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Objective Neurological manifestations of Sjögren’s syndrome can be severe but also treatment-responsive. We aimed to systematically evaluate neurological manifestations of primary Sjögren’s syndrome and find clinical features allowing sufficient identification of affected patients (pSSN) among those with Sjögren’s syndrome without neurological involvement (pSS). Methods Para-/clinical features of patients with primary Sjögren’s syndrome (2016 ACR/EULAR classification criteria) were compared between pSSN and pSS. At our university-based center, patients with suggestive neurological symptoms undergo screening for Sjögren’s syndrome, and newly diagnosed pSS patients are thoroughly evaluated for neurologic involvement. pSSN disease activity was rated by the Neurological Involvement of Sjögren’s Syndrome Disease Activity Score (NISSDAI). Results 512 patients treated for pSS/pSSN at our site between 04/2018 and 07/2022 were included (238 pSSN patients [46%] vs. 274 pSS patients [54%], cross-sectional design). Independent predictors of neurological involvement in Sjögren’s syndrome were male sex [p p p p = 0.04] and higher eosinophil values (treatment-naïve) [p = 0.02]. Univariate regression additionally showed older age at diagnosis [p p = 0.001], SSA(Ro)/SSB(La) antibodies [p = 0.03; p p = 0.02] and CK levels [p = 0.02] (treatment-naïve) in pSSN. Interpretation Patients with pSSN had different clinical characteristics than patients with pSS and represented a large proportion of the cohort. Our data suggest that neurological involvement in Sjögren’s syndrome has been underestimated. Intensified screening for neurologic involvement should be included in the diagnostic algorithm for Sjögren’s syndrome, especially in males of older age and with severe disease course requiring hospitalization.

BACKGROUND: Inborn errors of immunity (IEI) are rare diseases, which makes diagnosis a challenge. A better description of the initial presenting manifestations should improve awareness and avoid diagnostic delay. Although increased infection susceptibility is a well-known initial IEI manifestation, less is known about the frequency of other presenting manifestations.OBJECTIVE: We sought to analyze age-related initial presenting manifestations of IEI including different IEI disease cohorts.METHODS: We analyzed data on 16,486 patients of the European Society for Immunodeficiencies Registry. Patients with autoinflammatory diseases were excluded because of the limited number registered.RESULTS: Overall, 68% of patients initially presented with infections only, 9% with immune dysregulation only, and 9% with a combination of both. Syndromic features were the presenting feature in 12%, 4% had laboratory abnormalities only, 1.5% were diagnosed because of family history only, and 0.8% presented with malignancy. Two-third of patients with IEI presented before the age of 6 years, but a quarter of patients developed initial symptoms only as adults. Immune dysregulation was most frequently recognized as an initial IEI manifestation between age 6 and 25 years, with male predominance until age 10 years, shifting to female predominance after age 40 years. Infections were most prevalent as a first manifestation in patients presenting after age 30 years.CONCLUSIONS: An exclusive focus on infection-centered warning signs would have missed around 25% of patients with IEI who initially present with other manifestations.

Background and objective The work situation is an important dimension of professional life and wellbeing, and a policy lever to strengthen recruitment and retention. This study aims to explore the work situation of physicians and residents in internal medical rheumatology, considering the impact of the coronavirus pandemic COVID-19. Methods A questionnaire-based online survey was conducted in early 2021 at the Hannover Medical School, supported by the German Society of Rheumatology. Target groups were all rheumatology physicians and residents in Germany. The main areas of investigation included work hours, task delegation, and collaboration; workload and mental health issues; discrimination and sexual harassment experiences; and the impact of COVID-19. Descriptive statistical analysis was performed for the standardized items and qualitative content analysis for the free-text information. Results The respondents (n = 101) expressed positive attitudes towards cooperation and task delegation to medical assistants, especially those specialized in rheumatology, while attitudes towards cooperation with GPs pointed to blockades. There was a strong mismatch between actual and desired work hours both in the group of women and in the group of men. 81% rated their workload as high or very high; every sixth rheumatologist has suffered from stress or burnout syndromes at least once in the past. Experiences of gender discrimination and sexual harassment/violence were frequently reported, mostly by women. COVID-19 was an amplifier of stress, with major stressors being digitalization and increased demand for communication and patient education. Conclusion There is an urgent need to improve the work situation of rheumatologists and reduce stress and mental health risks.

