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Background/objectives: Current data on the care of patients with vasculitis in Germany are scarce. Patient-reported outcome (PRO) questionnaires can capture aspects of the disease that escape conventional scores for disease activity, remission, and damage. For this reason, the Association of Rheumatological Acute Care Clinics (VRA) initiated a data analysis as part of the KOBRA quality project, the results of which are presented here., Patients and Methods: Patients with vasculitis of vessels of any size or with polymyalgia rheumatica were included. The prospective survey included data on demographics, disease, pain, treatment, follow-up and satisfaction at the time of inpatient admission, discharge and follow-up after 2.5 months. All patients completed the AAV-PRO and EQ-5D-3L questionnaires on admission and follow-up., Results: In this study 420 patients were recruited and follow-up data were available from 302. On average, improvements were documented in all 5 dimensions of the EQ-5D, with the strongest effects in self-care and coping with activities of daily living. In the AAV-PRO, highly significant differences were seen in the domains systemic symptoms and physical functioning. Satisfaction with medical and nursing treatment was very high and did not correlate with pain level or with the AAV-PRO measures., Discussion: Under zreatment patient-reported outcomes improve at least partially in vasculitis patients. Satisfaction with medical treatment quality is independent of these outcomes., (© 2023. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Background: The drastic changes during the COVID-19 pandemic may have a negative impact on the psychological wellbeing of children and adolescents., Objectives: COPSY is the first national, representative German study to examine mental health and quality of life of children and adolescents during the pandemic. Results are compared with data of the representative longitudinal BELLA study conducted before the pandemic., Materials and Methods: Internationally established instruments for measuring health-related quality of life and mental health (including anxiety and depressive symptoms) were administered to n = 1586 parents with 7‑ to 17-year-old children and adolescents, of whom n = 1040 11- to 17-year-olds also provided self-reports, from 26 May to 10 June 2020. Data were analyzed using descriptive statistics and bivariate tests., Results: Seventy-one percent of the children and adolescents and 75% of the parents felt burdened by the first wave of the COVID-19 pandemic. Compared to the time before the pandemic, the children and adolescents reported a lower health-related quality of life, the percentage of children and adolescents with mental health problems almost doubled, and their health behavior worsened. Socially disadvantaged children felt particularly burdened by the COVID-19 pandemic. Two-thirds of the parents would like to receive support in coping with their child during the pandemic., Conclusions: The COVID-19 pandemic poses a mental health risk to children and adolescents. Schools, doctors, and society are called to react by providing low-threshold and target-group-specific prevention and mental health promotion programs., (© 2021. The Author(s).)

Severe, organ-threatening and life-threatening manifestations of inflammatory rheumatic diseases, such as diffuse alveolar hemorrhage in the context of small vessel vasculitis, sometimes inadequately respond to immunosuppressive treatment. In the case of an immanent or already occurring organ failure, immunosuppressive treatment may need to be supplemented with rapidly effective rescue treatment procedures. Due to the rarity of many rheumatic diseases, the evidence for the use of rescue treatment, such as plasmapheresis, extracorporeal membrane oxygenation (ECMO) and the administration of intravenous immunoglobulins (IVIG), is relatively low for many indications. The use of plasmapheresis is considered useful in acute anti-glomerular basement membrane (GBM) disease (Goodpasture's syndrome) or catastrophic antiphospholipid antibody syndrome (APS). The use of ECMO treatment may be considered for persistent respiratory failure despite mechanical ventilation due to diffuse alveolar hemorrhage or acute respiratory distress syndrome (ARDS). Administration of IVIG is indicated for acute cardiac involvement in Kawasaki's disease and may be considered in catastrophic APS and refractory myositis.

