Marc Schmalzing

Background: Axial spondyloarthritis (axSpA) is a chronic inflammatory rheumatic disease affecting the axial skeleton that requires regular physical exercise in addition to pharmacotherapy. However, a significant proportion of patients do not exercise frequently enough. Axia is a digital therapeutic (DTx) that offers a solution by providing a disease-specific and patient-tailored home-based exercise program for patients with axSpA. The app combines structured exercise with patient education and disease management features and incorporates gamification and behavioral conditioning to promote user engagement. However, it has remained unclear how Axia is perceived and evaluated by patients. Objective: This study aimed to describe app quality, user experience, and willingness to recommend the app to others after 12 weeks of use by applying standardized questionnaires. Methods: This single-center study was conducted at an outpatient tertiary clinic. After 12 weeks of ad libitum app use, 27 participants with axSpA completed paper-based assessments of the user version of the Mobile App Rating Scale (uMARS; range 1-5) and the net promoter score (NPS; range −100% to +100%). Results: The mean overall uMARS app quality score was 4.44 (SD 0.43). Functionality received the highest mean score (4.54, SD 0.53), whereas information received the lowest (4.34, SD 0.49). The mean NPS was 9.52 (SD 0.75), corresponding to an overall NPS of 93%. The uMARS overall quality score correlated strongly with the NPS (ρ=0.57; P=.002). Except for physical therapy at baseline, no other baseline characteristic or therapeutic effect correlated with either the uMARS or NPS. Conclusions: Axia was perceived as a user-friendly DTx after 12 weeks of use. The findings support the acceptability of disease-specific DTx for patients with axSpA and the potential integration of DTx into routine care.

Objective: Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are considered as manifestations of a shared disease spectrum (GCA-PMR spectrum disease, GPSD). Data on orbital involvement in isolated PMR and across the spectrum remain limited. This study evaluated inflammatory intraorbital MRI findings across the GCA-PMR spectrum. Methods: This retrospective observational study included patients with isolated PMR and combined GCA-PMR (presence of both clinical diagnoses and fulfilling classification criteria of GCA plus PMR). Patients with GCA and no signs and symptoms of PMR were not included. Contrast-enhanced MRI of the orbits was assessed for inflammatory findings, including optic nerve sheath enhancement and ophthalmic artery vessel wall enhancement. Imaging findings were correlated with clinically reported orbital symptoms. Results: 73 isolated PMR patients had evaluable MRI of the orbits. Nine (12.3%) showed intraorbital inflammatory changes: bilateral optic nerve sheath enhancement (n = 8) and bilateral ophthalmic artery enhancement (n = 4). None had clinical or imaging evidence of large-vessel vasculitis. Two patients reported acute visual symptoms, both with corresponding orbital MRI findings. 63 GCA-PMR patients had evaluable MRI of the orbits. Intraorbital findings were present in 20 (31.7%), most commonly bilateral optic nerve sheath (n = 16) and bilateral ophthalmic artery enhancement (n = 11). Fourteen patients reported acute visual symptoms; ten had corresponding MRI findings, while four were MRI-negative. Conclusion: Inflammatory orbital changes occur in patients with GCA-PMR and isolated PMR. Orbital and ophthalmic artery involvement may arise even without overt GCA, supporting a large overlap between GCA and PMR highlighting the potential value of orbital MRI.

