Improving organisation to improve care: ERN ReCONNET organisational reference model for systemic sclerosis patients' care pathway.

Objective: To optimise the organisation of care and encourage the adoption of good clinical practices, the RarERN Path methodology was designed within ERN ReCONNET. The aim of our work was to report the application of RarERN Path on systemic sclerosis within the ERN ReCONNET centres, providing a feasible and flexible organisational reference model for optimising the systemic sclerosis care pathway in different countries.

Methods: RarERN Path is a six-phase methodology which enables the creation of a reference organisational model co-designed on the basis of the expertise of different stakeholders.

The communication GAP between patients and clinicians and the importance of patient reported outcomes in Systemic Lupus Erythematosus.

Systemic Lupus Erythematosus (SLE) imposes a great burden on the lives of patients. Patients' and physicians' concerns about the disease diverge considerably. Physicians focus on controlling disease activity to prevent damage accrual, while patients focus on symptoms that impact on Health-Related Quality of Life (HRQoL).

Towards a novel clinical outcome assessment for systemic lupus erythematosus: first outcomes of an international taskforce.

Systemic lupus erythematosus (SLE) is a disease of high unmet therapeutic need. The challenge of accurately measuring clinically meaningful responses to treatment has hindered progress towards positive outcomes in SLE trials, impeding the approval of potential new therapies. Current primary end points used in SLE trials are based on legacy disease activity measures that were neither specifically designed for the clinical trial context, nor developed according to contemporary recommendations for clinical outcome assessments (COAs), such as that substantial patient input should be incorporated into their design.

Coping with systemic lupus erythematosus in patients' words.

Objective: Previous research on coping strategies of patients with SLE showed that there are no absolute adaptive or maladaptive strategies and that the range of potential coping strategies is large and heterogeneous. In this paper, we aimed to identify, in a large sample of patients with SLE (N=3222), the most frequent words used by patients to describe their coping strategies, to group them into significant themes and to test their possible association with specific patient characteristics.

Methods: Our analyses were based on the data set of the European survey 'Living with Lupus in 2020' (N=3222).

The added value of a European Reference Network on rare and complex connective tissue and musculoskeletal diseases: insights after the first 5 years of the ERN ReCONNET.

In order to address the main challenges related to the rare diseases (RDs) the European Commission launched the European Reference Networks (ERNs), virtual networks involving healthcare providers (HCPs) across Europe. The mission of the ERNs is to tackle low prevalence and RDs that require highly specialised treatment and a concentration of knowledge and resources. In fact, ERNs offer the potential to give patients and healthcare professionals across the EU access to the best expertise and timely exchange of lifesaving knowledge, trying to make the knowledge travelling more than patients.

Association of common variable immunodeficiency and rare and complex connective tissue and musculoskeletal diseases. A systematic literature review.

Objectives: To perform a systematic literature review (SLR) on the association of common variable immunodeficiency (CVID) and rare and complex connective tissue and musculoskeletal diseases, namely systemic lupus erythematosus (SLE), Sjögren's syndrome (SS), idiopathic inflammatory myopathies (IIM), systemic sclerosis (SSc), relapsing polychondritis, antiphospholipid syndrome, immunoglobulin (Ig) G4-related disease, as well as undifferentiated and mixed connective tissue disease.

Methods: An SLR on studies and cases about the association of CVID and rare and complex connective tissue and musculoskeletal diseases was performed. Animal studies were excluded.

What are the topics you care about making trials in lupus more effective? Results of an Open Space meeting of international lupus experts.

Despite promising candidates for new therapeutic options in the treatment of systemic lupus erythematosus (SLE), many clinical trials have failed in the past few years. The disappointing results have been at least partly be attributed to trial designs. With the aim of stimulating new developments in SLE trial design, an international open space meeting was held on occasion of the European Lupus Meeting 2018 in Duesseldorf, Germany about 'What are the topics you care about for making trials in lupus more effective?'.

Hydroxychloroquine availability during COVID-19 crisis and its effect on patient anxiety.

Objective: To report the results of a survey exploring the experience of patients with SLE facing hydroxychloroquine (HCQ) shortage that occurred during the early phases of the COVID-19 pandemic.

Methods: A survey was designed by Lupus Europe's patient advisory network and distributed through its social media, newsflash and members' network. People with lupus were asked about their last HCQ purchases and their level of anxiety (on a 0-10 scale) with regard to not being able to have access to HCQ, once in April 2020 (first wave) and after 11 August (second wave).

Living with systemic lupus erythematosus in 2020: a European patient survey.

Objective: The aim of this study was to analyse the 2020 burden of Systemic Lupus Erythematosus (SLE) in Europe, from the patients' perspective.