ObjectiveThe clinical spectrum of primary antibody deficiencies (PADs) and especially common variable immunodeficiency (CVID) includes various autoimmune disorders. We studied the prevalence and the features of articular rheumatic disease in a cohort of patient with PADs.MethodsIn this retrospective cohort study, complete clinical data of 268 patients with PADs, mainly consisting of patients with CVID, visiting the immunology outpatient clinic of a German tertiary hospital between 2018 and 2021 were collected. Those included case history, physical examination, laboratory as well as radiological findings.ResultsInflammatory arthritis was diagnosed in 16.4% of studied patients and was significantly more common among patients with PAD-associated enteropathy (OR 13.39, p=0.0001), splenomegaly (OR 6.09, p=0.0001) or atopic diseases (OR 3.31, p=0.021). Given HLA-B27 status, the involvement of the axial skeleton and the presence of features, such as anterior uveitis, inflammatory bowel disease, psoriasis and/or dactylitis, 75% of studied patients fulfilled the Assessment of Spondyloarthritis International Society classification criteria.ConclusionPAD-associated arthritis frequently shares features with spondyloarthritis (SpA) and enteropathic arthritis. The latter may suggest the interconnected pathomechanisms of inflammatory arthritis in SpA and PADs.

Background: Patients with Sjögren’s syndrome and polyneuropathy more frequently develop cranial nerve affection when compared to patients with chronic inflammatory demyelinating polyneuropathy (CIDP). We therefore aimed to analyze trigeminal corneal nerve fibre characteristics in both patient groups. Methods: A total of 26 patients with Sjögren’s syndrome associated neuropathy and 29 patients with CIDP were recruited at our university hospital and compared to 6 healthy controls. Dry eye symptoms and signs were assessed via clinical examination and the Ocular Disease Surface Index questionnaire. Trigeminal corneal nerve fibres were analyzed via corneal confocal microscopy (CCM) as a non-invasive in vivo microscopy. Results: CCM revealed significantly reduced corneal nerve fibre density and corneal nerve fibre main branch density in the Neuro-Sjögren group when compared with healthy controls. There were no significant group differences between the Neuro-Sjögren and the CIDP group for any of the microscopic parameters. Dry eye assessment showed similarly reduced scores for both patient groups, while healthy controls showed better results for objective dry eye signs. There was no correlation between microscopic parameters of the corneal confocal microscopy and parameters of dry eye assessment. Conclusions: Our data revealed trigeminal corneal nerve affection in patients with neuropathy associated with Sjögren’s syndrome and patients with CIDP detected by CCM. No difference was found between both neuropathy groups indicating that CCM is not able to distinguish between both entities.

Zusammenfassung Hintergrund und Fragestellung Fachkräftemangel in der Rheumatologie in Deutschland ist als Versorgungsproblem erkannt. Die Gesundheitspolitik hat mit neuen Planungszielen reagiert, aber es fehlen effektive Interventionsstrategien. Ziel dieser Studie ist ein systematischer berufsstruktureller Überblick, um die Grundlage für Interventionen zu verbessern und Möglichkeiten für ein effektives Fachkräftemanagement aufzuzeigen. Methode Die WHO National Health Workforce Accounts (NHWA) dienen als konzeptioneller Rahmen. Ausgewählt werden 4 Indikatoren: Personalbestand, Arbeitsmarktbewegungen, Komposition und Weiterbildung. Die Exploration von Entwicklungstrends stützt sich auf vergleichende Analysen von Altersgruppen und Zeitreihen. Die Erhebung nutzt öffentliche Statistiken und andere Sekundärliteratur; die Auswertung erfolgt deskriptiv. Ergebnisse In Deutschland sind 1076 Ärzt*innen mit einer Facharztqualifikation oder Schwerpunktbezeichnung in der internistischen Rheumatologie ärztlich tätig. Die absolute Zahl verdoppelte sich seit 2000 deutlich (91 %), aber mit einem demografischen Bias. Im Zeitraum 2000 bis 2019 stieg die Zahl der über 50-Jährigen deutlich, aber die der unter 50-Jährigen nur um 9 %; seit 2010 sind die Zahlen in der Gruppe 40 bis 50 Jahre rückläufig. Im Jahr 2019 waren mehr Rheumatolog*innen im Rentenalter als unter 40-Jährige ärztlich tätig. Seit 2015 schwächt sich der steigende Trend insgesamt ab, aber am stärksten im Krankenhaussektor; die Weiterbildungen lassen keine konstante Steigerung erkennen. Schlussfolgerungen Berufsstrukturelle Trends zeigen, dass die gesundheitspolitischen Planziele mit den verfügbaren Humanressourcen nicht zu erreichen sind. Gefordert ist ein besseres Fachkräftemanagement, insbesondere durch Innovation der Weiterbildung, Aufgabenverschiebung und verbesserte Geschlechtergerechtigkeit.

