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Objective: Since of the last publication of last recommendations on primary large-vessel vasculitis (LVV) endorsed by the Italian Society of Rheumatology (SIR) in 2012, new evidence emerged regarding the diagnosis and the treatment with conventional and biologic immunosuppressive drugs. The associated potential change of clinical care supported the need to update the original recommendations. Methods: Using the grading of recommendations assessment, development and evaluation (GRADE)-ADOLOPMENT framework, a systematic literature review was performed to update the evidence supporting the European Alliance of Associations for Rheumatology (EULAR) guidelines on LVV as reference. A multidisciplinary panel of 12 expert clinicians, a trained nurse, and a patients’ representative discussed the recommendation in cooperation with an Evidence Review Team. Sixty-one stakeholders were consulted to externally review and rate the recommendations. Results: Twelve recommendations were formulated. A suspected diagnosis of LVV should be confirmed by imaging or histology. In active GCA or TAK, the prompt commencement of high dose of oral glucocorticoids (40-60 mg prednisone-equivalent per day) is strongly recommended to induce clinical remission. In selected patients with GCA (e.g., refractory or relapsing disease or patients at risk of glucocorticoid related adverse effects) the use of an adjunctive therapy (tocilizumab or methotrexate) is recommended. In all patients diagnosed with TAK, adjunctive therapies, such as conventional synthetic or biological immunosuppressants, should be given in combination with glucocorticoids. Conclusions: The new set of SIR recommendations was formulated in order to provide a guidance on both diagnosis and treatment of patients suspected of or with a definite diagnosis of LVV.

Objectives: In systemic sclerosis (SSc), gastrointestinal involvement is one of the earliest events. We compared the gut microbiota (GM), its short-chain fatty acids (SCFAs) and host-derived free fatty acids (FFAs) in patients with very early diagnosis of SSc (VEDOSS) and definite SSc., Methods: Stool samples of 26 patients with SSc, 18 patients with VEDOSS and 20 healthy controls (HC) were collected. The GM was assessed through 16S rRNA sequencing, while SCFAs and FFAs were assessed by gas chromatography-mass spectrometry., Results: In patients with VEDOSS, an increase in Bacteroidales and Oscillospirales orders and a decrease in Bacilli class, Blautia, Romboutsia, Streptococcus and Turicibacter genera was detected in comparison with HC. In patients with SSc, an elevated number of Acidaminococcaceae and Sutterellaceae families, along with a decrease of the Peptostreptococcaceae family and Anaerostipes, Blautia, Romboutsia and Turicibacter genera was found in comparison with HC. Patients with SSc and VEDOSS had a significantly lower butyrate and higher acetate with respect to HC. In VEDOSS, an increase in Oscillospiraceae family and Anaerostipes genus, and a decrease in Alphaproteobacteria class, and Lactobacillales order was identified with respect to SSc. Moreover, patients with VEDOSS exhibited higher acetate and lower valerate compared with definite SSc., Conclusion: A GM dysbiosis with depletion of beneficial anti-inflammatory bacteria (especially butyrate-producing) and a significant decrease in faecal butyrate was identified in patients with VEDOSS. This early GM imbalance may foster the growth of inflammatory microbes, worsening intestinal dysbiosis and inflammation in early SSc stages. The potential butyrate administration in early disease phases might be considered as a novel therapeutic approach to mitigate gastrointestinal discomfort and progression preserving patient's quality of life., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Introduction: The recovery room (RR) is a hospital area where patients are monitored in the early postoperative period before being transferred to the surgical ward or other specialized units. The utilization of scores in the RR context facilitates the assignment of patients to the appropriate ward and directs necessary monitoring. Some scoring systems allow nurses to select patients who can be discharged directly to their homes., Aim and Methods: The aim of this narrative review was to describe and compare the scoring systems employed to discharge postoperative patients from RR, with a focus on item characteristics., Results: Nine scoring systems were identified and discussed: the "Aldrete Score System" and its modified version, the "Respiration, Energy, Alertness, Circulation, Temperature Score", the "Post Anesthetic Discharge Scoring System", the "White and Song Score", the "Readiness for Discharge Assessment Tool", the "Anesthesia and Perioperative Medicine Service Checklist", the "Post-Anesthetic Care Tool", the "Post-operative Quality Recovery Scale", and the "Discerning Post Anesthesia Readiness for Transition" instrument., Discussion and Conclusions: To obtain a comprehensive overview, the items included in the scoring systems were compared. Despite the availability of guidelines for patients' discharge readiness from the RR, there is no universally recommended scoring system. Next-generation scores must be improved to ease their use, minimize errors, and increase safety. The main goals of the scores included in this narrative review were to be simple to use, feasible, intuitive, comprehensive, and flexible. However, these goals frequently conflict because patient assessment takes time, and a smart and comprehensive score may not consider some clinical parameters that may be crucial for the discharge decision. Therefore, further research should be conducted on this topic.

Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Objective: Proton pump inhibitors (PPIs) are widely prescribed to treat gastroesophageal reflux disease (GERD) in Systemic Sclerosis (SSc). However, not all patients adequately respond to the treatment, and there are frequent concerns about the safety of long-term use of PPIs. Our aim was to identify the main problems/complaints of SSc patients on PPIs, as well as understand their unmet needs., Methods: SSc patients treated with PPIs were invited through international patient associations and social media to participate in an online survey., Results: We gathered 301 valid responses from 14 countries (United Kingdom 19.3% and United States 70.4%). Multiple PPIs use (two: 30% and three: 21% in series) was common. The majority (89%) reported improvement in gastrointestinal symptoms from receiving PPIs. Side effects attributed to receiving PPIs were uncommon (19%); however, most (79%) were potentially concerned. Around half (58%) had received lifestyle information, and most (85%) had searched online for information about PPIs. Only in the minority (12%) had a surgical approach been discussed; however, half (46%) indicated that they would be willing to undergo surgery to resolve their GERD symptoms but had important concerns., Conclusion: Despite the frequent use of PPIs in patients with SSc, there is significant heterogeneity in prescription, and combination therapy (PPIs plus other medication for acid reflux) is not uncommon (approximately 40%). Patients have significant concerns about PPIs side effects. Education about PPIs is often neglected, and patients very frequently use online sources to obtain information on drug treatment. A surgical approach is infrequently discussed, and patients fear this potential therapeutic approach., (© 2023 American College of Rheumatology.)

Self-care plays a critical role in symptom recognition, management, and risk factor modification for patients with chronic illnesses. Despite its significance, self-care levels in this population are generally poor. Health literacy (HL) is pivotal for promoting effective self-care, yet the association across specific chronic illnesses remains fragmented and conflicting. Therefore, a systematic review and meta-analysis will be conducted. Inclusion criteria encompass quantitative studies involving adult patients with at least one chronic illness reporting on the association between a measure of HL and one or more elements of self-care behaviors as outcomes. Databases to be searched include PubMed, CINAHL, APA PsycINFO, Embase, Web of Science, and Cochrane Central Register of Controlled Trials. The studies will undergo risk of bias and certainty of evidence assessment using ROBINS-E and GRADE. Extracted data will include authors, publication date, aim(s), study location, design, sample characteristics, chronic illness type, study length, HL, and self-care measures. Understanding the link between HL and self-care can aid healthcare providers in implementing strategies to enhance health-promoting behaviors, contributing valuable insights to the scientific community and fostering nuanced discussions. This protocol ensures methodological transparency, stimulates discourse, and paves the way for informed interventions to improve overall health outcomes.

Rationale and Aim: Health literacy (HL) is pivotal for the successful self-management of chronic diseases. Little HL information is currently available in SSc patients; therefore, the present study aims at evaluating the HL levels in an Italian cohort of SSc patients., Methods: SSc patients were enrolled with the support of Italian patient associations, from September 2022 to March 2023. Health literacy characteristics were derived from the Health Literacy Scale European Questionnaire-16 (HLS-EU-Q16), consisting of 16 items designed on a four-point Likert scale ranging from "very difficult" to "very easy", and three HL levels were identified: inadequate HL (0-8 score); problematic HL (9-12 score); and sufficient HL (13-16 score)., Results: Enrolled patients (n = 57, mean age = 59 years, SD = 13.2) were mostly female (98.2%), partnered (73.7%), and unemployed or retired (67.9%). Almost half of SSc patients were diagnosed more than 10 years ago, with first symptoms appearing on average 19 years ago (SD 10.5). In 63% of the participants, the overall health literacy skills were inadequate, or problematic, especially in the health care and disease prevention domains. Indeed, 49.2% of the patients declared difficulty in finding information on treatments for illnesses and where to get professional help (42.1%), 47.6% found difficulty in retrieving information on how to manage mental health problems, and 40.4% declared difficulties in judging whether the information on health risks in the media was reliable., Conclusions: Our findings show that SSc patients have inadequate or problematic levels of HL, suggesting the need for periodic screenings to uncover poor health literacy skills and to provide tailored and understandable educational material. This study was not registered.

