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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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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 rpoB1, rpoB1o and rpoB2 for the RNA-polymerase β subunit gene. Our data show significantly (p = 0.0211) lower LactobacillusspprpoB 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. [ABSTRACT FROM AUTHOR]

• Very little is available for early screening and diagnosis of bowel vasculopathy in SSc. • CDU can demonstrate impedance and elasticity impairment of splanchnic vessels in SSc. • SMA and IMA CDU may support a therapeutic approach targeted on early SSc involvement of splanchnic circulation. Gastrointestinal (GI) manifestations are frequent in systemic sclerosis (SSc) with an impact on quality of life and morbidity. Bowel vasculopathy is a key pathogenetic factor responsible for GI involvement. To compare abdominal ultrasound (US) and Color Doppler Ultrasonography (CDU) features of splanchnic vessels of SSc patients with healthy controls. The charts of SSc patients who underwent an abdominal US and CDU study were retrospectively analyzed. For Superior Mesenteric Artery (SMA) and Inferior Mesenteric Artery (IMA) caliber, Peak Systolic Velocity (PSV), Reverse Velocity (RV), End-Diastolic Velocity (EDV), Mean Velocity (mV), Blood-flow, Resistive Index (RI) and Pulsatility Index (PI) were recorded. 28 SSc patients and 28 controls were enrolled. In SSc, caliber of SMA was significantly smaller than in controls (5.75 ± 0.62 mm vs. 6.45 ± 0.60 mm, p < 0.0001 – p adj =0.0002). The flow study of SMA and IMA showed a significant reduction of RV (SMA: 7.25 ± 6.37 cm/s vs. 18.52 ± 6.16 cm/s, p < 0.0001 - p adj <0.0001; IMA: 2.69 ± 6.10 cm/s vs. 17.06 ± 5.75 cm/s, p < 0.0001 - p adj <0.0001) and PI (SMA: 3.33 ± 0.75 vs. 4.53 ± 1.03, p < 0.0001 - p adj =0.0002; IMA: 3.54 ± 0.95 vs. 6.08 ± 1.53, p < 0.0001 - p adj <0.0001) in SSc patients than controls. involvement of splanchnic vessels in SSc may be non-invasively investigated with abdominal US and CDU. Morphological and functional changes of Doppler parameters observed in SMA and IMA clearly demonstrate that these vessels are affected by SSc vasculopathy. [ABSTRACT FROM AUTHOR]

Renal Resistive Index (RRI), reflects changes in both renal vascular and tubular-interstitial compartments and in systemic vascular compliance related to age and comorbidities. a) To investigate determinants of RRI in SSc population, b) its association with SSc-related features and c) to test its prognostic impact on organ specific worsening or death. 380 SSc patients ≥18 years were enrolled after giving informed consent. Baseline data on RRI, laboratory, instrumental and therapeutic features were retrospectively collected. Age-SSc adjusted cut-offs were created by dividing the population in age quartiles and considering RRI values >75th percentile as pathologic. Clinical follow-up was performed until last available visit or the development/worsening of specific internal organ involvement or death. RRI was independently predicted by age and systolic pulmonary arterial pressure on Echo. Therefore, we created Age-SSc adjusted pathologic RRI cut-offs, which were significantly associated with various disease related skin and lung fibrotic manifestations, as well as vasculopathic complications. After a mean follow-up of 3.6 ± 2.6 years, RRI was one of the independent predictors (together with modified Rodnan skin score, interstitial lung disease, presence of dyspnoea and late nailfold-videocapillaroscopy pattern) for mortality, with 0.68 as best cut-off (sensitivity 88.5%, specificity 50.9%). If corroborated, Renal Resistive Index cut-offs might be used to evaluate renal and extrarenal involvement in SSc and could serve as predictors of mortality. • Renal Resistive Index (RRI) is applicable in systemic sclerosis. • RRI reflex renal and extra-renal involvement in scleroderma. • RRI has prognostic impact on mortality. • Age-SSc adjusted cut offs should be used when measuring RRI in scleroderma patients. [ABSTRACT FROM AUTHOR]