Zusammenfassung Hintergrund In der ambulanten Versorgung wurde Telemedizin im Lockdown von März bis Mai 2020 eingesetzt. Ziel der Studie war es, Patienten aus einer Praxis und der Hochschulambulanz bezüglich Patientenzufriedenheit mit Telemedizin, COVID-19-Sorgen und Impfverhalten auszuwerten sowie die Gesprächsführung durch eine rheumatologische Fachassistenz (RFA) mit einem Arzt zu vergleichen. Methoden Patienten mit rheumatoider Arthritis, Psoriasisarthritis oder Spondyloarthritis ohne Therapieänderung seit der letzten Vorstellung wurde ein telemedizinischer Ersatztermin im Rahmen dieser Studie bei Terminabsage durch die versorgenden Zentren angeboten. Randomisiert wurden sie von einem Arzt oder einer RFA (RFA nur Universität) telemedizinisch versorgt. Die Anamnese erfolgte telefonisch standardisiert mittels Fragebogen. Die Krankheitsaktivität wurde mittels modifizierten Clinical Disease Activity Score (CDAI) und BASDAI festgestellt. Im Anschluss erhielten die Patienten einen pseudonymisierten Evaluationsbogen. Ergebnisse Von 112/116 (96 %) eingeschlossenen Patienten schickten 88/112 (79 %) den Fragebogen zurück. RFAs führten 19/112 (17 %) Telefonate. Die Therapie wurde in 19/112 (17 %) geändert. Die meisten Sorgen bezüglich COVID-19 hatten Patienten mit der höchsten Krankheitsaktivität (p = 0,031), den meisten schmerzhaften Gelenken (p = 0,001) sowie den meisten Schmerzen (VAS Score ≥7) (p = 0,009). Diese Patienten hätten auch selbst ihren Termin abgesagt (p = 0,015). Die Patientenzufriedenheit mit der Gesprächsführung war gut (Mittelwert 4,3/5,0 modifizierter FAPI), unabhängig von der Institution, der Gesprächsdauer oder dem Gesprächspartner. Patienten mit hoher Schmerzintensität waren am unzufriedensten (p = 0,036); 42/100 (38,2 %) Patienten waren gegen Pneumokokken und 59/100 (53,6 %) gegen Influenza geimpft. Zusammenfassung Für ausgewählte Patienten ist die telemedizinische Versorgung im Rahmen eines Telefongespräches gut geeignet. Hinsichtlich der Patientenzufriedenheit ist die Delegation einer telefonischen Visite an eine RFA möglich. Bezüglich des Impfverhaltens besteht Verbesserungsbedarf.

This study addresses the challenging characterisation and differentiation of CIDP versus CIDP in association with Sjogren’s syndrome to facilitate the process in clinical routine. Patients with both CIDP and Sjogren’s syndrome and CIDP without Sjogren’s syndrome were compared concerning relevant differences in clinical, laboratory and electrophysiological findings. 154 patients who fulfilled the diagnostic EFNS/PNS criteria for CIDP were included in the analysis. 54 of these patients additionally fulfilled the ACR/EULAR classification criteria for Sjogren’s syndrome. The frequency of female patients was higher in patients with CIDP and Sjogren’s syndrome (52%) versus CIDP patients without Sjogren’s syndrome (28%). Furthermore, the occurrence of cranial nerve impairment was significantly higher in patients with Sjogren’s syndrome (39% versus 14%). There were no significant group differences in the evaluation of initial symptoms, severity of disability judged by INCAT disability scale score, presence or distribution of sensory deficits, limb weakness and the presence of ataxia, pain or dysautonomia, CSF laboratory or electrophysiological findings. In conclusion, our data indicate that cranial nerve impairment and female gender might represent red flags for an additional Sjogren’s syndrome in patients with CIDP. The patterns of clinical disabilities and electrophysiological findings due to peripheral nerve damage are similar in both CIDP entities.

