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OBJECTIVE: Mepolizumab proved to be an efficacious treatment for eosinophilic granulomatosis with polyangiitis (EGPA) at a dose of 300 mg every 4 weeks in the randomized, controlled MIRRA trial. In a few recently reported studies, successful real-life experiences with the approved dose for treating severe eosinophilic asthma (100 mg every 4 weeks) were observed. We undertook this study to assess the effectiveness and safety of mepolizumab 100 mg every 4 weeks and 300 mg every 4 weeks in a large European EGPA cohort. METHODS: We included all patients with EGPA treated with mepolizumab at the recruiting centers in 2015-2020. Treatment response was evaluated from 3 months to 24 months after initiation of mepolizumab. Complete response to treatment was defined as no disease activity (Birmingham Vasculitis Activity Score [BVAS] = 0) and a prednisolone or prednisone dose (or equivalent) of ���4 mg/day. Respiratory outcomes included asthma and ear, nose, and throat (ENT) exacerbations. RESULTS: Two hundred three patients, of whom 191 received a stable dose of mepolizumab (158 received 100 mg every 4 weeks and 33 received 300 mg every 4 weeks) were included. Twenty-five patients (12.3%) had a complete response to treatment at 3 months. Complete response rates increased to 30.4% and 35.7% at 12 months and 24 months, respectively, and rates were comparable between mepolizumab 100 mg every 4 weeks and 300 mg every 4 weeks. Mepolizumab led to a significant reduction in BVAS score, prednisone dose, and eosinophil counts from 3 months to 24 months, with no significant differences observed between 100 mg every 4 weeks and 300 mg every 4 weeks. Eighty-two patients (40.4%) experienced asthma exacerbations (57 of 158 [36%] who received 100 mg every 4 weeks; 17 of 33 [52%] who received 300 mg every 4 weeks), and 31 patients (15.3%) experienced ENT exacerbations. Forty-four patients (21.7%) experienced adverse events (AEs), most of which were nonserious AEs (38 of 44). CONCLUSION: Mepolizumab at both 100 mg every 4 weeks and 300 mg every 4 weeks is effective for the treatment of EGPA. The 2 doses should be compared in the setting of a controlled trial.

Um die Telemedizin erfolgreich in den rheumatologischen Alltag zu integrieren, sind sowohl die Patienten- als auch Arztperspektive wichtig. Hierzu wurde eine ausfuhrliche Studie mittels webbasierter Umfrage durch die Arbeitsgemeinschaft Junge Rheumatologie der Deutschen Gesellschaft fur Rheumatologie (DGRh) und die Deutsche Rheuma-Liga Bundesverband e. V. durchgefuhrt. Mittels Subgruppenanalyse der Daten im Hinblick auf die Videosprechstunde galt es nun herauszufinden, welche Anforderungen und Wunsche unter den Patienten/innen und Arzte/innen an die Videosprechstunde gestellt wurden. Die prospektive Umfrage wurde uber die sozialen Medien, QR-Code und E‑Mail verteilt. Es wurden deskriptive Statistiken und Regressionsanalysen in Bezug auf die Videosprechstunde durchgefuhrt und Korrelationen aufgezeigt. Die Daten zeigen eine positive Einstellung zur Videosprechstunde sowohl aufseiten der Patienten/innen (n = 299) als auch der Rheumatologen/innen (n = 129) auf. Eine Korrelation zwischen Alter und positiver Meinung zur Videosprechstunde zeigte sich unter den Patienten/innen (r = 0,161, p = 0,006), insbesondere bei den weiblichen Patienten fand sich mit zunehmendem Alter ein positiver Zuspruch fur die Videosprechstunde (r = 0,244, p

Einleitung: Die Nierenbeteiligung hat eine negative prognostische Bedeutung für den Verlauf der ANCA-assoziierten Vaskulitis (AAV). Diagnostischer Goldstandard ist die Nierenbiopsie. Herausforderungen bleiben die Beurteilung einer Reversibilität und die Therapiekontrolle. In Situationen eines [zum vollständigen Text gelangen Sie über die oben angegebene URL]

Reasons of mon- and oligoarthritis are heterogeneous. The diagnostic approach includes a detailed medical anamnesis, physical examination and imaging (conventional X-ray, sonography, MRI and, CT). Analysis of the synovial fluid is required in suspected septic arthritis and frequently helps in diagnosis and differential diagnosis of crystal arthropathies. Dual-energy-CT (DECT) detects sodium urate crystals and can replace joint puncture in some cases. In addition to crystal arthropathies and septic arthritis, differential diagnosis of mon-/oligoarthritis includes reactive arthritis, arthrosis and monarthritic courses of SpA/PsA. A rheumatologist should be consulted particularly in the case of persistent monarthritides, in order to initiate a specific therapy to prevent secondary damage.

