A patient-driven registry on Behçet's disease: the AIDA for patients pilot project.

Introduction: This paper describes the creation and preliminary results of a patient-driven registry for the collection of patient-reported outcomes (PROs) and patient-reported experiences (PREs) in Behçet's disease (BD). Methods: The project was coordinated by the University of Siena and the Italian patient advocacy organization SIMBA (Associazione Italiana Sindrome e Malattia di Behçet), in the context of the AIDA (AutoInflammatory Diseases Alliance) Network programme. Quality of life, fatigue, socioeconomic impact of the disease and therapeutic adherence were selected as core domains to include in the registry. Results: Respondents were reached via SIMBA communication channels in 167 cases (83.5%) and the AIDA Network affiliated clinical centers in 33 cases (16.5%). The median value of the Behçet's Disease Quality of Life (BDQoL) score was 14 (IQR 11, range 0-30), indicating a medium quality of life, and the median Global Fatigue Index (GFI) was 38.7 (IQR 10.9, range 1-50), expressing a significant level of fatigue. The mean Beliefs about Medicines Questionnaire (BMQ) necessity-concern differential was 0.9 ± 1.1 (range - 1.8-4), showing that the registry participants prioritized necessity belief over concerns to a limited extent. As for the socioeconomic impact of BD, in 104 out of 187 cases (55.6%), patients had to pay from their own pocket for medical exams required to reach the diagnosis. The low family socioeconomic status (p < 0.001), the presence of any major organ involvement (p < 0.031), the presence of gastro-intestinal (p < 0.001), neurological (p = 0.012) and musculoskeletal (p = 0.022) symptoms, recurrent fever (p = 0.002), and headache (p < 0.001) were associated to a higher number of accesses to the healthcare system. Multiple linear regression showed that the BDQoL score could significantly predict the global socioeconomic impact of BD (F = 14.519, OR 1.162 [CI 0.557-1.766], p < 0.001). Discussion: Preliminary results from the AIDA for Patients BD registry were consistent with data available in the literature, confirming that PROs and PREs could be easily provided by the patient remotely to integrate physician-driven registries with complementary and reliable information.

Empowering Patients in the Therapeutic Decision-Making Process: A Glance Into Behçet's Syndrome.

Behçet's syndrome (BS) represents a challenging condition, characterized by a variable spectrum of disease profile and associated with a significant limitation of the daily activities as well as a potential negative impact on relationships and psychological status. Considering also the complexity of the therapeutic management of BS, that often includes biological off-label treatments, the participation in the therapeutic decision-making process of the BS patients is essential to ensure the integration of the care process into the life of the patient. For this reason, the empowerment of BS patients represents a crucial need and the present work is aimed at fully exploring all the potential variables implicated in the BS patient empowerment, also highlighting major points to consider and concrete actions to be planned in the immediate future in order to implement a pragmatic facilitation of the patients' empowerment.

Adherence to treatment in Behçet's syndrome: a multi-faceted issue.

OBJECTIVES: The aim of this study is to explore the adherence to treatment in patients with Behçet's syndrome (BS), to identify the diverse adherence profiles and their correlations. METHODS: A cross-sectional study among adult BS patients was conducted administering an ad-hoc questionnaire to BS patients with the aim of investigating several dimensions related to BS management, including attitudes towards treatment. A Latent Class Analysis (LCA) was performed to identify adherence profiles and associated characteristics were identified using logistic regression analysis. RESULTS: A total of 207 patients answered the survey and 180 of them declared to take medication for BS, thus representing the study population. More than a third of the respondents have declared that they have skipped treatments before and autonomously modified (reduced or increased) the dosage of the treatment without medical consultation. LCA analysis allowed the identification of two distinct profiles, one more stick with recommended medication and the other less adherent to treatment. The less-adherent BS patient profile seems to be related with being in the third decade of life, being diagnosed with BS for more than 5 years and perceiving greater psychological impact of the disease. CONCLUSIONS: Addressing adherence in BS is not only related to measuring treatment adherence and identifying the barriers and the limitations; in fact, it should also encompass a wider approach that includes the awareness, the socio-psychological impact of the disease as well as patient education.

Being a caregiver of a Behçet's syndrome patient: challenges and perspectives during a complex journey.