Case presentation A 37-year-old previously healthy and athletic woman presented to the ED in October 2018 with acute-onset high fever, dyspnea, and productive cough. Chest radiograph showed bilateral infiltrates that correlated with multifocal ground glass opacities in a thoracic CT scan ( Fig 1 ). The patient was severely hypoxemic and required intensive care and oxygen administration via a high-flow nasal cannula. On admission, leucocyte counts were 23.3 k/μL; platelet counts were 518 k/μL; hemoglobin level was 12 g/dL; C-reactive protein was 83 mg/L, and procalcitonin was 0.7 μg/L. An auto-antibody panel that included antinuclear antibodies, extractable nuclear antigen (including anti-centromere-antibodies), antineutrophil cytoplasmic antibodies, and myositis- and granulocyte macrophage colony-stimulating factor-antibodies was negative, as was the rheumatoid factor. Immunoglobulins that included IgG1-4 and IgA and renal function were normal. Sicca symptoms like xerophthalmia and xerostomia were negated. The patient fully recovered after empiric administration of antibiotics and glucocorticoids (initially 500 mg methylprednisolone daily over 3 days with consecutive tapering).

Background Serology testing is explored for epidemiological research and to inform individuals after suspected infection. During the coronavirus disease 2019 (COVID-19) pandemic, frontline healthcare professionals (HCP) may be at particular risk for infection. No longitudinal data on functional seroconversion in HCP in regions with low COVID-19 prevalence and low pre-test probability exist. Methods In a large German university hospital, we performed weekly questionnaire assessments and anti-severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) immunoglobulin G (IgG) measurements with various commercial tests, a novel surrogate virus neutralisation test, and a neutralisation assay using live SARS-CoV-2. Results From baseline to week 6, 1080 screening measurements for anti-SARS CoV-2 (S1) IgG from 217 frontline HCP (65% female) were performed. Overall, 75.6% of HCP reported at least one symptom of respiratory infection. Self-perceived infection probability declined over time (from mean 20.1% at baseline to 12.4% in week 6, p

Patients with rheumatoid arthritis (RA) tend to be less physically active. Physical activity has been shown to have a positive impact on disease activity and quality of life and is recommended by the European League Against Rheumatism (EULAR) as an integral component of standard treatment.A cross-sectional analysis of RA patients was carried out assessing disease activity, functional capacity and fatigue associated with physical activity.Physical activity, functional capacity and the global fatigue index (GFI) were examined using standardized questionnaires: the international physical activity questionnaire short form (IPAQ-SF), the functional questionnaire Hannover (FFbH) and the multidimensional assessment of fatigue (MAF). The data were evaluated using SPSS 26 (IBM, Armonk, NY, USA). The level of significance was tested with bivariate and partial correlations and nonparametric tests.In total 164 patients were included in the study. The majority of the patients were female (127/164; 77%) and the median age of the cohort was 58.3 years (range 21-86 years). The median duration of disease-related symptoms at inclusion was 169 months (range 0-713 months). Physical activity was low in 39%, moderate in 37% and high in 24%. Patients reporting higher levels of physical activity reported significantly lower GFI (p 0.001), functional limitations (p 0.001) and disease activity (p = 0.045) scores than those with less physical activity.Physical activity in RA patients was significantly correlated with functional capacity and levels of fatigue. In order to reduce the proportion of patients with low physical activity, the possibilities for functional training should be expanded and the patients should be encouraged to undertake sporting activities.HINTERGRUND: Patienten mit rheumatoider Arthritis (RA) neigen dazu, körperlich weniger aktiv zu sein. Die körperliche Aktivität wirkt sich positiv auf die Krankheitsaktivität (KA) und Lebensqualität aus und wird von der European League Against Rheumatism (EULAR) als ein Teil der Standardtherapie empfohlen.Eine Querschnittanalyse der RA-Patienten wurde in Bezug auf die KA, die Funktionskapazität (FK) und Fatigue im Zusammenhang mit der körperlichen Aktivität durchgeführt.Die körperliche Aktivität, die FK und der globale Fatigue-Index (Global Fatigue Index [GFI]) wurden mittels standardisierter Fragebögen ermittelt: International Physical Activity Questionnaire-short form (IPAQ-SF), Funktionsfragebogen Hannover (FFbH) und Multidimensional Assessment of Fatigue (MAF). Die Daten wurden mittels SPSS Version 26 (IBM, Armonk, NY, USA) ausgewertet. Die Signifikanzprüfung erfolgte mittels bivariater und partieller Korrelation und nichtparametrischer Tests.Insgesamt wurden 164 Patienten in die Untersuchung eingeschlossen. Die Mehrheit der Patienten war weiblich (127/164; 77 %), das Durchschnittsalter der Kohorte betrug 58,3 (21 bis 86) Jahre. Die durchschnittliche Dauer der krankheitsrelevanten Symptome war 169 (0 bis 713) Monate; 39 % der Patienten zeigten eine niedrige, 37 % eine moderate und 24 % eine hohe körperliche Aktivität. Patienten mit hoher körperlicher Aktivität wiesen die niedrigsten Werte des GFI (p 0,001), eine unbeeinträchtigte FK (p 0,001) und die niedrigste KA (p = 0,045) auf.Ein signifikanter Zusammenhang der körperlichen Aktivität mit der Funktionskapazität und der Ausprägung der Fatigue bei RA-Patienten wurde nachgewiesen. Um den Anteil der Patienten mit niedriger körperlicher Aktivität zu senken, sollten die Möglichkeiten des Funktionstrainings ausgeschöpft und die Patienten zu sportlichen Aktivitäten motiviert werden.