Objectives: We characterized the microbiota in SSc, focusing on the skin-oral-gut axis and the serum and faecal free fatty acid (FFA) profile., Methods: Twenty-five SSc patients with ACA or anti-Scl70 autoantibodies were enrolled. The microbiota of faecal, saliva and superficial epidermal samples was assessed through next-generation sequencing analysis. GC-MS was used to quantify faecal and serum FFAs. Gastrointestinal symptoms were investigated with the University of California Los Angeles Scleroderma Clinical Trial Consortium Gastrointestinal Tract Instrument (UCLA GIT-2.0) questionnaire., Results: The ACA+ and anti-Scl70+ groups displayed different cutaneous and faecal microbiota profiles. The classes of cutaneous Sphingobacteriia and Alphaproteobacteria, the faecal phylum Lentisphaerae, the levels of the classes Lentisphaeria and Opitutae, and the genus NA-Acidaminococcaceae were significantly higher in faecal samples from the ACA+ patients than in samples from the anti-Scl70+ patients. The cutaneous Sphingobacteria and the faecal Lentisphaerae were significantly correlated (rho = 0.42; P = 0.03). A significant increase in faecal propionic acid was observed in ACA+ patients. Moreover, all levels of faecal medium-chain FFAs and hexanoic acids were significantly higher in the ACA+ group than in the anti-Scl70+ group (P < 0.05 and P < 0.001, respectively). In the ACA+ group, the analysis of the serum FFA levels showed an increasing trend in valeric acid., Conclusion: Different microbiota signatures and FFA profiles were found for the two groups of patients. Despite being in different body districts, the cutaneous Sphingobacteria and faecal Lentisphaerae appear interdependent., (© The Author(s) 2023. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Introduction: The COVID-19 pandemic has had a considerable impact on the psychological and psychopathological status of the population and health care workers in terms of insomnia, anxiety, depression, and post-traumatic stress disorder. The primary aim of this study was to describe and evaluate the impact of the pandemic on insomnia levels of a cohort of Italian nurses, particularly those involved in the care of COVID-19 patients. The secondary aim was to identify the interaction between insomnia and hardiness, anxiety, and sleep disturbances., Materials and Methods: A descriptive-exploratory study was conducted using an online survey during the first wave of the COVID-19 pandemic (March to July 2020). The questionnaire consisted of multiple-choice, open-ended, closed, and semi-closed questions. The psychometric tools administered were the Dispositional Resilience Scale (DRS-15), the State-Trait Anxiety Inventory (STAI-Y), and the Insomnia Severity Index (ISI)., Results: a cohort of 1167 nurses fully completed the questionnaire (86.2% of total respondents). The insomnia scale survey showed an increase in post-pandemic scores compared to those before the pandemic, implying that insomnia levels increased after the first pandemic wave. Insomnia scores were directly correlated with anxiety levels (r = 0.571; p ≤ 0.05) and inversely correlated with hardiness levels (r = -0.324; p < 0.001). Multivariate analysis revealed the following protective factors: not having worked in COVID-19 wards, high levels of hardiness (commitment), and the presence of high pre-pandemic insomnia disorder. The main risk factor for insomnia reported in the analysis was a high anxiety score., Discussion and Conclusion: Anxiety represented the main risk factor for insomnia severity in our sample, while hardiness was confirmed as a protective factor. Thus, it is necessary to design further studies to identify additional risk factors for poor sleep quality and to develop educational courses and strategies aimed at enhancing rest and sleep quality, especially for frontline nurses.