BACKGROUND: As often seen in many chronic diseases, the disease impact on patients also induces a significant impact on the quality of life (QoL) of caregivers. Caregivers are the ones who are really willing to offer care in the general approach of many aspects of the disease, including the awareness of the diseases itself, the daily management of therapy, and all the potential challenges that living with a chronic disease can include. The main objectives of the study were to explore the perspectives and views of caregivers of Behçet's syndrome (BS) patients, to study their level of awareness on the disease and the impact that BS may have on their lives by means of a survey co-designed with caregivers and patients with this purpose. A survey was entirely co-designed with a panel of caregivers of patients living with BS patients. RESULTS: Results show that BS caregivers organise their life according to the needs of the patient, that they (79%) considered themselves as helpful for the patient and 53% of them replied that they can freely express their emotions. Notably, 70% and 68% of the respondents reported they renounced with a variable frequency to sexual relationships due to concerns regarding the health of the partner or to the partner's illness, respectively. The majority (79%) of respondents indicated that they are familiar with the treatment taken by the patients and that 68% deal with the administration of some medicines. In terms of awareness, a good percentage (64%) of respondents reported to understand the illness and, in terms of education, 68% of participants are willing to take part in training programmes dedicated to BS. CONCLUSIONS: The results of this survey contribute to provide new information on BS caregivers and on their important role, and to identify areas in which new initiatives could provide BS caregivers (and therefore patients) with tools and knowledge that can empower them in reducing the burden of the disease on their lives, on families, and on the patient.

Prevalence and clinical course of SARS-CoV-2 infection in patients with Behçet's syndrome.

OBJECTIVES: We aimed to assess the prevalence of SARS-CoV-2 infection among Behçet's syndrome (BS) patients, evaluating the possible association between demographic and clinical features and the risk of infection. Moreover, we aimed to evaluate the possible association between BS disease activity and treatment, and the risk of SARS-CoV-2 infection. METHODS: A survey was conducted on BS patients followed at the Behçet's Centre of the Careggi University Hospital, Florence, Italy. We further evaluated the possible association between BS disease activity and treatment, and the risk of SARS-CoV-2 infection. RESULTS: Out of 335 BS patients contacted, fourteen cases of SARS-CoV-2 were identified between April 1st, 2020 and February 9th, 2021, suggesting a prevalence of SARS-CoV-2 infection among BS patients of 4.2%, in line with the data of the general population in Italy (4.4%). When comparing clinical features between SARS-CoV-2 cases and matched SARS-CoV-2 negative BS patients, we found that the presence of different disease manifestations did not significantly differ between the two groups. SARS-CoV-2 cases and controls were also comparable in terms of immunosuppressive therapy, with the only exception of corticosteroids (71.4% vs. 35.7%, p=0.030), whose daily dose was significantly higher in cases than controls [5mg/day (IQR 0-10,) vs. 0 mg/day (IQR 0-5), p=0.005], suggesting that the right timing of usage and the more appropriate dosage of corticosteroid are a key question for the better management of these patients. CONCLUSIONS: Based on our results, patients with BS do not seem to be at a greater risk of SARS-CoV-2 infection or severe complications compared with the general population.

Biologics, colchicine, corticosteroids, immunosuppressants and interferon-alpha for Neuro-Behçet's Syndrome.

BACKGROUND: Neuro-Behçet Syndrome (NBS) is a severe chronic inflammatory vascular disease involving the Central Nervous System (CNS), and it is an invalidating condition with disability and a huge impact on quality of life. Recommendations on treatments for NBS include the use of disease-modifying therapies in general, although they are not supported by a systematic review of the evidence. OBJECTIVES: To assess the benefit and harms of available treatments for NBS, including biologics, colchicine, corticosteroids, immunosuppressants and interferon-alpha. SEARCH METHODS: We searched the following databases up to 30 September 2014: Trials Specialised Register of The Cochrane Multiple Sclerosis and Rare Diseases of the Central Nervous System Group, CENTRAL, MEDLINE, EMBASE, CINAHL, LILACS, ORPHANET, Clinicaltrials.gov and World Health Organization (WHO) International Clinical Trials Registry Portal. SELECTION CRITERIA: Randomised controlled trials (RCTs), controlled clinical trials (CCTs), prospective and retrospective controlled cohort studies were eligible to assess the benefit. Patients over 13 years of age with a diagnosis of NBS. For assessment of harms, open-label extension (OLE), case-control studies, population-based registries, case-series and case-reports were additionally planned to be evaluated. DATA COLLECTION AND ANALYSIS: Selection of studies, data extraction and assessment of risk of bias were planned to be carried out independently by two review authors. Standard methodological procedures expected by The Cochrane Collaboration were followed. We planned to perform standard pair-wise meta-analyses for RCTs, and meta-analyses based on the adjusted estimates using the inverse-variance weighted average method for non-randomised studies (NRSs). We planned to present the main results of the review in a 'Summary of Findings' table using the GRADE approach. MAIN RESULTS: No RCTs, CCTs or controlled cohort studies on the benefit of the treatments for NBS met the inclusion criteria of the review. Only one potentially eligible study was identified, but it did not report sufficient details on the patient characteristics. The author of this study did not provide additional data on request, and therefore it was excluded. Hence, no studies were included in the present review. Since no studies were included in the assessment of benefit, no further search was performed in order to collect data on harms. AUTHORS' CONCLUSIONS: There is no evidence to support or refute the benefit of biologics, colchicine, corticosteroids, immunosuppressants and interferon-alpha for the treatment of patients with NBS. Thus, well-designed multicentre RCTs are needed in order to inform and guide clinical practice.