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Background The increased availability of myositis autoantibodies represents new possibilities and challenges in clinical practice (Lundberg IE, Tjärnlund A, Bottai M, Werth VP, Pilkington C, de Visser M, et al. 2017 European League Against Rheumatism/American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups. Ann Rheum Dis. 2017;76:1955–64. https://doi.org/10.1136/annrheumdis-2017-211468 .). The aim of this study was to perform a retrospective data analysis of patient cases with positive myositis autoantibodies to analyse their significance in routine rheumatology practice. Methods A monocentric analysis of all the orders used to determine myositis autoantibodies from July 2019 to May 2022 in the Department of Rheumatology, Krankenhaus Porz am Rhein, Cologne, Germany, was carried out. Results In the defined time interval, a total of 71,597 laboratory values for the antibodies mentioned above were obtained. A total of 238 different positive autoantibodies ​​were detected in 209 patients. Idiopathic inflammatory myopathy was diagnosed in 37 patients (18%), and inflammatory rheumatic diseases other than idiopathic inflammatory myopathy were diagnosed in 90 patients (43%). No inflammatory rheumatic disease was diagnosed in 82 patients (39%). General clusters of clinical manifestations were observed. Conclusions In our cohort, we were able to show that a relevant proportion of patients with positive myositis antibodies did not have idiopathic inflammatory myopathies or inflammatory rheumatic diseases. This finding indicates the importance of myositis autoantibodies in this group of patients. However, further studies on the course of symptoms and examination results in patients without inflammatory rheumatic diseases and with positive myositis antibodies are necessary.

Objective Patient involvement in scientific research is becoming increasingly important to ensure a patient-centered approach to medicine. The objective of this study is twofold: firstly, to ascertain patients’ perspectives on adherence to therapy and secondly, to integrate this information into a systematic review (SR) on interventions to improve adherence in chronic obstructive pulmonary disease (COPD). Methods In parallel with the SR, two focus group interviews with a COPD self-help group were conducted using semi-structured interview guidelines. The interviews were analyzed using computer-assisted qualitative content analysis according to Kuckartz with the aim of complementing the results of the systematic literature review and to develop propositions for further scientific use. Results The first focus group interview comprising 321 codes included 14 (mean age 67.7 ± 6.8 years; 71.4% female) and the second interview comprising 139 codes included 10 (mean age 68,5 ± 8,2 years; 50% female) patients. Ten categories of the content analysis informed the logic models and the applicability analysis of the SR and six propositions representing the patient’s perspective were developed for further scientific use: Main themes were (I) Enhancement of patient’s self-efficacy (II) Access to trusted medical information (III) Patient-physician relationship (IV) Measures to improve adherence (V) Sociocultural/medical environment (VI) Methods to measure adherence. Conclusion The inclusion of the patient perspective in an SR can be successfully achieved by conducting focus group interviews. Multimodal measures aimed at enhancing patient’s self-efficacy helping them to achieve individual goals reinforces adherence to therapy from a patient’s perspective.

