV-2 infection emerged, causing the disease called COVID-19, which primarily affects the respiratory tract and lung at alveolar and interstitial levels. Systemic sclerosis (SSc) is an autoimmune connective disease characterized by vascular abnormalities and diffuse and progressive fibrosis of the skin and internal organs. Raynaud phenomenon (RP) occurs in virtually all patients affected by SSc and, in most cases, is an onset symptom of the disease; that is, RP may appear several years before overt illness. Although the exact pathophysiologic pathways leading to RP and SSc are still unknown, several infectious agents, especially viruses, have been suggested as possible triggering factors. Here, the authors describe the first case of RP secondary to SSc following SARS-CoV-2 infection.
Here we describe the case of a 60-year-old-woman with systemic sclerosis sent to our Scleroderma Unit to treat digital stumps. The stumps were successfully treated with autologous fat grafting (crown-shape infiltration). Our technique of autologous lipotransfer improved wound healing in a scleroderma patient with stump-digital ulcers where all other options failed.
OBJECTIVE To explore the effect of topical cannabidiol (CBD) in treating digital ulcers in patients with systemic sclerosis (SSc). METHODS In total, 45 patients with SSc who had digital ulcers were consecutively enrolled between January 2019 and December 2019. Of the participants, 25 were treated with CBD during surgical debridement and 20 were treated with standard local therapy. A numeric rating scale for pain and Health Assessment Questionnaire Disability Index were administered at the baseline and at the end of treatment. RESULTS Local treatment with CBD was significantly associated with lower pain scores, higher health assessment scores, and an increase in participants’ total hours of sleep. Patients in the control group more frequently required additional analgesic therapy. CONCLUSIONS Topical CBD may be a valuable tool to treat pain related to digital ulcers in patients with SSc.
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.
Background:Systemic Sclerosis-SSc is an autoimmune disease, characterized by fibrosis due to immune-mediated microangiopathy. Digital ulcers-DUs represent frequent complications, they are recurrent, painful and often resistant to traditional treatments. Standard therapy, in particular oral opioids, is often inadequate or limited by side effect. Cannabidiol-CBD, is the major non-psychotropic component of the Cannabis sativa, recent studies on its effectiveness as an anxiolytic, anti-inflammatory, and antipsychotic drug showed promising results, in the setting of chronic pain too.Objectives:We evaluated the efficacy of CBD drop in pain management in a cohort of SSc patients using standard rating scale VAS, PSQI and HAQ. We further assessed the safety profile and the potential use as opioid-sparing.Methods:From January to November 2019 we consecutively enrolled 31 SSc patients (F/M 26/5, mean age 53.0±14.6SD-years) referred to our Scleroderma Unit. All patients satisfied the EULAR/ACR SSc classification criteria. All cases were complicated by painful DUs resistant to analgesics and pain was classified as severe, according to WHO guidelines. CBD drops consist of cannabis sativa seed in olive oil, 10% CBD, laboratory tested to confirm a tetrahydrocannabinol-THC level<0.3%. The CBD oil was administrated sublingually twice-a-day. All patients started with CBD 3 drops twice-a-day, and progressively increased to the maximum dosage of 6 drops twice-a-day (from 27.6 g to 55.2 g dose/day). All patients continued local/systemic treatments for SSc: 24/31 subjects performed calcium-channel blockers, 31/31 prostanoids infusion, 24/31 anti-endothelin drugs. All subjects were provided with a daily diary to record self-evaluation of pain using VAS, PSQI, hours of sleep per night, use of other analgesics, eventual side effects. HAQ-DI was also administrated. These indicators were assessed baseline and during follow-up. Safety of CBD was evaluated by patient’s records of side effects. All data were analyzed by paired t-test. This investigation was a monocentric, prospective study. Ethical approval was obtained from the Competent Ethics Committee (protocol n. 282/15) and all participants gave written consent.Results:CBD was administered for a mean period of 5.9±3.2SD-months. After the first month, VAS decreased from 94.80±8.72SD to 54.70±9.40SD (p<0.0001), PSQI decreased from 9.27±2.9SD to 4.47±1.06SD (p<0.001), total hours of sleep increased from 2.56±1.28 SD to 5.67±0.85SD (p< 0.0001). The additional analgesic therapy was necessary in 22/31 patients: 6/22 only paracetamol, 12/22 paracetamol+oxycodone reducing the dosage of oxycodone at the minimum, 2/22 oxycodone 20 mg twice-a-day, 2/22 need fentanyl transdermal patch. After 3 months, VAS further reduced to 40.90±12.90, PSQI decreased to 3.1±1.4SD, the mean total hours of sleep per night was 6.10±0.79SD