Objective Despite increased physician’s awareness and improved diagnostic and serological testing in the recent years, the interval between the initial symptoms and the diagnosis of Systemic lupus erythematosus (SLE) is still very long. Our aim was to study this delay and its association to the outcome of the disease. Methods Information on demographics, onset of first symptoms, first physicians visit and time of diagnosis was assessed by self-reported questionnaires among SLE patients in Germany (LuLa cohort, n = 585) in the year 2012. Disease activity (Systemic Lupus Activity Questionnaire; SLAQ), disease related damage (Brief Index of Lupus Damage; BILD), health related quality of life (Short Form 12) and fatigue (FSS) were chosen as proxies for outcome. Linear regression analysis was used to analyze the association of the delay in diagnosis to the outcome, adjusted for age, disease duration and sex. Results Mean duration between the onset of symptoms and the diagnosis of SLE was 47 months (SD 73). The longer the time to diagnosis, the higher the disease activity (β = 0.199, p Conclusion In systemic lupus erythematosus, longer time to diagnosis was associated with worse outcome. Concepts in care with the intention to shorten the time to diagnosis are needed to improve the long-term outcome of the disease.

An international consensus on antineutrophil cytoplasm antibodies (ANCA) testing in eosinophilic granulomatosis with polyangiitis (EGPA) is presented. ANCA, specific for myeloperoxidase (MPO), can be detected in 30-35% of patients with EGPA. MPO-ANCA should be tested with antigen-specific immunoassays in any patient with eosinophilic asthma and clinical features suggesting EGPA, including constitutional symptoms; purpura; polyneuropathy; unexplained heart, gastrointestinal, or kidney disease; and/or pulmonary infiltrates or hemorrhage. A positive MPO-ANCA result contributes to the diagnostic workup for EGPA. Patients with MPO-ANCA-associated EGPA have vasculitis features, such as glomerulonephritis, neuropathy, and skin manifestations, more frequently than patients with ANCA-negative EGPA. However, the presence of MPO-ANCA is neither sensitive nor specific enough to identify whether a patient should be subclassified as having "vasculitic" or eosinophilic" EGPA. At present, ANCA status cannot guide treatment decisions, that is, whether cyclophosphamide, rituximab, or mepolizumab should be added to conventional glucocorticoid treatment. In EGPA, monitoring of ANCA is only useful when MPO-ANCA was tested positive at disease onset.

Haufig werden uns als Rheumatologen Patienten mit einer Muskelschwache und Erhohung der Kreatinkinase (CK) vorgestellt. Nicht immer kann eine Myositis als Ursache der Beschwerden festgestellt werden. Wir prasentieren 2 Kasuistiken aus unserer Klinik, deren Diagnose erst mithilfe der Muskelbiopsie gestellt werden konnte: Im ersten Fall handelt es sich um einen Patienten mit einer Muskelschwache, einem pathologischen Gewichtsverlust und einer deutlichen CK-Erhohung. Mithilfe der Muskelbiopsie konnte die Diagnose einer immunvermittelten nekrotisierenden Myopathie bedingt durch HMG-CoA-Reduktase-Autoantikorper nach Statineinnahme gestellt werden. Die zweite Patientin prasentierte sich mit krampfartigen und brennenden Muskelschmerzen sowie einer Schwache der Extremitaten ohne relevante CK-Erhohung. Auch hier erfolgte die histologische Untersuchung eines betroffenen Muskels, in der sich ein Myoadenylat-Deaminase-Mangel zeigte. Dieser konnte in einer genetischen Untersuchung bestatigt werden.