Objective: In distinguishing urate arthritis (UA) from non-crystal-related arthritides, joint sonography including the detection of the double contour sign (DCS) and hypervascularization using power Doppler ultrasound (PDUS) is an important step in the diagnostic process. But are these sonographic features equally reliable in every accessible joint under real-life conditions?, Methods: We retrospectively analyzed 362 patients with acute arthritis and evaluated the DCS and the degree of PDUS hypervascularization in patients with gout and in those with arthritis other than urate arthritis (non-UA). We classified all joints into the groups small, medium, and large. Sensitivities, specificities, positive and negative predictive values (PPV/NPV), and a binary regression model were calculated. We also evaluated the influence of serum uric acid levels (SUA) on the presence of a DCS in each joint category., Results: Sensitivity of the DCS in gout was 72.5% in the entire cohort, 66.0% in large, 78.8% in medium, and 72.3% in small joints. In wrist joints the DCS sensitivity maxed at 83.3%, with a specificity of 81.8%. The lowest rates of DCS sensitivity were found in gout patients with elbow joint involvement (42.9%). In all joints except metatarsophalangeal joint 1 (MTP-1), the incidence of a DCS increased by the increment of SUA levels above 7.5 mg/dl (p < 0.001). PDUS signals were most commonly found in medium and small joints and were only scarce in large joints, independent of the underlying diagnosis., Conclusions: In our study we detected different rates of accuracy regarding DCS and PDUS in patients with acute arthritis. The best results were seen in medium-size joints, especially wrists.

Large-vessel vasculitis (LVV) is a group of diseases mainly comprised of giant-cell arteritis (GCA), Takayasu arteritis, and a series of rare diseases like Behçet's disease, IgG4-related disease, infectious aortitis, and other unfrequent entities. Besides clinical and laboratory features, Doppler sonography (DS) can assist in establishing the diagnosis. Its diagnostic sensitivity has been evaluated in various studies, most of them, however, in temporal arteritis (TA) respectively in LVV with involvement of the temporal artery. Little is known in extracranial LVV. We retrospectively evaluated the diagnostic accuracy of DS in 30 patients with extracranial, non-temporal LVV using the highly sensitive PET/CT as method of reference in comparison to 20 controls who were found to have no LVV. We investigated ten arterial sites and documented the presence of the sonographic halo sign. Sensitivities of DS for LVV were highest in the subclavian and axillary arteries (71.4%/72.2%) and low in the abdominal aorta (26.1%) and the common femoral artery (16.7%). DS detected 24 out of 30 cases of LVV (overall sensitivity 80.0%). The LVV cases where DS was completely negative did not significantly differ in leukocyte count, C-reactive protein, or erythrocyte sedimentation rate from LVV cases with positive DS. DS is a potent method in diagnosing extracranial LVV especially in the axillary and the subclavian arteries. Aortic, intraabdominal, and lower extremity artery manifestations, however, are often missed by DS. A second imaging modality (e.g., PET/CT) is therefore required.

Renal involvement in sarcoidosis is much more common than generally assumed from old epidemiological studies and is often only detected when actively searched for. Many patients with renal sarcoidosis present with no or only few symptoms. The diagnostic work-up of sarcoidosis should always include a possible renal involvement. In cases of impaired renal function, proteinuria or a pathological urine sediment, a renal biopsy specimen should be obtained to assess the type, severity and prognosis of the kidney disease. Treatment is primarily based on the use of corticosteroids. Steroid-sparing agents, such as disease-modifying antirheumatic drugs and infliximab can be applied; however, the evidence for efficacy of these therapies is mostly based on case series and expert opinions. Discontinuation of immunosuppression therapy bears a high risk of relapse.

IgG4-related disease (IgG4-RD) are a group of autoinflammatory diseases often presenting as tumor-like lesions because of their infiltrative or mass forming behavior. They are characterized by a typical histology consisting of storiform fibrosis, high numbers of infiltrating IgG4-positive plasma cells, obliterative phlebitis, and a moderate presence of eosinophilic cells. Serum IgG4 levels can be elevated. We present a case of a 57 year-old male patient with immobilizing lower back pain, fever, and night sweats. We diagnosed IgG4-related periaortitis using serum IgG4 levels, abdominal ultrasound, PET/CT, and histology. We successfully treated the patient with glucocorticoids (GC) and azathioprine. Periaortitis is a rare presentation of IgG4-RD and therefore noteworthy. It has to be considered in patients with a retroperitoneal mass.