Background Non-invasive ventilation (NIV) is a well-established treatment for chronic hypercapnic respiratory failure (CHRF). While studies have demonstrated benefits for mortality, hospitalization rates, and health related quality of life (HRQL), evidence is particularly sparse regarding HRQL determinants in the older population. Methods In a prospective, monocentric observational study, HRQL was assessed using the established Severe Respiratory Insufficiency Questionnaire (SRI). The study was prospectively registered in the German Clinical Trials Register on 17 June 2015 under the registration number DRKS00008759. Patients were categorized into two age-based groups: older patients (≥ 65 years) and younger patients (< 65 years). Multiple linear regression analyses were used to analyze factors on HRQL, including SRI scores, anemia, autonomy impairment, exacerbation history and other factors. Results 237 Patients with COPD with CHRF receiving NIV therapy were included. The mean SRI summary score was 49.9 ± 16.8. with 23.2% ( N  = 55) suffering from anemia and 36.7% ( N  = 87) experiencing  ≥  2 exacerbations annually. Autonomy impairment was observed in 49.4% ( N  = 117) of patients. The updated Charlson Comorbidity Index (uCCI) was 2.2 ± 1.86. No significant differences were found in SRI Summary Scale scores between age groups ( p  = 0.581), but notable disparities were present in the uCCI ( p  = 0.014). Multiple regression analysis revealed a negative association of exacerbation history (Young group: -9.2; 95% CI = -14.8/ -3.55 vs. Older group: -6.17; 95% CI = -11.91/ -0.43) and level of autonomy impairment (e.g. Level of Care 2 Young group: -13.91; 95% CI = -21.4/ -6.43 vs. Older group: -14.94; 95% CI = -22.64/ -7.24) on SRI scores with age-related differences. Anemia only had a negative association on the SRI scores in younger patients with COPD (Young group: -7.9; 95% CI = -14.0/ -1.75 vs. Older group: -1.78; 95% CI = -9.21/ 5.65). Discussion Frequent exacerbations and a higher level of autonomy impairment had a negative association on HRQL across all ages. However only higher levels of impairment (≥ 2) have a detrimental impact on older patients. Anemia was a negative HRQL factor in younger patients, where it was more prevalent. Overall, HRQL was found to be comparably favorable in both older and younger patients, despite age-specific differences in influencing factors. Registration of the clinical trial The study from which the data were analyzed was prospectively registered in the German Clinical Trials Register (DRKS00008759) on June 17, 2015.

Health-related-quality-of-life is frequently reduced following intensive care treatment. Unwarranted or unwanted therapeutic interventions should be avoided at all costs. Since COPD patients are often faced with difficult decisions, an assessment was made of their desire for disease education. Our aim was to identify patients understanding of their disease and what their attitudes are towards different treatment options and whether this correlates to demographic factors. The COPD-Assessment-Test (CAT) was used to measure subjective disease burden. The COPD-Questionnaire (COPD-Q) was used to assess subjects' own knowledge of their disease. In addition, a specifically designed questionnaire was used to assess patient's subjective level of desire to be educated on COPD-specific topics. A multiple linear regression analysis was performed to identify the demographic factors associated with a greater desire for disease-specific information. 127 patients (67.2±8.8 years) were prospectively enrolled. Mean CAT score was 21.3±8.9 (95% CI:1-40). The desire for medical consultation was highly individual. In terms of vaccination, 31.5% of patients wished for more information while 34.6% wished for less. This also held true for information on long-term pharmacological therapy (29.1% vs 30.7%, respectively). Information on behaviour in case of emergencies as well as smoking cessation were wished for 38% and 42% of patients, respectively. Results of the COPD-Q showed that subjects were well-informed about specific topics (vaccination, etiology, emergency-inhaler) and less informed about long-term pharmacotherapy. In linear regression analyses, age (p=0.086), sex (p=0.906), education (p=0.833), health literacy (p=0.336) and burden of disease (p=0.296) did not influence patients´ desire for disease-specific information. Based on our cohort, COPD patients wish for more medical information related to behaviour in emergency situations and smoking cessation. The desire for education on disease-specific topics did not naturally correlate with demographic characteristics. The provision of medical information to patients remains a highly individualized and essential part of patient care. German Clinical Trials Registry (DRKS00022109).

Non-invasive ventilation (NIV) is vital for managing chronic hypercapnic respiratory failure in COPD patients, yet the impact of handling issues like mask compliance triggering hospitalisations is often underestimated. A prospective, monocentric observational study was performed in COPD patients hospitalized for acute exacerbation with established home NIV therapy. Various questionnaires (CAT, SRI, BORG) and blood gas analysis were used to determine the severity and cause of respiratory insufficiency. 59 patients (mean age 66.57 years ± 9.42, mean BMI 26.99 ± 8.63) were included. 54.24% were female (n=32). The overall cohort had a mean exacerbation rate of 2.24 ± 1.48 within the last 12 months prior to admission. Patients were divided into 4 sub cohorts based on their exacerbation trigger: infection (n=25), handling problem (n=12), non-infection (n=8), and an overlap cohort with evidence of both handling problem and non-handling problem (n=14). Significant differences exist when comparing exacerbation rate (handling-issue cohort: 2.58 ± 1.68 vs infection cohort: 1.76 ± 1.13, p=0.043), total hospital stay (handling-issue cohort: 9.25 ± 5.94 days vs infection cohort: 12.96 ± 5.76 days, p=0.039). There was no significant difference in health-related quality of life measured by the SRI (Summary Score 40.6±12.3 vs 46.8±14.2; p=0.103). In our study, we were able to show that handling problems are associated with frequent exacerbations, cause long hospitalisation periods and are associated with a reduced aspects of quality of life. Patient education and training should therefore play a key role in the treatment of patients.