At present, only a few reports have addressed the possible contribution of the lymphatic vascular system to the pathogenesis of systemic sclerosis (SSc). Based on the evidence that blood vascular endothelial cells can undertake the endothelial-to-myofibroblast transition (EndMT) contributing to SSc-related skin fibrosis, we herein investigated whether the lymphatic endothelium might represent an additional source of profibrotic myofibroblasts through a lymphatic EndMT (Ly-EndMT) process. Skin sections from patients with SSc and healthy donors were immunostained for the lymphatic endothelial cell-specific marker lymphatic vessel endothelial hyaluronan receptor-1 (LYVE-1) in combination with α-smooth muscle actin (α-SMA) as the main marker of myofibroblasts. Commercial human adult dermal lymphatic microvascular endothelial cells (HdLy-MVECs) were challenged with recombinant human transforming growth factor-β1 (TGFβ1) or serum from SSc patients and healthy donors. The expression of lymphatic endothelial cell/myofibroblast markers was measured by quantitative real-time PCR, Western blotting and immunofluorescence. Collagen gel contraction assay was performed to assess myofibroblast-like cell contractile ability. Lymphatic endothelial cells in intermediate stages of the Ly-EndMT process (i.e., coexpressing LYVE-1 and α-SMA) were found exclusively in the fibrotic skin of SSc patients. The culturing of HdLy-MVECs with SSc serum or profibrotic TGFβ1 led to the acquisition of a myofibroblast-like morphofunctional phenotype, as well as the downregulation of lymphatic endothelial cell-specific markers and the parallel upregulation of myofibroblast markers. In SSc, the Ly-EndMT might represent a previously overlooked pathogenetic process bridging peripheral microlymphatic dysfunction and skin fibrosis development.

Nurses and paramedics play a pivotal role when mass casualty incidents (MCI) occur, yet they often feel unprepared for such events. Implementation strategies for training activities, including virtual reality (VR) and augmented reality (AR) simulations, offer realistic and immersive learning experiences, enhancing skills and competencies for nursing students. The aim of this work was to investigate the adopted tools in studies on VR and AR simulations for training nursing and paramedic students in managing MCI. A scoping review was performed following the PRISMA-ScR statement, and the search strategy was conducted through five electronic databases from December 2022 to March 2023. Of 162 records identified, 27 full texts were screened and, six studies were included in this review. These studies involved students who were assigned to different training methods, including immersive VR simulation, written instruction, and traditional lecture. VR and AR and immersive simulation generally show promising evidence in enhancing practical skills and knowledge in MCI management. VR and AR showed to be promising in disaster education and preparedness training, offering different levels of immersiveness and engagement, encouraging active and experiential learning. Further research is needed to determine their long-term effectiveness. The choice of training method should consider program goals, target population, and available resources.

Psychological concerns in Systemic Sclerosis (SSc) patients represent an important issue and should be addressed through non-pharmacological treatments. Thus, the aim of the present study was to assess the effects of the Mindfulness-Based Stress Reduction (MBSR) program on psychological variables and the perspectives and experiences of patients with an SSc diagnosis. Notably, 32 SSc patients were enrolled and assigned to either the intervention (MBSR) group or the waitlist group. Inclusion criteria were (i) age ≥ 18 years, SSc diagnosis according to EULAR/ACR diagnostic criteria and informed consent. Exclusion criteria were previous participation in any Mind-Body Therapy or psychiatric diagnosis. Quantitative and qualitative outcomes were investigated through clinometric questionnaires and individual interviews. MBSR did not significantly impact outcomes such as physical functionality, anxiety, hopelessness, depression, physical health status, perceived stress, mindfulness and mental health status. For the anger evaluation, statistically significant differences are found for both controlling and expressing anger, indicating that the MBSR program had a favorable impact. As for qualitative results, more awareness of daily activities, stress reduction in terms of recognizing the causes and implementing self-strategies to prevent them, adherence to therapy, and recognition of the effect of medication on their bodies were reported. In conclusion, it is important to highlight the absence of negative or side effects of the MBSR program and the positive impact on patients' experience and perspective; thus, we suggest this approach should be taken into account for SSc patients.

Background: Noninvasive ventilation (NIV) in COVID-19 patients outside of intensive care unit (ICU) settings was a feasible support during the pandemic outbreak. The aim of this study was to assess the effectiveness of an “on the job” NIV training program provided to 66 nurses working in 3 COVID-19 wards in an Italian university hospital. Methods: A quasi-experimental longitudinal before−after study was designed. The NIV Team education program, provided by expert ICU nurses, included: 3 h sessions of training on the job during work-shifts about the management of helmet-continuous positive airway pressure (CPAP) Venturi systems, and NIV with oronasal and full-face masks. An eleven-item “brief skills self-report tool” was administered before and after the program to explore the perception of NIV education program attendees about their level of skills. Results: In total, 59 nurses responded to the questionnaire. There was an improvement in the skill levels of the management of Helmet-CPAP (median before training 2, inter-quartile range (IQR) 0−6; median after training 8, IQR 3−9; p < 0.0001), and mask-NIV (median before training 2, IQR 0−6; median after training 8, IQR 3−9; p < 0.0001). Conclusions: Training on the job performed by expert ICU nurses can be a valuable and fast means to implement new Helmet-CPAP and mask-NIV skills outside of ICUs.

