Objective. Some features of Behçet’s syndrome (BS) tend to go together. We aimed to explore the association and timing of various vascular events in both the venous and the arterial vascular tree. Methods. We conducted a chart survey on the type and time of vascular involvement of BS. The cross-relationships of involvement were assessed by phi correlation coefficients. Multiple correspondence analysis was used to identify patterns of vascular involvement. The risk of vascular recurrence was also estimated. Results. We identified 882 patients with vascular involvement among 5970 BS patients (14.7%). Deep vein thrombosis (DVT), almost always in the legs, was the most frequent single vascular event (592/882; 67.1%). The cumulative risk of a recurrent vascular event was 38.4% at 5 years. Patients with extrapulmonary artery involvement (EPAI) were significantly older than those with venous and pulmonary artery involvement (PAI). There were significant correlations between dural sinus thrombosis (DST) and PAI, Budd–Chiari syndrome (BCS) and inferior vena cava syndrome (IVCS) and between IVCS and superior vena cava syndrome (SVCS). Multiple correspondence analysis further indicated clustering of PAI, DST, BCS, IVCS and SVCS. However, EPAI and DVT clustered separately from forms of vascular disease, the separate clustering of the DVT being attributed to its propensity to occur solo. Conclusion. The most common type of vascular involvement in BS is solo DVT, almost always occurring in the legs. Various forms of venous disease in BS segregate together and PAI is included in this group. EPAI segregates separately.

Background: Mobile health (mHealth) defines the support and practice of health care using mobile devices and promises to improve the current treatment situation of patients with chronic diseases. Little is known about mHealth usage and digital preferences of patients with chronic rheumatic diseases. Objective: The aim of the study was to explore mHealth usage, preferences, barriers, and eHealth literacy reported by German patients with rheumatic diseases. Methods: Between December 2018 and January 2019, patients (recruited consecutively) with rheumatoid arthritis, psoriatic arthritis, and axial spondyloarthritis were asked to complete a paper-based survey. The survey included questions on sociodemographics, health characteristics, mHealth usage, eHealth literacy using eHealth Literacy Scale (eHEALS), and communication and information preferences. Results: Of the patients (N=193) who completed the survey, 176 patients (91.2%) regularly used a smartphone, and 89 patients (46.1%) regularly used social media. Patients (132/193, 68.4%) believed that using medical apps could be beneficial for their own health. Out of 193 patients, only 8 (4.1%) were currently using medical apps, and only 22 patients (11.4%) stated that they knew useful rheumatology websites/mobile apps. Nearly all patients (188/193, 97.4%) would agree to share their mobile app data for research purposes. Out of 193 patients, 129 (66.8%) would regularly enter data using an app, and 146 patients (75.6%) would welcome official mobile app recommendations from the national rheumatology society. The preferred duration for data entry was not more than 15 minutes (110/193, 57.0%), and the preferred frequency was weekly (59/193, 30.6%). Medication information was the most desired app feature (150/193, 77.7%). Internet was the most frequently utilized source of information (144/193, 74.6%). The mean eHealth literacy was low (26.3/40) and was positively correlated with younger age, app use, belief in benefit of using medical apps, and current internet use to obtain health information. Conclusions: Patients with rheumatic diseases are very eager to use mHealth technologies to better understand their chronic diseases. This open-mindedness is counterbalanced by low mHealth usage and competency. Personalized mHealth solutions and clear implementation recommendations are needed to realize the full potential of mHealth in rheumatology.

Background: Chronic rheumatic diseases need long-term treatment and professional supervision. Mobile apps promise to improve the lives of patients and physicians. In routine practice, however, rheumatology apps are largely unknown and little is known about their quality and safety. Objective: The aim of this study was to provide an overview of mobile rheumatology apps currently available in German app stores, evaluate app quality using the Mobile Application Rating Scale (MARS), and compile brief, ready-to-use descriptions for patients and rheumatologists. Methods: The German App Store and Google Play store were systematically searched to identify German rheumatology mobile apps for patient and physician use. MARS was used to independently assess app quality by 8 physicians, 4 using Android and 4 using iOS smartphones. Apps were randomly assigned so that 4 apps were rated by all raters and the remaining apps were rated by two Android and two iOS users. Furthermore, brief app descriptions including app developers, app categories, and features were compiled to inform potential users and developers. Results: In total, 128 and 63 apps were identified in the German Google Play and App Store, respectively. After removing duplicates and only including apps that were available in both stores, 28 apps remained. Sixteen apps met the inclusion criteria, which were (1) German language, (2) availability in both app stores, (3) targeting patients or physicians as users, and (4) clearly including rheumatology or rheumatic diseases as subject matter. Exclusion criteria were (1) congress apps and (2) company apps with advertisements. Nine apps addressed patients and 7 apps addressed physicians. No clinical studies to support the effectiveness and safety of apps could be found. Pharmaceutical companies were the main developers of two apps. Rheuma Auszeit was the only app mainly developed by a patient organization. This app had the highest overall MARS score (4.19/5). Three out of 9 patient apps featured validated questionnaires. The median overall MARS score was 3.85/5, ranging from 2.81/5 to 4.19/5. One patient-targeted and one physician-targeted app had MARS scores >4/5. No significant rater gender or platform (iOS/Android) differences could be observed. The overall correlation between app store ratings and MARS scores was low and inconsistent between platforms. Conclusions: To our knowledge, this is the first study that systematically identified and evaluated mobile apps in rheumatology for patients and physicians available in German app stores. We found a lack of supporting clinical studies, use of validated questionnaires, and involvement of academic developers. Overall app quality was heterogeneous. To create high-quality apps, closer cooperation led by patients and physicians is vital.

