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Objectives To evaluate the performance of oropharyngoesophageal scintigraphy (OPES) in the assessment of dysphagia in patients with systemic sclerosis (SSc), and to compare OPES results with those of barium esophagogram. Methods Adult SSc patients who underwent OPES for the assessment of dysphagia were enrolled. OPES was performed with both liquid and semisolid boluses and provided information regarding oropharyngeal transit time, esophageal transit time (ETT), oropharyngeal retention index (OPRI), esophageal retention index (ERI), and site of bolus retention. Barium esophagogram results were also collected. Results Fifty-seven SSc patients (87.7% female, mean age 57.7 years) with dysphagia were enrolled. OPES identified at least one alteration in each patient and findings were generally worse for the semisolid bolus. Esophageal motility was widely impaired with 89.5% of patients with an increased semisolid ERI, and middle-lower esophagus was the most frequent site of bolus retention. However, oropharyngeal impairment was highlighted by widespread increased OPRI, especially in anti-topoisomerase I positivity. Older patients and with longer disease duration presented slower semisolid ETT (p = 0.029 and p = 0.002, respectively). Eleven patients with dysphagia had a negative barium esophagogram: all of them presented some alterations in OPES parameters. Conclusion OPES revealed a marked SSc esophageal impairment, in terms of both slowed transit time and increased bolus retention, but also shed light on oropharyngeal swallowing alterations. OPES showed high sensitivity, being able to detect swallowing alterations in dysphagic patients with negative barium esophagogram. Therefore, the use of OPES for the assessment of SSc-related dysphagia in clinical practice should be promoted.

Introduction The aim of this study was to investigate the incidence and clinical presentation of SARS-CoV-2 infections in a Systemic Lupus Erythematosus (SLE) cohort; to assess correlations with disease characteristics and rheumatic therapy; and to evaluate the occurrence of treatment discontinuation and its impact on disease activity. Materials and methods SLE patients monitored by a single Italian centre were interviewed between February and July 2020. Patients were considered to be positive for SARS-CoV-2 infections in case of 1) positive nasopharyngeal swab; 2) positive serology associated with COVID19 suggesting symptoms. The following data were also recorded: clinical symptoms, adoption of social distancing measures, disease activity and treatment discontinuation. Results 332 patients were enrolled in the study. Six patients (1.8%) tested positive for SARS-CoV-2 infection, with the incidence being significantly higher in the subgroup of patients treated with biological Disease-Modifying Anti-Rheumatic Drugs (p = 0.005), while no difference was observed for other therapies, age at enrollment, disease duration, type of cumulative organ involvement or adoption of social isolation. The course of the disease was mild. Thirty-six patients (11.1%) discontinued at least part of their therapy during this time period, and 27 (8.1%) cases of disease flare were recorded. Correlation between flare and discontinuation of therapy was statistically significant (p Conclusion Treatment discontinuation seems to be an important cause of disease flare. Our findings suggest that abrupt drug withdrawal should be avoided or evaluated with caution on the basis of individual infection risk and comorbidities.

Systemic sclerosis (SSc) is a connective tissue disease characterised by diffuse microangiopathy and immune dysregulation which ultimately results in widespread fibrosis of the skin and internal organs. This complex autoimmune disease is characterised by heterogeneous clinical manifestations and variable disease course in which the severity of pathology dictates the disease prognosis and course. Every year novel insights into the pathogenesis, organ involvement and treatment of this severe disease are published. Herewith, we provide an overview of the most significant literature contributions published last year, with the aim of helping the clinician in the understanding and management of SSc patients.

Digital ulcers (DUs) represent one of the major burdens for patients with systemic sclerosis (SSc), especially when associated with skin calcinosis (SC). The aim of this work is to evaluate the impact of SC in DUs of patients with SSc for clinical characteristics and prognosis assessed by the wound bed score (WBS). We prospectively enrolled 55 patients with DUs and SSc followed in our dedicated wound care clinic. For all the patients we collected clinical and anthropometric data and characteristics of the DU, and we calculated the WBS for each DU. Ninety-nine DUs were evaluated (24 with SC). SC was prevalent in limited cutaneous SSc (75%) and in patients with longer disease duration (P = 0.02). SC-DUs were prevalent at the fingertip (P = 0.04). The healing time was significantly higher in patients with SC (10.4 ± 7.9 weeks) compared with non-SC (7.0 ± 5.7 weeks) P = 0.03. The WBS negatively correlated with the time to achieve complete healing (r = -0.237 P = 0.023) and the correlation was maintained in the non-SC (r = -0.46, P = 0.033). DUs in SSc patients with SC are common and difficult to heal. When DUs are treated in dedicated centres, the prognosis is good. The WBS is fast and easy and maybe commonly applied in clinical practice.

Systemic sclerosis (SSc) is a complex disorder characterised by the involvement of small arteries, microvessels and connective tissue, with deposition of fibrotic tissue and microvascular obliteration in the skin and internal organs. Due to the multifaceted nature of the disease, several articles are published in the medical literature every year, aimed at exploring different aspects of the pathogenesis, internal organ involvement and clinical aspects, and possible therapeutic approaches. In this article we have reviewed the literature on SSc of the past year, with the aim of identifying novel approaches that may help the treating physician in the clinical management of patients.