Background: Clotting is a major drawback of continuous renal replacement therapy (CRRT) performed on critically ill pediatric patients. Although anticoagulation is recommended to prevent clotting, limited results are available on the effect of each pharmacological strategy in reducing filter clotting in pediatric CRRT. This study defines which anticoagulation strategy, between regional citrate anticoagulation (RCA) and systemic anticoagulation with heparin, is safer and more efficient in reducing clotting, patient mortality, and treatment complications during pediatric CRRT., Methods: A systematic literature review was run considering papers published in English until December 2021 and describing patients' and treatments' complications in CRRT performed with heparin and RCA on patients aged less than 18 years., Results: Eleven studies were considered, cumulatively comprising 1.706 CRRT sessions (62% with systemic anticoagulation and 38% with RCA). Studies have consistently identified RCA's superiority over systemic anticoagulation with heparin in prolonging circuit life. The pooled estimate (95% CI) of filter clotting risk showed that RCA is a protective factor for clotting risk (RR = 0.204)., Conclusions: RCA has a potential role in prolonging circuit life and seems superior to systemic anticoagulation with heparin in decreasing the risk of circuit clotting during CRRT performed in critically ill pediatric patients.

Objectives: Lung ultrasound (LUS), through assessment of B-lines and pleural line alterations, is able to evaluate interstitial lung disease (ILD), a frequent complication of SSc. Different scanning schemes and counting methods have been proposed but no clear cut-off values have been indicated for screening. We aimed to evaluate the accuracy of different LUS methodological approaches to detect ILD compared with high-resolution CT (HRCT) as the gold standard., Methods: Sixty-nine SSc patients underwent LUS and chest HRCT on the same day. Both exams were scored by expert readers. The accuracy of different scanning schemes and counting methods was assessed and clinical and functional data were compared with imaging findings., Results: B-lines were more numerous in patients with the diffuse skin subset and Scl70 autoantibody positivity. The number of B-lines correlated with the Scleroderma Lung Study (SLS) I HRCT score (R = 0.754, P < 0.0001). A total of >10 B-lines on the whole chest or >1 B-line on the postero-basal chest showed 97% sensitivity for detecting even very early ILD signs (corresponding to an SLS I score of 1). Sensitivity increased to 100% when pleural line alterations were included in the analysis., Conclusions: LUS has a very high sensitivity in detecting SSc-related ILD. A cut-off value of >10 B-lines on the whole chest or >1 B-line on the postero-basal chest can be used for the screening of SSc-ILD. Assessing only the postero-basal chest seems to be mostly effective, combining high sensitivity with a less time-consuming approach., (© The Author(s) 2021. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Background: In women with rheumatic diseases (RDs) menstruation-related disorders have never been investigated. The aim of this study was to evaluate gynecological symptoms/disorders in fertile age women with RDs. Materials and methods: All patients (n = 200) filled up a self-administered questionnaire on their gynecological history, menstrual cycle pattern, menstrual-related symptoms, and quality of life (QoL). The RD group was then compared to a control group of 305 age-matched fertile age women. Results: Among patients with RDs, 58% had arthritis, 40% connective tissue diseases (CTDs), and 1.5% systemic vasculitis. No differences were observed between CTDs and arthritis, except for a family history of HMB which was more common among women with CTDs ( p < .01). When compared to controls, women with RDs reported more frequent heavy menstrual bleeding (HMB) during adolescence (51.7 and 25.4%, respectively; p = .0001) and adult life (37.7 and 25.9%, respectively; p = .0065). Also, dysmenorrhea in adolescence was significantly more common among cases (55.6 and 45.4%, respectively; p = .0338). Gynecological pain (dysmenorrhea, non-menstrual pelvic pain, dyspareunia, dysuria, and dyschezia) in patients with RDs was more frequent than in controls ( p = .0001, .0001, .0001, .0001, .0002, respectively). Considering women who reported moderate and severe symptoms in RDs, dysmenorrhea and dyspareunia remain significantly more frequent in women with RDs than in controls ( p = .0001; p = .0022; respectively). QoL scores were significantly reduced in women with RDs, either in physical ( p = .0001) and mental domains ( p = .0014) of short-form 12. Conclusion: Women affected by RDs frequently presented menstruation-related disorders; thus, female patients with RDs should be questioned about gynecological symptoms and referred to the gynecologist for an accurate evaluation., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Orlandi, Vannuccini, El Aoufy, Melis, Lepri, Sambataro, Bellando-Randone, Guiducci, Cerinic and Petraglia.)