Objective Our aim was to study the quality of medical care in patients with systemic lupus erythematosus (SLE) to understand gaps and to analyze the association with outcome of the disease. Methods Information on demographics and medical care was assessed by self-reported questionnaires among SLE patients (LuLa cohort, 2011, n = 580). In total, 21 aspects of medical care were analyzed. Univariate analysis selected 10 predictor variables for further analysis: (1) urine examination and (2) blood test in the previous year, (3) taking antimalarials, (4) taking vitamin D and calcium if the dosage of prednisolone was greater than 7.5 mg/day, counseling regarding (5) lipid metabolism, (6) vaccination, and (7) blood pressure, and treatment of the comorbidities (8) hypertension, (9) osteoporosis and (10) lipid metabolism disorder. The association of these 10 items with the outcome of the disease, assessed in 2015, was analyzed by linear regression analysis, adjusted for age, disease duration and sex. Results On average six of the 10 items were met (±1.7). Receiving more clinical care in 2013 was predictive for low disease activity (SLAQ, p = 0.024, β = –0.104, corr. R2 = 0.048), low progress in disease-related damage (Delta Brief Index of Lupus Questionnaire, p = 0.048, β = –0.132, corr. R2 = 0.036) and high health-related quality of life (SF-12 physical, p = 0.035, β = 0.100, corr. R2 = 0.091) in 2015. Conclusion Our study illustrates a link between the quality of care and the SLE outcome parameters disease activity, disease-related damage and quality of life. Consistent considerations of these care parameters, which are recommended in several management guidelines, could therefore be a good approach to improve the outcome of patients with SLE.

Objective The aim of this study was to identify factors associated with impaired work productivity and impaired daily activities in patients with systemic lupus erythematosus (SLE). Methods The LuLa study is a longitudinal patient-reported study. Beyond sociodemographic data, work productivity, daily activities and fatigue, several other clinical outcome parameters (e.g. mental health–related quality of life and physical functioning, disease activity, damage and pain) were surveyed with validated questionnaires. The effects of confounders on work productivity (WPAI 2) and daily activity domains (WPAI 4) were studied by multivariate regression analysis. Results A total of 585 patients completed the questionnaire of whom 259 were employed and analysed. The median impairment in work productivity (WPAI 2) was 20% (Q1–3 0–40), and the median impairment in daily activities (WPAI 4) was 30% (Q1–3 10–50%). Multivariate regression analysis revealed that fatigue, pain, disease activity and health-related quality of life affected WPAI 2 and 4. Furthermore, we observed distinct synergistic effects of fatigue, disease activity and pain on both work productivity and daily activities: a higher impact of fatigue was associated with the reported extent of pain or disease activity. Conclusion In employed patients with SLE, impaired work productivity and impaired daily activities were frequently reported. Fatigue, pain, disease activity and health-related quality of life demonstrated a detrimental impact, with a synergistic effect of fatigue, disease activity and pain. Hence, both optimized pain management and targeted immunomodulatory therapy are important for preserving active participation in life among patients with fatigue.

Substance P (SP), a product of the tachykinin 1 (Tac1) gene, is expressed in many hypothalamic neurons. Its wake-promoting potential could be mediated through histaminergic (HA) neurons of the tuberomamillary nucleus (TMN), where functional expression of neurokinin receptors (NKRs) waits to be characterized. As in the process of nociception in the peripheral nervous system (PNS) capsaicin-receptor (transient potential vanilloid 1: TRPV1) signalling is amplified by local release of histamine and SP, we tested the involvement of tachykinins in the capsaicin-induced long-lasting enhancement (LLEcaps) of HA neurons firing by investigating selective neurokinin receptor ligands in the hypothalamic mouse brain slice preparation using patch-clamp recordings in cell-attached mode combined with single-cell RT-PCR. We report that the majority of HA neurons respond to SP (EC50 3 nM), express the SP precursor tachykinin 1 (Tac1) gene and at least one of the neurokinin receptors. Responses to selective agonists of three known neurokinin receptors were sensitive to corresponding antagonists. LLEcaps was significantly impaired by the neurokinin receptor antagonists, indicating that in hypothalamus, as in the PNS, release of tachykinins downstream to TRPV1 activation is able to boost the release of histamine. The excitatory action of SP on histaminergic neurons adds another pathway to the noradrenergic and orexinergic ones to synergistically enhance cortical arousal. We show NK1R to play a prominent role on HA neurons and thus the control of wakefulness.