and the HAQ-DI decreased from 2.19±0.67SD (baseline) to 0.79±0.46SD at the last patients’ evaluation. At the end of the observation, 18/31 patients (58%) showed DUs healing. We also interestingly reported improvement of dysphagia and appetite in 70%, and an improvement in constipation related to opiods in 48%. No patients experienced severe side effects in particular no psychoactive aspects. Mild side effects, namely dry mouth was referred by 9/31 (29%), mild abdominal pain and changes in appetite by 10/31 (32%). No interaction with other drugs was observed.Conclusion:Our study suggests that oral CBD is effective and safe in maintaining analgesia in SSc patients with DUs. Furthermore, CBD could be helpful in opioids tapering and to treat dysphagia, even if these observations need focused investigations. In conclusion, CBD might be a useful tool to manage chronic pain in SSc-DUs. These data provide a compelling rationale for further research to clarify the therapeutic potential of CBD in SSc.Disclosure of Interests:None declared
Several authors reported an increased risk of cancer in SSc patients, including breast cancer (BC). Nevertheless, the mechanisms underlying this association have not yet been clarified. SSc and BC share several molecular pathways, which seem to play a common etiopathogenetic role. The previously published Sclero-Breast study demonstrated the development of BC with a good prognosis among these patients, which could be explained by an autoimmune background as a possible mechanism for limiting tumor extension. Here, we report the results of an IHC analysis of molecular pathways known to be common drivers for both diseases, with the aim to better define the mechanisms underlying a good prognosis of BC in patients affected by SSc. The analysis demonstrated higher TILs rates in all BC subgroups, with a high rate of PD-L1 expression especially in TNBC and HER2-positive BC, suggesting a less aggressive behavior in these patients compared to the general population. These results support a possible de-escalation strategy of cancer therapies in these fragile patients. These data could represent a starting point for future prospective studies based on the clinical application of these biomarkers with a larger sample size to promote a personalized and targeted oncological treatment for this specific subset of patients.
Systemic sclerosis (SSc) is a connective tissue disease characterized by immune-system alterations, fibrosis involving the skin and internal organs and diffuse microangiopathy. Pulmonary arterial hypertension (PAH) is a severe complication of SSc affecting about 10–15% of the patients and it is a leading cause of mortality. Due to the devastating nature of SSc-PAH, there is a clear need to systematically adopt appropriate screening programs. Nail fold videocapillaroscopy (NVC) studies have shown a more severe peripheral microvascular dysfunction in SSc patients with PAH suggesting that abnormalities in peripheral microcirculation may correlate with pulmonary microangiopathy. This is a cross-sectional study involving four tertiary University Rheumatology Units in the Center-North of Italy. Seventy patients, 35 adults with SSc and PAH confirmed by RHC (F/M 34/1; median age 65.2 ± 8.9 SD yrs), and 35 SSc patients without PAH were enrolled (F/M 3471; median age 63.3 ± 10.3 SD yrs). Clinical, laboratoristic and instrumental data were collected and NVC was performed in all patient. Specific NVC parameters were evaluated and a semi-quantitative rating scale was adopted to score these changes. Finally, patients were distributed into the suitable NVC pattern belonging to the scleroderma pattern. Our aim was to compare the peripheral microangiopathy changes in SSc patients with and without PAH, and to investigate the relationship between NVC findings and the main hemodynamic parameters of pulmonary vasculopathy. Patients with SSc-PAH+ showed a significant higher frequency of interstitial lung disease (ILD). No significant differences regarding clinical and laboratoristic parameters were observed. NVC abnormalities, avascular areas were more frequent in SSc patients with PAH, respect to those without (p = 0.03), and capillary density was significantly lower when considering grade 3 (p = 0.02). A higher NVC semiquantitative mean was found in SSc-PAH+ patients and a greater rate of the “late” pattern was detected in SSc-PAH+ subjects in respect to PAH- (57.1% vs. 25.7%) (p = 0.03). A significant correlations between pulmonary pressure values (sPAP by TTE and mPAP by RHC) and the capillary density (Spearman's rho 0.35, p = 0.04 for both). Our findings provide additional evidence to the literature data, confirming that a higher degree of peripheral nailfold microangiopathy is more common in SSc-PAH patients, and further strengthening the concept that NVC changes may run parallel with similar abnormalities inside pulmonary microcirculation.
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