Journal of neurology (2022). doi:10.1007/s00415-022-11431-1, Published by Steinkopff, [Darmstadt]

Patienten mit rheumatoider Arthritis (RA) neigen dazu, korperlich weniger aktiv zu sein. Die korperliche Aktivitat wirkt sich positiv auf die Krankheitsaktivitat (KA) und Lebensqualitat aus und wird von der European League Against Rheumatism (EULAR) als ein Teil der Standardtherapie empfohlen. Eine Querschnittanalyse der RA-Patienten wurde in Bezug auf die KA, die Funktionskapazitat (FK) und Fatigue im Zusammenhang mit der korperlichen Aktivitat durchgefuhrt. Die korperliche Aktivitat, die FK und der globale Fatigue-Index (Global Fatigue Index [GFI]) wurden mittels standardisierter Fragebogen ermittelt: International Physical Activity Questionnaire-short form (IPAQ-SF), Funktionsfragebogen Hannover (FFbH) und Multidimensional Assessment of Fatigue (MAF). Die Daten wurden mittels SPSS Version 26 (IBM, Armonk, NY, USA) ausgewertet. Die Signifikanzprufung erfolgte mittels bivariater und partieller Korrelation und nichtparametrischer Tests. Insgesamt wurden 164 Patienten in die Untersuchung eingeschlossen. Die Mehrheit der Patienten war weiblich (127/164; 77 %), das Durchschnittsalter der Kohorte betrug 58,3 (21 bis 86) Jahre. Die durchschnittliche Dauer der krankheitsrelevanten Symptome war 169 (0 bis 713) Monate; 39 % der Patienten zeigten eine niedrige, 37 % eine moderate und 24 % eine hohe korperliche Aktivitat. Patienten mit hoher korperlicher Aktivitat wiesen die niedrigsten Werte des GFI (p

The work situation is an important dimension of professional life and wellbeing, and a policy lever to strengthen recruitment and retention. This study aims to explore the work situation of physicians and residents in internal medical rheumatology, considering the impact of the coronavirus pandemic COVID-19.A questionnaire-based online survey was conducted in early 2021 at the Hannover Medical School, supported by the German Society of Rheumatology. Target groups were all rheumatology physicians and residents in Germany. The main areas of investigation included work hours, task delegation, and collaboration; workload and mental health issues; discrimination and sexual harassment experiences; and the impact of COVID-19. Descriptive statistical analysis was performed for the standardized items and qualitative content analysis for the free-text information.The respondents (n = 101) expressed positive attitudes towards cooperation and task delegation to medical assistants, especially those specialized in rheumatology, while attitudes towards cooperation with GPs pointed to blockades. There was a strong mismatch between actual and desired work hours both in the group of women and in the group of men. 81% rated their workload as high or very high; every sixth rheumatologist has suffered from stress or burnout syndromes at least once in the past. Experiences of gender discrimination and sexual harassment/violence were frequently reported, mostly by women. COVID-19 was an amplifier of stress, with major stressors being digitalization and increased demand for communication and patient education.There is an urgent need to improve the work situation of rheumatologists and reduce stress and mental health risks.HINTERGRUND UND FRAGESTELLUNG: Die Arbeitssituation ist ein wichtiger Aspekt im Berufsleben und für das Wohlergehen und ein politischer Hebel, um den Verbleib im Beruf sowie die Fachkräfterekrutierung zu verbessern. Ziel dieser Studie war es, die Arbeitsbedingungen internistischer Rheumatolog*innen und Weiterbildungsassistent*innen unter Berücksichtigung der Auswirkungen der Coronavirus-Pandemie COVID-19 zu untersuchen.Eine fragebogenbasierte Online-Erhebung wurde Anfang 2021 an der Medizinischen Hochschule Hannover durchgeführt, unterstützt von der Deutschen Gesellschaft für Rheumatologie. Zielgruppe waren alle Rheumatolog*innen und Weiterbildungsassistent*innen in Deutschland. Thema waren Arbeitszeiten, Kooperation und Delegation, Arbeitsbelastungen und Burnout-Syndrome, Diskriminierung und sexuelle Belästigung sowie Auswirkungen von COVID-19. Standardisierte Items wurden deskriptiv und Freitextinformationen mittels qualitativer Inhaltsanalyse ausgewertet.In der Untersuchungsgruppe (n = 101) zeigten sich positive Einstellungen zu Kooperation und Aufgabendelegation an medizinische Fachangestellte, v. a. Rheumatologische Fachassistenz, während die Einstellungen zur Kooperation mit Hausärzt*innen Blockaden sichtbar machten. Die tatsächliche Arbeitszeit unterschied sich sowohl in der Gruppe der Frauen als auch der Männer sehr deutlich von der Wunscharbeitszeit. Ihre Arbeitsbelastung bewerteten 81 % als hoch oder sehr hoch; jede*r 6. Befragte war mindestens einmal in der Vergangenheit von Stress und Burnout betroffen. Diskriminierungserfahrungen und sexuelle Belästigungen waren weitverbreitet und betrafen Frauen stärker als Männer. COVID-19 erwies sich als Stressverstärker; Digitalisierung, erhöhter Bedarf an Kommunikation und Patientenaufklärung waren wesentliche Stressfaktoren.Die Arbeitssituation von Rheumatolog*innen sollte dringend verbessert werden, um Stress und Gesundheitsrisiken zu verringern.