Background Systemic sclerosis (SSc) is an autoimmune chronic disease characterized by prominent vascular involvement. Intravenous iloprost (IV ILO), according to the recently updated EULAR recommendations, is indicated for Ssc Raynaud’s phenomenon (RP) after failure of oral therapy. Moreover, IV ILO could be useful in DU healing and may represent a potential disease modifying medication. However, there are no uniform data regarding type of regimen (dosage, duration and frequency) and infusion modality. Objectives The purpose of this study was to compare effectiveness (in terms of Dus) and direct costs, of two regimens of IV ILO infusion in the same cohort of consecutive SSc subjects. Methods Protocol A (A): the patient was admitted and 100 mcg of ILO was diluted in 500 ml of normal saline or 5% dextrose solution and infused continually at the maximum tolerated dose until exhaustion. Protocol B (B): the patient was followed as outpatient at infusion clinic and 50 mcg of ILO was diluted in 250 of normal saline or 5% dextrose and infused at a dose range from 0.5 to 1.5 ng/Kg/min. The dose was escalated at 30 minutes intervals and maintained at the maximum tolerated dose for 5 consecutive hours for two consecutive days. The intervals between the infusions were between 6-8 weeks in both protocols depending on clinical response and availability of places at the Unit. 44 patients who received long term IV ILO as inpatients (Cohort A), after a wash out of 3 months, were switched to ambulatory administration (Cohort B). Thereafter, after one year of follow-up 24 patients of the protocol B were lost to follow-up and 20 patients were maintained on this regimen. Comparison was made between Cohort A (44 patients=A44) in the period between march 2015 and October 2016 (period A), Cohort B (44 patients=B44) between October 2016 and March 2017 (period B44), Cohort B (20 patients=B20), between October 2017 and March 2018 (period B20). Comparison between groups was made by non parametric tests and contingency table analysis when appropriate. Costs were estimated multiplying data related to resource use (drug consumpion, hospital stay, outpatient visits, management of complications, etc) by unit cost obtained from the accounting office of the Hospital. All costs were expressed in Euro Results Mean number of DUs at the end of period A was 0,37±0,98 in A44 as compared to 0,90±1,39 of period B44 cohort B44 (p=0,045) and 0,55±0,95 of period B20 Cohort B20 (p=n.s.). Cumulative number of DUs was 9/44 in Cohort A (20.5%) as compared to 20/44 in Cohort B44 (45% p=0.002) and 7/20 in cohort B20 (35% p=0,05). High number of drop outs (24/44) in protocol B was due mainly to statistical significant difference in tolerability (adverse events 11% protocol A vs 42% protocol B p Conclusion Protocol A seems to represent a regimen better tolerated and more effective than protocol B. Moreover, protocol B is affected by high rate of drops out due mainly to worse tolerability. Admitting SSc-patients for continuous IV ILO infusion seems to represent a better tolerated and more effective choice than following them as outpatients at infusion clinic, with substantially comparable direct costs in the same SSc cohort. Disclosure of Interests Alessandra Della Rossa: None declared, Simone Barsotti: None declared, Marco Di Battista: None declared, Lorenzo Puccetti: None declared, Valentina Lorenzoni: None declared, Giuseppe Turchetti: None declared, Marta Mosca Paid instructor for: GlaxoSmithKline, Lilly, UCB

Our objective was to compare three algorithms for cardiovascular (CV) risk estimation, namely Framingham, ACC/AHA and QRISK3, in a cohort of patients with systemic lupus erythematosus (SLE).Consecutive patients with SLE according to the ACR criteria were enrolled. Traditional risk factors, ongoing therapies, comorbidities and SLE-specific evaluations were assessed. In those without previous myocardial infarction or stroke, Framingham, ACC/AHA and QRISK3 algorithms were then used to estimate the individual risk of developing a CV disease over the next 10 years.Patients eligible for CV risk estimation were 123 out of 135 enrolled. Framingham index reported a median risk score of 4.7% (IQR 9.5-2.2), considering 29 patients (23.6%) at high CV risk. ACC/AHA index showed a median risk score of 1.4% (IQR 4.5-0.7), with 17 patients (13.8%) at high-risk. QRISK3 revealed a median risk score of 6.2% (IQR 12.5-2.8), making it possible to classify 44 patients (35.8%) at high CV risk. The subgroup analysis of subjects older than 40 years confirmed the same number of high-risk patients for both Framingham and ACC/AHA, whereas QRISK3 classified 38 subjects at high CV risk.QRISK3 classifies a greater number of SLE patients at high-risk of developing CV diseases over the next 10 years in comparison with classic algorithms as Framingham and ACC/AHA. If its predictive accuracy were confirmed by longitudinal data, QRISK3 could become an important tool in the early detection of a considerable part of CV high-risk SLE patients that would be underestimated when applying classic algorithms.

Systemic sclerosis is a rare acquired systemic disease characterised by a complex pathogenesis and multi organ involvement. Every year the scientific world contributes to enrich the knowledge on the pathogenesis, clinical manifestations, diagnosis and treatment of this complex and severe disease. Herewith, we provide an overview of the most significant literature contributions published over the last year.

Systemic lupus erythematosus (SLE) is a systemic autoimmune condition characterised by a wide spectrum of clinical manifestations, partly related to the disease itself, but also linked to its comorbidities and drugs adverse reactions. Following the previous annual reviews, we focused on new insights in SLE clinical features, pathogenic pathways, biomarkers of specific organ involvement and therapeutic strategies. We finally concentrated on SLE aspects that could significantly influence patients' quality of life and that need to be investigated in detail through the development and validation of disease-specific patient-reported outcomes.

This article deals with probabilistic upper bounds for the error in functional estimation defined on some interpolation and extrapolation designs, when the function to estimate is supposed to be analytic. The error pertaining to the estimate may depend on various factors: the frequency of observations on the knots, the position and number of the knots, and also on the error committed when approximating the function through its Taylor expansion. When the number of observations is fixed, then all these parameters are determined by the choice of the design and by the choice estimator of the unknown function. The scope of the article is therefore to determine a rule for the minimal number of observation required to achieve an upper bound of the error on the estimate with a given maximal probability.