Einleitung: Die Heterogenität und Vielfalt möglicher Differentialdiagnosen bei Kollagenosen stellt auch heute noch eine Herausforderung in der Diagnostik dar. Ziel unserer Studie war die Untersuchung, inwieweit sich die Latenzzeit zwischen Symptombeginn und erster rheumatologischer Vorstellung[zum vollständigen Text gelangen Sie über die oben angegebene URL], Deutscher Rheumatologiekongress 2021, 49. Kongress der Deutschen Gesellschaft für Rheumatologie (DGRh), 35. Jahrestagung der Deutschen Gesellschaft für Orthopädische Rheumatologie (DGORh), Wissenschaftliche Herbsttagung der Gesellschaft für Kinder- und Jugendrheumatologie (GKJR)

Vorgeschichte: An Vorerkrankungen bestehen ein Z.n. Myokardinfarkt, ein arterieller Hypertonus sowie ein normofrequentes, permanentes Vorhofflimmern. Auf Nachfrage berichtet der 78-jährige Patient zudem von wiederholten Gelenkentzündungen, bei denen Kortison gut helfe. Rheumatologisch wurde[zum vollständigen Text gelangen Sie über die oben angegebene URL], Deutscher Rheumatologiekongress 2021, 49. Kongress der Deutschen Gesellschaft für Rheumatologie (DGRh), 35. Jahrestagung der Deutschen Gesellschaft für Orthopädische Rheumatologie (DGORh), Wissenschaftliche Herbsttagung der Gesellschaft für Kinder- und Jugendrheumatologie (GKJR)

Background: Evidence on the efficacy of Mepolizumab (MEPO) in Eosinophilic Granulomatosis with Polyangiitis (EGPA) is scarce [1]. Objectives: To assess the efficacy and safety of MEPO in real-life clinical practice. Methods: We retrospectively included patients diagnosed with EGPA and treated with MEPO (100 or 300 mg/month). MEPO efficacy was evaluated in the first 12 months in terms of systemic disease and asthma control. The occurrence of any adverse event (AE) was recorded. Results: 142 patients were included (38% males; median age 46.4 (IQR 36.7-54.4); 110 and 32 on MEPO 100 and 300 mg/month, respectively). General, ear-nose-throat, pulmonary, and neurological symptoms significantly decreased during treatment (table 1). MEPO accounted for a significant reduction in the BVAS (figure 1) and for a steroid sparing effect (figure 2). The proportion of patients with asthma attacks decreased by 90% at 12 months compared to t0, and asthma-related emergency accesses dropped from 17.4% to 2.3%. Overall, 21.1% of patients had a non-serious AE.