Background and purpose The phenotype of Sjogren's syndrome-associated neuropathy has been better characterized in recent years. However, Sjogren's syndrome-associated neuropathy remains an underdiagnosed entity with only few insights considering the pathomechanisms of nerve damage. Nerve ultrasound has proven to be a useful and efficient tool in detecting nerve damage of autoimmune origin. We, therefore, aimed to evaluate this method for Sjogren's syndrome-associated neuropathy. Methods Patients with Sjogren's syndrome and clinical signs of neuropathy underwent sonographic examination of both median and ulnar nerves. Nerve thickening was classified for cross-sectional areas of >12 mm² at the median nerve and for >10 mm² at the ulnar nerve. Fascicle thickening was documented for cross-sectional areas ≥5 mm² at the median and ≥3 mm² at the ulnar nerve. Results Forty-three patients were included in the analysis (median age 60 years [interquartile range 53-73 years], female rate 60%). 31/43 patients (72%) showed abnormalities on nerve ultrasound, while nerve thickening was found more frequently than fascicle thickening (90% vs. 52% of patients with sonographic abnormalities, respectively). Abnormal findings were observed more frequently at the median nerve and in proximal localization. Abnormal findings on nerve conduction studies were evident in 36/43 patients (84%). Nerve conduction studies revealed a tendency of demyelinating nerve damage patterns being associated with abnormal findings on nerve ultrasound. Conclusions In addition to nerve conduction studies, nerve ultrasound may have a supporting role in the diagnosis of Sjogren's syndrome-associated neuropathy. Also, our data support an immune-mediated inflammatory demyelinating pathogenesis of Sjogren's syndrome-associated neuropathy.

Ein 64-jahriger Patient entwickelte uber 20 Jahre zunehmende sensible und motorische Schadigungen der Arm- und Beinnerven. Histologisch wurde mittels Suralisbiopsie eine schwere axonale Neuropathie gesichert. Die Genese konnte trotz ausfuhrlicher Laboruntersuchungen inklusive immunologischer und metabolischer Tests nicht identifiziert werden. Schlieslich wurde eine reduzierte Tranenproduktion durch einen pathologischen Schirmer-Test nachgewiesen. Eine Speicheldrusenbiopsie aus der Unterlippe zeigte eine lymphozytare Entzundung, Grad III nach Chisholm und Mason, sodass ein Sjogren-Syndrom diagnostiziert wurde.