Background: The benefits of cancer screening in the elderly are uncertain. While the risk of cancer increases with age the participation in cancer screening decreases., Aim: The study investigated the attitudes of older adults towards cancer screening as well as their motives for or against participation., Material and Methods: This population-based explorative mixed methods study was based on a survey of residents aged 69-89 years from the district of Western Pomerania-Greifswald (northeast Germany). Criteria for exclusion were cognitive deficits and/or cancer. Attitudes towards different statements regarding cancer screening were assessed by a questionnaire using 5-point Likert scales. Semi-structured interviews were used to explore the motivations., Results: Out of 630 contacted residents, 120 (19%) with an average age of 77 years (SD ± 6 years) participated in the face-to-face survey. The majority were in favor of lifelong cancer screening, 14% stated other health problems to be more important than cancer screening and 7% assumed that they would not live long enough to benefit from screening. Motives for participation in cancer screening were habit, regularity, sense of obligation, fear and belief in benefits. Motives for discontinuing screening included a lack of interest, no assumed necessity and fear. Disadvantages were not feared., Conclusion: Elderly people show great trust in cancer screening. They overestimated the benefits of cancer screening and their risk to die of cancer. The elderly should be better informed about the benefits and risks of cancer screening. Shared decision-making should be based on life expectancy and personal preferences.

Objective: Reconstruction of the medial patellofemoral ligament with autologous tendon augmentation and soft tissue fixation at the patellar insertion with resorbable suture material., Indications: Patellofemoral instability due to insufficiency of the medial passive stabilizers and dysplastic trochlea., Contraindications: Primary traumatic dislocation of the patella without risk factors for patellar redislocation, severe osteoarthritis of the patellofemoral joint, infection., Surgical Technique: Diagnostic arthroscopy to evaluate cartilage and shape of trochlea and to treat associated injuries. Harvesting of the gracilis tendon and arming with resorbable suture material. Transfer of the tendon through the medial capsule in the anatomical layer of the MPFL and weaving in u-shape through the capsule and periosteum near the patella. Soft tissue fixation with resorbable suture material. Anatomical reconstruction of the femoral insertion site. Femoral fixation with interference screw., Postoperative Treatment: For 4 weeks, partial (20 kg) weight bearing with crutches; cast with physiotherapy (limited ROM extension, flexion 0-0-90°). Thereafter free range of motion and full weight bearing., Results: 27 patients (age 12-45 years) with patellofemoral instability underwent reconstruction of the medial patellofemoral ligament. Clinical follow-up was assessed up to 12 months postoperatively. After 1 year, the Kujala and Flandry scores increased from preoperatively 72 points to 95 points and 65.7 points to 89.9 points, respectively. One redislocation was observed. Patient satisfaction was significantly increased at 6 months postoperatively. Reconstruction of the medial patellofemoral ligament shows good clinical results after 12 months.

Background: For general practioners (GP) the treatment of patients suffering from multimorbidity is an everyday challenge. For these patients guidelines which each focus on a specific chronic disease cannot be applied comprehensively and equally; therefore, it is necessary to prioritize., Objective: Given this situation the study aimed at analyzing how GPs and patients deal with this challenge and what their priorities are., Material and Methods: Narrative interviews were conducted with 9 GPs and 19 of their multimorbid patients. The data were analyzed by means of content analysis., Results: The majority of interviewed patients felt well or very well cared for by their GPs; however, GPs and multimorbid patients often had relatively different priorities. Whereas GPs mostly focused on the management of diseases that could lead to life-threatening situations, patients put an emphasis on maintaining autonomy and a social life., Conclusion: The results of this study suggest that there is room for development in the way GPs and multimorbid patients communicate with each other, particularly as far as shared priority setting is concerned.

Pharmacokinetic data of disease modifying antirheumatic drugs during hemodialysis are limited to sulfasalazine, methotrexate, and cyclosporine. Only respective anecdotal data have been reported on leflunomide. We repeatedly measured teriflunomide (A77-1726), the active metabolite of leflunomide, during standard hemodialysis sessions and calculated teriflunomide clearances in five patients with rheumatoid arthritis (RA) and end-stage renal disease. The calculated teriflunomide clearances during a standardized dialysis session of 3-4.5 h at a blood flow rate of 160-300 ml/min were between 0 and 4.3 ml/min, the mean clearances of the total dialysis ranged between 1.1 and 3.4 ml/min. Total amount of teriflunomide removed was 5.8-8.8 μg per dialysis session. Dialytic removal of the active metabolite of leflunomide, teriflunomide (A77-1726), is negligible. Leflunomide can be used for RA patients on chronic dialysis without any dosage modification.