In order to successfully integrate telemedicine into the daily routine of rheumatology, both the patient's and the physician's perspective are important. For this purpose, a detailed study by means of a web-based survey was conducted by the Working Group Young Rheumatology (AGJR) of the German Society for Rheumatology (DGRh) and the German Rheumatism League National Association. By means of subgroup analysis of the data regarding video consultation, the aim was now to find out which requirements and wishes patients and physicians have for video consultations.The prospective survey was distributed via social media, QR code and email. Descriptive statistics and regression analysis related to video consultation were performed and correlations were shown.The data indicated positive attitudes toward video consultation on the part of both patients (n = 299) and rheumatologists (n = 129). A correlation between age and positive opinion of the video consultation was found among the patients (r = 0.161, p = 0.006), especially among female patients a positive approval of the video consultation was found with increasing age (r = 0.244, p 0.001 to male patients: r = -0.190, p = 0.145). Regarding the travelling time to the treating rheumatologist, male patients found the video consultation more attractive with increasing travelling time (r = 0.229, p = 0.078). With respect to the wishes of patients and physicians, video consultation should be used primarily for follow-up or emergency appointments. Video consultation for initial appointments, on the other hand, was very rarely mentioned.During the COVID 19 pandemic, video consultation was increasingly popular among rheumatology patients as well as among rheumatologists.EINLEITUNG: Um die Telemedizin erfolgreich in den rheumatologischen Alltag zu integrieren, sind sowohl die Patienten- als auch Arztperspektive wichtig. Hierzu wurde eine ausführliche Studie mittels webbasierter Umfrage durch die Arbeitsgemeinschaft Junge Rheumatologie der Deutschen Gesellschaft für Rheumatologie (DGRh) und die Deutsche Rheuma-Liga Bundesverband e. V. durchgeführt. Mittels Subgruppenanalyse der Daten im Hinblick auf die Videosprechstunde galt es nun herauszufinden, welche Anforderungen und Wünsche unter den Patienten/innen und Ärzte/innen an die Videosprechstunde gestellt wurden.Die prospektive Umfrage wurde über die sozialen Medien, QR-Code und E‑Mail verteilt. Es wurden deskriptive Statistiken und Regressionsanalysen in Bezug auf die Videosprechstunde durchgeführt und Korrelationen aufgezeigt.Die Daten zeigen eine positive Einstellung zur Videosprechstunde sowohl aufseiten der Patienten/innen (n = 299) als auch der Rheumatologen/innen (n = 129) auf. Eine Korrelation zwischen Alter und positiver Meinung zur Videosprechstunde zeigte sich unter den Patienten/innen (r = 0,161, p = 0,006), insbesondere bei den weiblichen Patienten fand sich mit zunehmendem Alter ein positiver Zuspruch für die Videosprechstunde (r = 0,244, p 0,001 zu männlichen Patienten: r = −0,190, p = 0,145). Bezüglich der Fahrtzeit bis zum behandelnden Rheumatologen tendierten die männlichen Patienten mit zunehmender Fahrzeit zur Videosprechstunde (r = 0,229, p = 0,078), dies war jedoch nur schwach positiv. Hinsichtlich der Wünsche von Patienten/innen und Ärzten/innen sollte die Videosprechstunde v. a. für Folge- oder Notfalltermine genutzt werden. Eine Videosprechstunde für die Erstvorstellungen war hingegen nur sehr selten gewünscht.Während der COVID-19-Pandemie hat die Videosprechstunde zunehmenden Zuspruch unter den rheumatologischen Patienten/innen als auch unter den behandelnden Rheumatologen/innen erhalten.

Reasons of mon- and oligoarthritis are heterogeneous. The diagnostic approach includes a detailed medical anamnesis, physical examination and imaging (conventional X-ray, sonography, MRI and, CT). Analysis of the synovial fluid is required in suspected septic arthritis and frequently helps in diagnosis and differential diagnosis of crystal arthropathies. Dual-energy-CT (DECT) detects sodium urate crystals and can replace joint puncture in some cases. In addition to crystal arthropathies and septic arthritis, differential diagnosis of mon-/oligoarthritis includes reactive arthritis, arthrosis and monarthritic courses of SpA/PsA. A rheumatologist should be consulted particularly in the case of persistent monarthritides, in order to initiate a specific therapy to prevent secondary damage.

ObjectiveTo analyse the impact of the COVID-19 pandemic on rheumatic patients’ and rheumatologists’ usage, preferences and perception of digital health applications (DHAs).MethodsA web-based national survey was developed by the Working Group Young Rheumatology of the German Society for Rheumatology and the German League against Rheumatism. The prospective survey was distributed via social media (Twitter, Instagram and Facebook), QR code and email. Descriptive statistics were calculated, and regression analyses were performed to show correlations.ResultsWe analysed the responses of 299 patients and 129 rheumatologists. Most patients (74%) and rheumatologists (76%) believed that DHAs are useful in the management of rheumatic and musculoskeletal diseases (RMDs) and felt confident in their own usage thereof (90%; 86%). 38% of patients and 71% of rheumatologists reported that their attitude had changed positively towards DHAs and that their usage had increased due to COVID-19 (29%; 48%). The majority in both groups agreed on implementing virtual visits for follow-up appointments in stable disease conditions. The most reported advantages of DHAs were usage independent of time and place (76.6%; 77.5%). The main barriers were a lack of information on suitable, available DHAs (58.5%; 41.9%), poor usability (42.1% of patients) and a lack of evidence supporting the effectiveness of DHAs (23.2% of rheumatologists). Only a minority (ConclusionThe COVID-19 pandemic instigated an increase in patients’ and rheumatologists’ acceptance and usage of DHAs, possibly introducing a permanent paradigm shift in the management of RMDs.