Aim: Few studies in the literature specifically address the hardiness of nurses during the COVID-19 pandemic. Thus, the primary aim of this study was to assess the impact of COVID-19 on the hardiness levels in an Italian cohort of nurses. The secondary aims were to assess the level of hardiness in nurses directly caring for patients with COVID-19 and to verify the presence of related risk and promoting factors., Methods: A descriptive and explorative study was performed through an online survey from March to July 2020. The survey was composed of a multiple answer questionnaire with open, closed, and semi-closed-ended questions. Hardiness and anxiety were assessed using two psychometric instruments: the Dispositional Resilience Scale (DRS-15) and State-Trait Anxiety Inventory (STAI-Y)., Results: A total of 1250 nurses completed the questionnaire entirely (92.3% of respondents). The average length of service was 17.8 ± 11.5 years. A decrease in the hardiness was recorded after the first wave of COVID-19 if compared to the baseline (mean Δ DRS-15 total = 1.3 ± 5.0), whereas in the subsample of nurses caring for COVID-19 patients, the total hardiness level decreased more consistently (mean Δ DRS Total = 1.9 + 5.3). Multivariate analysis showed that high levels of anxiety were risk factors for reducing hardiness. In contrast, anxiety, when associated with a greater length of service, was a promoting factor for the increase in hardiness., Conclusions: The correlation between anxiety and years of length of service appears to be pivotal. Future research should focus on the role of anxiety to establish its actual role as a predictor of hardiness.

In systemic sclerosis (SSc), the gastrointestinal tract (GIT) plays a central role in the patient's quality of life. The microbiome populates the GIT, where a relationship between the Lactobacillus and gastrointestinal motility has been suggested. In this study, the analysis of oral Lactobacillus species in SSc patients and healthy subjects using culture-independent molecular techniques, together with a review of the literature on microbiota and lactobacilli in SSc, has been carried out. Twenty-nine SSc female patients (mean age 62) and twenty-three female healthy subjects (HS, mean age 57.6) were enrolled and underwent tongue and gum swab sampling. Quantitative PCR was conducted in triplicate using Lactobacillus specific primers rpoB 1, rpoB 1o and rpoB 2 for the RNA-polymerase β subunit gene. Our data show significantly ( p = 0.0211) lower Lactobacillus spp rpoB sequences on the tongue of patients with SSc compared to HS. The mean value of the amount of Lactobacillus ssprpoB gene on the gumsofSSc patients was minor compared to HS. A significant difference between tongue and gums ( p = 0.0421) was found in HS but not in SSc patients. In conclusion, our results show a lower presence of Lactobacillus in the oral cavity of SSc patients. This strengthens the hypothesis that Lactobacillus may have both a protective and therapeutic role in SSc patients.

Background: Systemic sclerosis (SSc) alterations of the face and of the mouth cause aesthetic modifications and disability, impairing self-esteem and quality of life (QoL). The aim of this study was to verify the effects of two rehabilitation protocols on facial mimic and mouth opening., Methods: A total of 47 SSc patients (40 females and 7 males, mean age ± SD 59.08 ± 10.31 years), were consecutively selected: 22 were randomly assigned to protocol 1 [home exercises for temporomandibular joint (TMJ), mimic, masticatory and cervical spine muscles] and 25 to protocol 2 (home exercises and combined physiotherapeutic procedures performed by a physiotherapist). Each treatment had a duration of 12 weeks with a follow up of 8 weeks. TMJ dysfunction, orofacial involvement, disability, QoL, and safety were assessed at enrollment (T0), at the end of the treatment (T1), and at follow up (T2)., Results: Both Protocol 1 and Protocol 2 induced significant improvements of some clinical and clinimetric parameters, but better results were obtained with Protocol 2. In the comparison between the effects of Protocol 1 and Protocol 2 at T1 and T2, a significant difference was observed only for Mouth Handicap in SSc [MHISS; Total ( p  = 0.00178] and for MHISS Mouth opening ( p  = 0.0098) at T1. No significant difference of indices of short-form 36 was observed., Conclusion: The present data suggest that TMJ involvement in SSc may be managed by rehabilitation treatments. The action of a physiotherapist prescribing and personalizing exercises may induce better therapeutic effects., Competing Interests: Conflict of interest statement: The authors declare that there is no conflict of interest., (© The Author(s), 2021.)