Short-chain fatty acids are microbial metabolites that have been shown to be key regulators of the gut–joint axis in animal models. In humans, microbial dysbiosis was observed in rheumatoid arthritis (RA) patients as well as in those at-risk to develop RA, and is thought to be an environmental trigger for the development of clinical disease. At the same time, diet has a proven impact on maintaining intestinal microbial homeostasis. Given this association, we performed a feasibility study in RA patients using high-fiber dietary supplementation with the objective to restore microbial homeostasis and promote the secretion of beneficial immunomodulatory microbial metabolites. RA patients (n = 36) under routine care received daily high-fiber bars or cereals for 28 days. Clinical assessments and laboratory analysis of immune parameters in blood and stool samples from RA patients were done before and after the high-fiber dietary supplementation. We observed an increase in circulating regulatory T cell numbers, favorable Th1/Th17 ratios, as well as decreased markers of bone erosion in RA patients after 28 days of dietary intervention. Furthermore, patient-related outcomes of RA improved. Based on these results, we conclude that controlled clinical studies of high-fiber dietary interventions could be a viable approach to supplement or complement current pharmacological treatment strategies.

Objective: Behçet's syndrome is characterized by clusters of disease expression, one of which is the coexistence of acne and arthritis. The aim of this study was to test the hypothesis that enthesopathy is increased in this cluster, having features reminiscent of acne‐associated arthritis.Methods: The study group comprised 35 patients with Behçet's syndrome who had acne and arthritis (BS‐AA), 38 patients with Behçet's syndrome who did not have arthritis (BS‐WA), 37 patients with ankylosing spondylitis (AS), 25 patients with rheumatoid arthritis (RA), and 25 healthy control subjects. Five entheseal sites (the superior and inferior poles of the patella, the tibial tuberosity, and the superior and inferior poles of the calcaneus) on both lower extremities were examined by 2 independent observers, using ultrasonography. A previously validated composite score was calculated for each patient. The numbers of entheseal sites giving a positive power Doppler signal in each group were also compared.Results: Patients with AS had the highest enthesopathy score (mean ± SD 4.1 ± 2.4; F [4df] = 8.43, P < 0.001) followed by BS‐AA patients (3.0 ± 1.9; F [3df] = 3.63, P = 0.015). The mean ± SD enthesopathy scores among the remaining 3 groups were similar (for BS‐WA, 1.8 ± 1.4; for RA, 2.2 ± 1.6; for healthy controls, 2.0 ± 1.5 [F (2df) = 0.65, P = 0.53]). Power Doppler scores were highest for the BS‐AA group (mean ± SD 3.0 ± 1.5; F [4df] = 15.54, P < 0.001) followed by the AS group (2.7 ± 1.8; F [3df] = 14.38, P < 0.001), the RA group (2.6 ± 1.8; F [2df] = 13.88, P < 0.001), the BS‐WA group (1.2 ± 1.3; F [1df] = 4.48, P = 0.038), and the control group (0.5 ± 1.1). There was 87% agreement between the 2 observers (κ = 0.55, intraclass correlation coefficient 0.71).Conclusion: The increased presence of enthesopathy among BS‐AA patients compared with that among BS‐WA patients further supports the hypothesis that patients with Behçet's syndrome who also have arthritis and acne form a distinct cluster.

Chimeric antigen receptor (CAR)-T cells are considered a powerful therapeutic tool to reset the immune system in patients with autoimmune diseases. Innovative trial designs are needed to allow feasible testing of the safety and efficacy of CAR-T cells in clinical studies. CASTLE (CAR-T cells in systemic B cell mediated autoimmune disease) is a phase 1/2a two-stage optimal design basket study that investigated the safety and efficacy of zorpocabtagene autoleucel (Zorpo-cel, also known as MB-CART19.1), an autologous CD19 CAR-T cell product, in patients with treatment-resistant systemic lupus erythematosus (SLE), systemic sclerosis (SSc) and idiopathic inflammatory myopathies (IIM). The primary safety outcome was the rate of cytokine release syndrome (CRS) and immune effector cell-associated neurotoxicity syndrome (ICANS). The secondary clinical efficacy outcomes were remission of SLE according to DORIS criteria, no progression of interstitial lung disease in SSc and American College of Rheumatology (ACR) major/moderate response in IIM after 24 weeks. A total of 24 patients were enrolled (10 with SLE, 9 with SSc and 5 with IIM), all receiving a single infusion of Zorpo-cel after stopping immunosuppressive treatments and receiving standard lymphodepletion with cyclophosphamide and fludarabine. Primary and secondary endpoints of CASTLE were met. Regarding safety, no CRS higher than grade 2 and no ICANS occurred. Regarding efficacy, 22 of the 24 patients achieved predefined efficacy endpoints, with 9 out of 10 patients with SLE reaching DORIS remission, 9 out of 9 patients with SSc showing no disease progression, and 4 out of 5 patients with IIM reaching ACR major/moderate response. Furthermore, all patients remained free of glucocorticoids and any other immunosuppressive treatment over the entire observation period of 24 weeks. CASTLE suggests the feasibility, safety and efficacy of Zorpo-cel in three different autoimmune diseases and paves the way for conducting a pivotal study. ClinicalTrials.gov identifier: NCT06347718 , EudraCT identifier: 2022-001366-35 .