Introduction: Giant cell arteritis (GCA) is characterized by an increased release of IFN-γ by Th1 cells and dendritic cells accompanied by an invasion of lymphocytes, dendritic cells and monocytes. The mechanisms contributing to cellular trafficking in GCA are incompletely understood. The most[for full text, please go to the a.m. URL], Deutscher Rheumatologiekongress 2020, 48. Kongress der Deutschen Gesellschaft für Rheumatologie (DGRh), 34. Jahrestagung der Deutschen Gesellschaft für Orthopädische Rheumatologie (DGORh)

Einleitung: Ziel der Auswertung war die Identifizierung (beeinflussbarer) Faktoren eingeschränkter Arbeitsproduktivität und Alltagsaktivität bei Patienten mit systemischem Lupus erythematodes. Methoden: Die Lupus Langzeitstudie ist eine prospektive Patientenbefragung von Lupuspatienten[zum vollständigen Text gelangen Sie über die oben angegebene URL], Deutscher Rheumatologiekongress 2020, 48. Kongress der Deutschen Gesellschaft für Rheumatologie (DGRh), 34. Jahrestagung der Deutschen Gesellschaft für Orthopädische Rheumatologie (DGORh)

Rheumatologists are often confronted by patients with muscle weakness and elevated creatine kinase (CK) levels. Myositis cannot always be determined to be the cause of the complaints. This article presents two cases from our hospital where the diagnosis could only be determined by muscle biopsy. In the first case the patient presented with muscle weakness, pathological weight loss and a significant increase in CK levels. A muscle biopsy revealed an immune-mediated necrotizing myopathy (IMNM) caused by anti-3-hydroxy-3-methyl-gulatryl-CoA reductase (HMG-CoA reductase) autoantibodies due to the intake of statins. The second patient presented with cramp-like and burning muscle pain and weakness of the extremities without a relevant increase in CK level. Myoadenylate deaminase deficiency was also detected by muscle biopsy, and further confirmed by genetic testing.

Purpose Our aim was to study the delay from the onset of symptoms to the diagnosis of systemic lupus erythematosus (SLE) and its association to the outcome of the disease. Methods Information on demographics, onset of first symptoms, first physicians visit and time of diagnosis was assessed by self-reported questionnaires among SLE patients in Germany in 2010 (LuLa cohort, n=585). Disease activity (Systemic Lupus Activity Questionnaire; SLAQ), disease related damage (Brief Index of Lupus Damage; BILD) and health related quality of life were chosen as relevant proxies for outcome. Linear regression analysis was used to analyze the association to the outcome of the disease, adjusted for age, disease duration and sex. Results Mean reported duration between the onset of symptoms and the diagnosis of SLE was 45.7 months (SD 72.6), including a mean duration of 13.2 month (SD 40.9) between the onset of symptoms and the first physicians visit. In our cohort, the mean disease duration was 17.7 years (SD 7.89). A delayed diagnosis was associated with high disease activity (SLAQ, p Conclusion The time to diagnosis was associated with a worse outcome in systemic lupus erythematosus, assessed by self-reported questionnaires for disease activity, disease-related damage and quality of life, unaffected by the organ involvement at the time of diagnosis. An early diagnosis should therefore be strived to improve the long-term outcome of the disease. German Clinical Trial Register, www.germanctr.de, DRKS00011052 Acknowledgement The LuLa study is supported by unrestricted grants from GlaxoSmithKline and UCB Pharma.

A few decades ago, the therapy goal of patients with systemic lupus erythematosus (SLE) was survival and the prevention of organ failure. Today, clinical remission and low disease activity are believed to be the optimal therapeutic targets. These aims are difficult to reach for many patients, but they still do not address the health-related quality of life (QoL) that is significantly impaired in SLE patients. Even in the state of remission, QoL and fatigue are insufficient controlled. Thus, patient-oriented research is essential to design new strategies for the management of lupus patients. The INTEGRATE project analyses the patients’ and physicians’ perspectives to pave the way to design an innovative therapeutic strategy for lupus and focuses on the multifaceted dimensions of the disease burden. Shared decision making (SDM) could include the patient’s perspective of SLE to treatment strategy and consider QoL and the burden of lupus into the process of therapy decision.