Objective: Immune checkpoint inhibitors (ICIs) induce a broad range of immune‐related adverse events (irAEs), including rheumatic manifestations. Evidence from dedicated rheumatology cohorts on the clinical spectrum, severity, treatment, and outcomes of rheumatic irAEs (rh‐irAEs) remains limited. We aimed to describe clinical phenotypes, treatment, and outcomes of rh‐irAEs in a national registry. Methods: The German ERIN Registry (Registry for the Documentation of Rheumatic Immunotherapy‐Related Adverse Events) is a multicenter observational registry of adults receiving ICIs. Rh‐irAEs were classified using a rheumatology‐oriented phenotype‐based framework. Variables included demographics, oncological characteristics, treatments, and outcomes. Severity was graded by Common Terminology Criteria for Adverse Events version 5.0. Univariate analyses identified predictors of second‐line systemic therapy. Results: As of January 1, 2026, 60 patients (56.7% men; median age 66 years) were included. Predominant malignancy was melanoma (40.0%). The leading phenotype was rheumatoid arthritis‐like polyarthritis (41.7%), followed by polymyalgia rheumatica‐like (18.3%) and peripheral spondyloarthritis‐like syndrome (11.7%). Axial spondyloarthritis (axSpA)–like syndrome occurred in younger patients. Rh‐irAEs were moderate or severe in 90%; 23.3% required hospitalization and 38.3% led to permanent ICI discontinuation. Systemic glucocorticoids (GCs) were used in 85.0%; 58.3% were GC‐refractory and 53.3% required second‐line immunomodulatory therapy. Complete remission was achieved in 33.3% and partial remission in 51.7%. Oncological progression occurred in 15.0% overall and in 28.1% of patients requiring second‐line therapy. Male sex was the strongest predictor of second‐line systemic therapy. Conclusion: Rh‐irAEs in a rheumatologists‐referred population were more severe than previously described, with frequent ICI discontinuation and escalation beyond GCs. Male sex was associated with treatment escalation. An underrecognized axSpA‐like phenotype occurred in younger patients, warranting further study. image

Background: Lupus erythematosus (LE) is a chronic autoimmune disease that significantly impacts patients’ quality of life. Photosensitivity is a key impairment that severely limits the quality of life, especially in cutaneous lupus erythematosus (CLE), where exposure to sunlight can lead to rashes, exacerbations, and pain. In systemic lupus erythematosus (SLE), other manifestations such as joint pain, fatigue, and organ damage may contribute to decreased physical function and emotional distress. Mobile health apps (MHA) offer potential support for comprehensive disease management for the symptoms mentioned above. However, there is a lack of systematic analysis of available lupus management apps. Objective: This study aims to systematically identify publicly available German or English MHA for lupus management as well as to assess their quality by surveying both patients and physicians. Methods: A systematic search and assessment of German or English mobile apps for patients with lupus, available in the Google Play Store and Apple App Store, was conducted independently by two reviewers. The two apps that met all relevant criteria were then reviewed independently by seven physicians using the German Mobile Application Rating Scale (MARS) and the System Usability Scale (SUS). Subsequently, they were reviewed by five patients (three with SLE and two with CLE), using the user version of MARS (uMARS) and SUS. Additionally, the Affinity for Technology Interaction (ATI) scale was collected from both patients and physicians to evaluate the technical affinity in both groups. Results: In total, 29 apps were available on the Apple Store and 26 on the Google Store, with 18 apps being present and downloadable on both platforms. Of the 18 apps, 16 were excluded because they did not meet the inclusion and exclusion criteria. Only two apps, Lupus Log and Lupus Minder met all the required criteria and were included in the study. The mean MARS scores varied from 2.61/5 to 4.17/5 and mean SUS from 17.5/100 to 100/100 between physicians. The app with the highest mean overall MARS score was Lupus Log , which was rated with 3.91/5 on average by the physicians. Patients evaluated the app with a comparably mean uMARS score (3.95/5). Technical affinity, objectified by ATI, was higher in patients than physicians (3.9 vs 3.68). Conclusions: Systematic identification and evaluation showed high-quality apps for patient-centered lupus MHA as indicated by MARS and uMARS scores greater than 3 for both Lupus Log and Lupus Minder .

Objectives: This study aimed to analyse survival in SS-associated pulmonary hypertension (SSc-PH) patients with or without interstitial lung disease (ILD) stratified by PH monotherapy and PH combination therapy, respectively. Methods: Among the 6003 patients registered with the German Network for Systemic Sclerosis (DNSS) registry, SSc patients with incident PH diagnosed after registry entry were included. Kaplan-Meier analysis was performed to estimate the overall survival, and the log-rank test was used to assess differences between groups stratified by the presence of ILD or PH treatment. Results: Of, the 384 patients who fulfilled the inclusion criteria, 271 had concomitant ILD (70.6%). The mean follow-up period was 8.69 ± 4.94 years. Forced vital capacity (FVC) and diffusing capacity of the lungs for carbon monoxide (DLCO) numerically decreased in PH patients with and without ILD and were significantly lower in patients with ILD (mean value of minimal FVC during follow-up: 70.79 ± 24.36%). There was no significant difference in the distribution of PH therapy categories (mono, dual, or triple therapy) between PH patients with and without ILD involvement (P = 0.865). No survival difference was observed between the monotherapy and combination therapy groups (P = 0.197). Subgroup analysis indicated that varying the treatment strategies did not significantly impact the survival outcomes of PH patients, whether or not ILD was present. Conclusion: SSc patients with ILD and incident PH are often treated with PH combination therapy in Germany. This treatment strategy seems feasible and does not worsen survival in PH patients, provided that ILD is not severe.