Objectives: The aim of this study is to ascertain systemic sclerosis patients' preferences regarding the formulations of the medications they use., Methods: We undertook questionnaires and interviews aimed at understanding systemic sclerosis patients' preferences with respect to the medications they used., Results: Among 160 systemic sclerosis patients, we found that the majority does not have difficulty taking their medication. However, preferences were identified (81.25% - 65/80 - preferred oral meds and 47.50% - 38/80 - disliked rectal/vaginal meds), as well as some systemic sclerosis patients have significant difficulties using their medications. In fact, factors such as swallowing and fine finger motion difficulties were frequent, while intravenous/intramuscular/subcutaneous medicines were usually not preferred because they are felt as inconvenient (intravenous = 33.4% and subcutaneous/intramuscular = 10%) or painful (intravenous = 37.50% and subcutaneous/intramuscular = 10%)., Conclusion: Most systemic sclerosis patients are able to take their medication despite having some difficulties. However, as there were clear preferences, we could improve patients' adherence to drug therapy if taking these preferences into account., Competing Interests: Declaration of conflicting interest: The author(s) declared the following potential conflicts of interest with respect to the research, authorship and/or publication of this article: B.C.: Eli Lilly and Actelion; M-C.M.: Actelion, Bayer, BMS, Chemomab, J&J, Janssen, Lilly, MSD, and Pfizer, F.D.E.: Amgen, BMS, Boerinher-Ingelheim, Corbus, Galapagos, Genentech, Gilead, NIH, Novartis, Pfizer, Sanofi and Roche., (© The Author(s) 2020.)

Objective: Foot problems are often present in Systemic Sclerosis (SSc) patients, however studies regarding podiatric problems related to SSc are lacking and there are no data evaluating the foot biomechanical changes. The aim of the present pilot study was to evaluate podiatric problems in an Italian cohort of SSc patients by assessing received podiatric services, foot pain and disability and biomechanical foot deformity., Material and Methods: 25 consecutive SSc patients were enrolled from the Division of Rheumatology, University of Florence. All SSc patients were assessed by: Standards of Care for People with Foot Musculoskeletal Health problems: Audit Tool, Foot Function Index (FFI), Weight and non-weight bearing foot joint assessment, (Foot Posture Index (FPI) and Gait Cycle), Health Assessment Questionnaire (HAQ) and Medical Outcomes Survey Short Form 36 (SF-36)., Results: Audit Tool - Only 7 (28%) out of the 25 patients with SSc had a specific podiatric assessment and treatment: no patient received a foot health assessment within the first 6 months of disease diagnosis and no patient received information about foot involvement. 1 patient (4%) received foot assessment every year; 1 patient (4%) received specific information about the disease and 5 patients (20%) received information about the benefits of using adapted footwear and insoles. FFI - Values of pain, disability and activity limitations, reported in FFI, are 4.7±5.1, 5.1±3.2 and 3.2±3.1 (M±DS), respectively. Non-weight bearing foot joint assessment shows a rearfoot varus deformity in 64% of patients, forefoot varus deformity in 42% and 6% forefoot valgus deformity. Weight bearing foot joint assessment, through FPI shows a pronated foot 20% of patients with and 34% with highly pronated overall foot posture. Gait analysis shows that 64% of patients has a contact of the calcaneus in invertion while 36% in eversion. In the midstance, 78% have the foot in pronation and 22% in supination, while in propulsion 12% presents a takeoff of the foot in supination and 88% in the pronation. HAQ result is 1.13±0.80, SFI and SMI scales of SF-36 have scores of 32.38±10.65 and 38.67±11.40, respectively., Conclusion: Our results shows that podiatric problems in SSc patients are common, serious but foot assessment and health care are inadequate. Thus, foot health information should be improved in order to better empower patients to self-manage low risk problems and help identify high-risk problems, which require specialist care., Competing Interests: No conflict of interest was declared by the authors.