Introduction: Rheumatoid arthritis (RA) is known to affect the musculoskeletal system and, consequently, may lead to sarcopenia, but the role of respiratory muscle involvement in RA patients is unclear. Methods: This prospective, exploratory, single-center, matched-pair analysis study was designed to compare respiratory muscle strength and handgrip strength in RA patients and controls. Results: RA patients with low disease activity as estimated from the Disease Activity Score 28 (2.3 ± 1.2) and without signs of interstitial lung disease (n = 36, 72% female, 28% smoker, mean age 48 + 15 years, mean forced vital capacity 3.9 ± 1.0 L, 98% ± 11% predicted) and control subjects (n = 36, 72% female, 11% smoker, mean age 48 + 14 years, mean forced vital capacity 4.1 ± 1.1 L, 98% ± 16% predicted) were well balanced. Maximal inspiratory mouth pressure (PImax, primary endpoint) tended to be lower in RA patients, but this was statistically not significant (−0.9 kPa; 95%CI = −2.11/0.32). However, RA patients more frequently had PImax values below the lower limit of normal (OR 1.74 kPa; 95% CI 0.65/4.77). RA patients had lower handgrip strength (−5.97 kg; 95%CI = −9.43/−2.50). In addition, PImax was correlated to handgrip strength both in RA patients (R = 0.51, p = 0.0017) and controls (R = 0.48, p = 0.0029) and to the 6-minute walking distance (RA-patients: R = 0.30, p = 0.075; controls: R = 0.52, p = 0.0012). Conclusions: Even though the primary endpoint has not been reached, an impairment of respiratory muscle strength in RA cannot be excluded at least in a subset of patients. Further studies also involving RA patients with more disease activity are needed.

Background: Pulmonary involvement is the most common prognosis-related organ involvement in idiopathic inflammatory myopathy (IIM). Owing to the large number of antibodies, the evidence for lung involvement and rare antibodies is limited. In everyday clinical practice, the interpretation of positive myositis antibodies represents a challenge. Methods: This study is a retrospective monocentric analysis. The data collection regarding positive myositis antibodies and possible pulmonary involvement was carried out from July 2019 to May 2022. Data analysis revealed positive results for one of the following antibodies: EJ, PL7, OJ, PL12, Mi-2α, TIF1γ, MDA5, SAE, NXP2, SRP, Ku, PM-Scl100 and PM-Scl75. In our analysis, patients with IIM, patients with inflammatory rheumatic disease other than IIM and patients without inflammatory rheumatic disease are described. The results of high-resolution computed tomography (HRCT), pulmonary function tests, echocardiographic examinations and their associated clinical findings are examined. Results: In the entire cohort, 209 patients with positive myositis antibodies were detected. In total, 22 (10.5%) patients had interstitial lung disease (ILD) patterns on HRCT. In the subgroup of patients with IIM, a significantly higher proportion of patients with lung involvement (n = 13, 35.1%) was found than in the group with other inflammatory rheumatic diseases (IRDs) (n = 6, 6.7%) or in the group without IRDs (n = 3, 3.7%). When the antibody groups were considered, the PL12-positive patients had the largest proportion of ILD (42%), followed by the MDA5-positive patients (40%). Conclusions: In patients with IIM, myositis antibodies are highly relevant for assessing the risk of lung involvement. In groups with other IRD or without IRD, antibody detection does not represent this high relevance for lung involvement. A differentiated assessment of the various MSAs or MAAs detected, as well as clinical parameters, allows for further important risk assessment for prognosis-relevant lung involvement.