Methods: In May 2020, Lupus Europe, the European umbrella patient association for SLE, designed and disseminated a multilingual anonymous online survey to individuals with a self-reported physician's diagnosis of SLE living in Europe.

Results: Data from 4375 SLE survey respondents (95.

Hydroxychloroquine treatment in European patients with lupus erythematosus: dosing, retinopathy screening and adherence.

Background: Use of hydroxychloroquine (HCQ) is common in patients with lupus erythematosus. Long-term use (ie, ≥5 years) and high-dose HCQ (ie, >5 mg/kg/day) are both risk factors for developing HCQ retinopathy. Advances in our understanding of HCQ retinopathy have led to changes in the recommendations for HCQ dosing and retinopathy screening.

The impact of COVID-19 on rare and complex connective tissue diseases: the experience of ERN ReCONNET.

During the COVID-19 pandemic, the need to provide high-level care for a large number of patients with COVID-19 has affected resourcing for, and limited the routine care of, all other conditions. The impact of this health emergency is particularly relevant in the rare connective tissue diseases (rCTDs) communities, as discussed in this Perspective article by the multi-stakeholder European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases (ERN ReCONNET). The clinical, organizational and health economic challenges faced by health-care providers, institutions, patients and their families during the SARS-CoV-2 outbreak have demonstrated the importance of ensuring continuity of care in the management of rCTDs, including adequate diagnostics and monitoring protocols, and highlighted the need for a structured emergency strategy.

Exploring patient education unmet needs for rare and complex connective tissue and musculoskeletal diseases: A survey of health care providers' and patients' expectations in Europe.

Objective: The European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases involves health care providers (HCPs) from 8 European countries and 7 patients' representatives of European Patient Advocacy Groups. The objective was to evaluate current practice and unmet needs for patient education (PE) in Europe.

Methods: A questionnaire was sent to HCP members asking about the PE practices and another, to enquire about their needs, was sent to patients' associations in the different countries.

Systemic lupus erythematosus: state of the art on clinical practice guidelines.

Systemic lupus erythematosus (SLE) is the paradigm of systemic autoimmune diseases characterised by a wide spectrum of clinical manifestations with an unpredictable relapsing-remitting course. The aim of the present work was to identify current available clinical practice guidelines (CPGs) for SLE, to provide their review and to identify physicians' and patients' unmet needs. Twenty-three original guidelines published between 2004 and 2017 were identified.

Living with systemic lupus erythematosus: A patient engagement perspective.

Patient engagement is recognized as a crucial component of high-quality healthcare services. Among rheumatic diseases, systemic lupus erythematosus (SLE) appears particularly challenging for the engagement of patients in their own care. According to the patient health engagement (PHE) model, patient engagement is a dynamic phenomenon that proceeds through four experiential positions (blackout, arousal, adhesion and eudaimonic project).

Synchronized Fabry-Perot cavity used for enhanced sensitivity electro-optic measurements of electric fields in the microwave range.

An electro-optic (EO) sensing system for measuring free-space electric fields in the microwave range has been developed. The system is based on a phase modulation heterodyning technique using a Mach-Zehnder interferometer. In one of the arms of the interferometer, an acousto-optic frequency shifter is used to downconvert the frequency of the detected signal in order to discriminate it from parasites emitted at the external electric field frequency.

Equations for solar tracking.

Direct sunlight absorption by trace gases can be used to quantify them and investigate atmospheric chemistry. In such experiments, the main optical apparatus is often a grating or a Fourier transform spectrometer. A solar tracker based on motorized rotating mirrors is commonly used to direct the light along the spectrometer axis, correcting for the apparent rotation of the Sun.

Improved detection sensitivity of D-mannitol crystalline phase content using differential spectral phase shift terahertz spectroscopy measurements.

We report quantitative measurement of the relative proportion of δ- and β-D-mannitol crystalline phases inserted into polyethylene powder pellets, obtained by time-domain terahertz spectroscopy. Nine absorption bands have been identified from 0.2 THz to 2.

Spectral filtering of visible light by the cuticle of metallic woodboring beetles and microfabrication of a matching bioinspired material.

Samples of the cuticle taken from the body of Buprestidae Chrysochroa vittata have been studied by scanning electron microscopy and optical reflectance measurements, related to numerical simulations. The cause of the metallic coloration of the body of these insects is determined to be the structure of the hard carapace constructed as a stack of thin chitin layers separated by very thin irregular air gaps. In particular the change of color as a function of the observation angle is elucidated in terms of an infinite photonic-crystal model, confirmed by finite multilayer calculations.