Health workforce shortage in German rheumatology has been identified as a healthcare service and delivery problem. Health policy has increased staffing targets, yet effective intervention strategies are lacking. This research aimed to systematically map the rheumatology workforce to improve the evidence for interventions and explore possibilities for more effective health workforce management.The WHO National Health Workforce Accounts provided a conceptual framework for the mapping exercise. Four major sets of indicators were selected, comprising staffing levels, health labor market flows, composition and education/training. A comparison of age groups and time series was applied to explore trends. Public statistics and other secondary sources served our analysis using descriptive methodology.In Germany there are 1076 physicians specialized in internal medical rheumatology. Absolute numbers have nearly doubled (91%) since 2000 but with a strong demographic bias. Between 2000 and 2019 numbers markedly increased in the group aged 50 years and older but only by 9% in the younger group under 50 years; since 2010 the group aged 40-50 years even faces a decrease. In 2019, the absolute numbers of rheumatologists in retirement age exceeded those aged 40 years and under. Since 2015 an expanding workforce trend has overall flattened but this was strongest in the hospital sector; the numbers in resident training did not show any relevant growth.Health workforce trends reveal that an available number of rheumatologists cannot meet new health policy planning targets. There is a need for effective health workforce management, focusing on innovation in resident training, improved task delegation and gender equality.HINTERGRUND UND FRAGESTELLUNG: Fachkräftemangel in der Rheumatologie in Deutschland ist als Versorgungsproblem erkannt. Die Gesundheitspolitik hat mit neuen Planungszielen reagiert, aber es fehlen effektive Interventionsstrategien. Ziel dieser Studie ist ein systematischer berufsstruktureller Überblick, um die Grundlage für Interventionen zu verbessern und Möglichkeiten für ein effektives Fachkräftemanagement aufzuzeigen.Die WHO National Health Workforce Accounts (NHWA) dienen als konzeptioneller Rahmen. Ausgewählt werden 4 Indikatoren: Personalbestand, Arbeitsmarktbewegungen, Komposition und Weiterbildung. Die Exploration von Entwicklungstrends stützt sich auf vergleichende Analysen von Altersgruppen und Zeitreihen. Die Erhebung nutzt öffentliche Statistiken und andere Sekundärliteratur; die Auswertung erfolgt deskriptiv.In Deutschland sind 1076 Ärzt*innen mit einer Facharztqualifikation oder Schwerpunktbezeichnung in der internistischen Rheumatologie ärztlich tätig. Die absolute Zahl verdoppelte sich seit 2000 deutlich (91 %), aber mit einem demografischen Bias. Im Zeitraum 2000 bis 2019 stieg die Zahl der über 50-Jährigen deutlich, aber die der unter 50-Jährigen nur um 9 %; seit 2010 sind die Zahlen in der Gruppe 40 bis 50 Jahre rückläufig. Im Jahr 2019 waren mehr Rheumatolog*innen im Rentenalter als unter 40-Jährige ärztlich tätig. Seit 2015 schwächt sich der steigende Trend insgesamt ab, aber am stärksten im Krankenhaussektor; die Weiterbildungen lassen keine konstante Steigerung erkennen.Berufsstrukturelle Trends zeigen, dass die gesundheitspolitischen Planziele mit den verfügbaren Humanressourcen nicht zu erreichen sind. Gefordert ist ein besseres Fachkräftemanagement, insbesondere durch Innovation der Weiterbildung, Aufgabenverschiebung und verbesserte Geschlechtergerechtigkeit.

This study addresses the challenging characterisation and differentiation of CIDP versus CIDP in association with Sjögren's syndrome to facilitate the process in clinical routine.Patients with both CIDP and Sjögren's syndrome and CIDP without Sjögren's syndrome were compared concerning relevant differences in clinical, laboratory and electrophysiological findings. 154 patients who fulfilled the diagnostic EFNS/PNS criteria for CIDP were included in the analysis. 54 of these patients additionally fulfilled the ACR/EULAR classification criteria for Sjögren's syndrome.The frequency of female patients was higher in patients with CIDP and Sjögren's syndrome (52%) versus CIDP patients without Sjögren's syndrome (28%). Furthermore, the occurrence of cranial nerve impairment was significantly higher in patients with Sjögren's syndrome (39% versus 14%). There were no significant group differences in the evaluation of initial symptoms, severity of disability judged by INCAT disability scale score, presence or distribution of sensory deficits, limb weakness and the presence of ataxia, pain or dysautonomia, CSF laboratory or electrophysiological findings.In conclusion, our data indicate that cranial nerve impairment and female gender might represent red flags for an additional Sjögren's syndrome in patients with CIDP. The patterns of clinical disabilities and electrophysiological findings due to peripheral nerve damage are similar in both CIDP entities.