Introduction VEXAS syndrome, characterized by a UBA1 gene mutation, is a rare and severe systemic inflammatory disease predominantly affecting men. Since its initial description in 2020, it has been noted for its broad clinical phenotype and frequent misdiagnosis. Case Presentation A 76-year-old Caucasian male patient diagnosed with VEXAS syndrome is presented in this case report. He presented with typical symptoms including pulmonary manifestations (infiltrates and effusions), systemic inflammation, and haematological abnormalities. The diagnosis was challenging due to the disease's heterogeneous presentation, often resembling autoimmune or haematological diseases. This patient’s case featured ground-glass opacities and pleural effusions, underlining the significant pulmonary involvement seen in 50–67% of VEXAS patients. His condition was further complicated by recurrent fever and systemic inflammation affecting multiple organs. Conclusion VEXAS syndrome demands an aggressive treatment approach due to its high mortality rate and refractory nature. This case underscores the importance of including VEXAS syndrome in differential diagnoses, particularly for patients with systemic inflammation and pulmonary symptoms, and calls for multidisciplinary management and extensive research to understand its full range of clinical phenotypes. Established facts and novel insights VEXAS syndrome is a rare systemic inflammatory disease with UBA1 gene mutation. VEXAS syndrome involves various UBA 1 gene mutations, including the p.splice c.118-1G > C. It mainly affects men, often misdiagnosed due to its broad clinical phenotype. Novel insights Significant pulmonary involvement in VEXAS, including ground-glass opacities and pleural effusions. Patients with the same mutation exhibit a broad range of disease phenotypes A specific UBA1 mutation as the p.splice c.118-1G > C cannot be directly linked to a distinct disease phenotype Need for aggressive treatment strategies targeting the mutated clone and cytokine storms

Constant-minute-volume and constant-bolus devices serve as two different means of portable oxygen conservation. A prospective randomised crossover study was conducted in COPD GOLD IV patients to investigate the effect of these two devices on dyspnea, oxygenation and 6-minute walking test (6MWT) distance. The primary endpoint was the final operating level required (operating level range 1-5 for both devices) by either device to meet the success criteria for mobile oxygen therapy, as outlined in the British Thoracic Society guidelines (SpO ≥90% throughout 6MWT; ≥10% increase in walking distance from baseline; improvement in BORG of at least 1 point from baseline). Twenty-five patients were enrolled in the study and randomly assigned to one of two sequences involving the use of each type of portable oxygen conservation device. 14 female, 67.9 years (±7.8); FEV1: 27.3%pred. (±8.4); PaO at rest without oxygen: 50.3mmHg (±5.9). For both systems, 24/25 patients (96%) were successfully recruited. The mean operating-level difference when success criteria were met was -0.58 in favor of the constant bolus device (95% CI: -0.88 to -0.28, <0.001). Secondary endpoints (walking distance, respiratory rate and BORG dyspnea) showed no statistically significant or clinically relevant differences. An algorithm created especially for this study showed a high success rate in terms of titration for the required operating level. Both portable oxygen-conserving devices met the success criteria in 96% of patients in the 6MWT when they were titrated to the correct level. The constant-bolus device required a significantly lower operating level to achieve the success criteria, hereby reducing energy consumption. Individual titration of the respective device is recommended, which can be facilitated by the novel titration algorithm described here.

We present a GPU accelerated N-body integrator using the Bulirsch–Stoer method, called GANBISS (GPU accelerated n-body code for binary star systems). It is designed to simulate the dynamical evolution of planetesimal disks in binary star systems which contain some thousand disk objects. However, it can also be used for studies of non-interacting massless bodies where up to 50 million objects can be studied in a simulation. GANBISS shows the energy and angular momentum conservation behavior of non-symplectic integration methods. The code is written in CUDA C and can be run on NVIDIA GPUs of compute capability of at least 3.5. A comparison of GPU and CPU computations indicates a speed-up of the GPU performance of up to 100 times—depending on the number of disk objects.