No abstract

ObjectivePulmonary hypertension (PH) may occur in Takayasu arteritis (TA), mostly due to pulmonary arteritis, but also due to left heart disease and/or chronic thromboembolism (CTE). Using transthoracic echocardiography (TTE), we investigated the frequency of PH caused by pulmonary arteritis.MethodsThis cross-sectional study included 70 patients with TA fulfilling the 1990 ACR criteria, 68 healthy controls, and 67 patients with systemic sclerosis (SSc) fulfilling the 1980 ACR criteria representing the disease control group. Patients with severe left heart disease or CTE were excluded. The ESC-ERS guideline definition was considered for diagnosis of PH.ResultsThe mean systolic pulmonary artery pressure (SPAP) values in TA, SSc, and healthy control groups were 20.93±6.06, 31.57±12.75, and 18.88±5.39 mmHg, respectively. While the SPAP values were similar between TA and healthy groups, the SPAP values in the SSc group were significantly higher than in other groups. Based on conventional and/or magnetic resonance angiography findings, pulmonary arteritis was present in 4 out of 70 TA patients; however, PH was not detected in any patients with TA, including those with pulmonary arteritis.ConclusionThe TTE findings suggested that the frequency of PH was not increased in TA. However, a low frequency of pulmonary arteritis in our series might have affected our results.

No abstract

Purpose of the present paper is to point out the design, development and deployment of the AutoInflammatory Disease Alliance (AIDA) International Registry dedicated to pediatric and adult patients with Behçet’s disease (BD). The Registry is a clinical physician-driven non-population- and electronic-based instrument implemented for the retrospective and prospective collection of real-life data about demographics, clinical, therapeutic, laboratory, instrumental and socioeconomic information from BD patients; the Registry is based on the Research Electronic Data Capture (REDCap) tool, which is thought to collect standardised information for clinical real-life research, and has been realised to change over time according to future scientific acquisitions and potentially communicate with other existing and future Registries dedicated to BD. Starting from January 31st, 2021, to February 7th, 2022, 110 centres from 23 countries in 4 continents have been involved. Fifty-four of these have already obtained the approval from their local Ethics Committees. Currently, the platform counts 290 users (111 Principal Investigators, 175 Site Investigators, 2 Lead Investigators, and 2 data managers). The Registry collects baseline and follow-up data using 5993 fields organised into 16 instruments, including patient’s demographics, history, clinical manifestations and symptoms, trigger/risk factors, therapies and healthcare access. The development of the AIDA International Registry for BD patients will facilitate the collection of standardised data leading to real-world evidence, enabling international multicentre collaborative research through data sharing, international consultation, dissemination of knowledge, inclusion of patients and families, and ultimately optimisation of scientific efforts and implementation of standardised care.Trial registration NCT05200715 in 21/01/2022.

Objectives: In this present study, we aimed to perform the reliability and validity analyses of the Turkish Form of Short Form-12 version 2 (SF-12v2) in different groups of patients. Patients and methods: After the permission for the validation study of the Turkish Form, Optum provided the authors the validly translated Turkish Form of SF-12v2. The study was carried out in rheumatological, psychiatric, and surgical wards of Manisa Celal Bayar University Hafsa Sultan Training and Research Center between September 2019 and June 2020. Taking possible dropouts into consideration, a total of 136 patients (67 males, 69 females; mean age: 43.5±14.4 years; range 19 to 82 years) constituted the study group. In addition to SF-12v2, for concurrent validity, Nottingham Health Profile (NHP) was used as the comparator instrument. Results: In internal consistency, for the physical component summary score, the Cronbach alpha coefficient was 0.80, and item-total score correlation coefficients were between 0.32 and 0.73. The Cronbach alpha coefficient of the mental component summary score was found to be 0.88 where the item-total correlation coefficients varied between 0.60 and 0.78. Exploratory factor analysis revealed a two-factor solution, representing mental and physical components. For criterion validity, convergent and discriminant validity analyses were performed using NHP with SF-12v2, and domains of SF-12v2 correlated well with the domains of NHP accordingly. In criterion validity, the psychiatric group had the lowest mean score in mental health, vitality, social functioning and role difficulties due to emotional problems, whereas the surgical group had the lowest mean score in bodily pain, role difficulties due to physical problems, and physical functioning. Conclusion:Our study results show that the Turkish form of SF-12v2 is valid and reliable both in clinical practice and clinical trials.