Objectives: To assess the prevalence of aortitis and aortic dilation at diagnosis, estimate the progression of aortic diameter over time, and identify predictors of incident aortic dilation development in patients with newly diagnosed GCA. Methods: We conducted a retrospective cohort study involving 157 patients with new-onset GCA from two European centres. All patients underwent baseline thoracic aortic imaging within 6 months of diagnosis and at least one follow-up imaging ≥6 months later. Outcomes included baseline aortic diameter, aortic expansion (cm2/year), and incident aortic dilation. Multivariable regression models were adjusted for sex and age. Results: Aortitis was present in 60.4% of patients at baseline. Baseline aortic dilation was identified in 19.6% of patients, predominantly in the mid-ascending aorta. Aortitis was associated with larger aortic diameters and higher odds of baseline aortic dilation (adjusted odds ratio 2.3; 95% CI: 1.0, 5.1). Over a median follow-up of 30 months, incident aortic dilation developed in 9.8% of patients. Baseline aortic diameter was the strongest predictor of aortic expansion (β = 0.088; P = 0.006) and incident aortic dilation (adjusted hazard ratio 3.9; 95% CI: 2.0, 7.3). Conclusion: Aortic involvement is common at the time of GCA diagnosis, and baseline aortic diameter is the strongest predictor of future dilation in our cohort. These findings highlight the importance of early imaging assessment and longitudinal monitoring of aortic dimensions in patients with newly diagnosed GCA.

BACKGROUND Axial spondyloarthritis (axSpA) is a chronic inflammatory rheumatic disease affecting the axial skeleton that requires regular physical exercise in addition to pharmacotherapy. However, a significant proportion of patients do not exercise frequently enough. Axia is a digital therapeutic (DTx) that offers a solution by providing a disease-specific and patient-tailored home-based exercise program for patients with axSpA. The app combines structured exercise with patient education and disease management features and incorporates gamification and behavioral conditioning to promote user engagement. However, it has remained unclear how Axia is perceived and evaluated by patients. OBJECTIVE This study aimed to describe app quality, user experience, and willingness to recommend the app to others after 12 weeks of use by applying standardized questionnaires. METHODS This single-center study was conducted at an outpatient tertiary clinic. After 12 weeks of ad libitum app use, 27 participants with axSpA completed paper-based assessments of the user version of the Mobile App Rating Scale (uMARS; range 1-5) and the net promoter score (NPS; range −100% to +100%). RESULTS The mean overall uMARS app quality score was 4.44 (SD 0.43). Functionality received the highest mean score (4.54, SD 0.53), whereas information received the lowest (4.34, SD 0.49). The mean NPS was 9.52 (SD 0.75), corresponding to an overall NPS of 93%. The uMARS overall quality score correlated strongly with the NPS (ρ=0.57; P=.002). Except for physical therapy at baseline, no other baseline characteristic or therapeutic effect correlated with either the uMARS or NPS. CONCLUSIONS Axia was perceived as a user-friendly DTx after 12 weeks of use. The findings support the acceptability of disease-specific DTx for patients with axSpA and the potential integration of DTx into routine care.

The introduction of biosimilars into global markets has increased utilisation and reduced costs of biological therapies. However, the uptake varies by country because of differences in biosimilar knowledge and concerns about their safety and efficacy. This review examines clinical and real-world data on the effects of switching between reference and biosimilar (SDZ-ETN, SB4, LBEC0101, YLB113) etanercept on treatment efficacy and safety in patients with inflammatory rheumatic and musculoskeletal diseases. To date, all controlled clinical trials and real-world studies indicate that switching between reference and biosimilar etanercept does not affect treatment efficacy and safety. These findings support broader biosimilar adoption to improve patient access and reduce healthcare costs. However, published data on multiple biosimilar switches and patient-reported outcomes remain limited, warranting further research efforts in these areas.