Background The importance of a sustainable health workforce is increasingly recognised. However, the specialised heath workforce remains marginal in health workforce planning, policy and research. This paper aims to improve the integration of small specialised professional groups in health workforce governance and to critically assess existing tools, using Germany and rheumatology physicians as a case study. Methods A qualitative explorative approach was applied, which draws on a scoping review of the literature, public statistics and expert information. The National Health Workforce Accounts (NHWA) and the WHO 'AAAQ' framework served as guidance to assess workforce development in rheumatology in Germany. Results On average physician density is high in Germany, yet access to specialised care may be seriously delayed and services may not be accessible to all patients; e.g. currently only half of the patients with spondyloarthritis are treated by a rheumatologist which impacts quality of care. An estimated 850 to 1000 additional rheumatologists are needed to ensure early diagnoses and treatment and prevent long-term health damage. However, shortage will even reinforce in future: annual inflow of newly registered rheumatologists over recent years did not exceed 30 to 50 physicians and young rheumatologists more often prefer part-time work. Conclusions Specialised health professions with small numbers and long duration of training face specific challenges to create a sustainable workforce. This calls for (1) greater investment in education and training, (2) greater attention to qualitative indicators, such as assess and prevention measurements like early diagnosis, and (3) a dynamic health workforce approach to respond to both new technologies/ treatments and changing preferences of the health professionals, like part-time work. Key messages Integrating the specialised health workforce in health workforce governance is important to improve prevention, quality and access to care. NHWA may be aligned to qualitative indicators and prevention policies to respond more effectively to future population needs.

BackgroundSerology testing is explored for epidemiological research and to inform individuals after suspected infection. During the COVID-19 pandemic, frontline healthcare professionals (HCP) may be at particular risk for infection. No longitudinal data on functional seroconversion in HCP in regions with low COVID-19 prevalence and low pre-test probability exist.MethodsIn a large German university hospital, we performed weekly questionnaire assessments and anti-SARS-CoV-2 IgG measurements with various commercial tests, a novel surrogate virus neutralization test, and a neutralization assay using live SARS-CoV-2.ResultsFrom baseline to week six, n=1,080 screening measurements for anti-SARS CoV-2 (S1) IgG from n=217 frontline HCP (65% female) were performed. Overall, 75.6% of HCP reported at least one symptom of respiratory infection. Self-perceived infection probability declined over time (from mean 20.1% at baseline to 12·4 % in week six, pConclusionWhen assessing anti-SARS-CoV-2 immune status in individuals with low pre-test probability, we suggest confirming positive results from single measurements by alternative serology tests or functional assays. Our data highlight the need for a methodical serology screening approach in regions with low SARS-CoV-2 infection rates.

Uncertain rates of asymptomatic infections have raised concerns about potentially high rates of thus far undiagnosed SARS-CoV-2 infections. Serological testing for SARS-CoV-2 specific IgG can be helpful in identification of asymptomatic infections. We report baseline results of the CVOID-19 Contact (CoCo) Study, which follows 217 frontline healthcare workers at a university hospital and performs weekly SARS-CoV-2 specific serology (IgA/IgG). The majority of participants had direct contact to patients with infectious respiratory diseases. Study participants estimated their personal likelihood of having had a SARS-CoV-2 infection with a mean of 20.9% (range 0 to 90%). In contrast, anti-SARS-CoV-2-IgG prevalence was in the range of 1-2% among health care workers. The CoCo Study is not fully representative for other hospitals and the sensitivity of anti-SARS-CoV-2 serology in low prevalence conditions may require further improvement. Taken together, low rates of SARS-CoV-2 specific IgG in healthcare workers in Northern Germany are in sharp contrast to the high personal risk perception. Regular anti-SARS-CoV-2 IgG testing of health-care workers may aid in monitoring the pandemic, assessing the quality of immune responses, directing resources for protective measures, and assuring CVID-19 care in the long run.

There have been concerns about high rates of thus far undiagnosed SARS-CoV-2 infections in the health-care system. The COVID-19 Contact (CoCo) Study follows 217 frontline health-care professionals at a university hospital with weekly SARS-CoV-2-specific serology (IgA/IgG). Study participants estimated their personal likelihood of having had a SARS-CoV-2 infection with a mean of 21% [median 15%, interquartile range (IQR) 5–30%]. In contrast, anti-SARS-CoV-2 IgG prevalence was about 1–2% at baseline. Regular anti-SARS-CoV-2 IgG testing of health-care professionals may aid in directing resources for protective measures and care of COVID-19 patients in the long run.

The phenotype of primary immunodeficiency disorders (PID), and especially common variable immunodeficiency (CVID), may be dominated by symptoms of autoimmune disorders. Furthermore, autoimmunity may be the first manifestation of PID, frequently preceding infections and the diagnosis of hypogammaglobulinemia, which occurs later on. In this case, distinguishing PID from hypogammaglobulinemia secondary to anti-inflammatory treatment of autoimmunity may become challenging. The aim of this study was to evaluate the diagnostic accuracy of peripheral blood lymphocyte phenotyping in resolving the diagnostic dilemma between primary and secondary hypogammaglobulinemia. Comparison of B and T cell subsets from patients with PID and patients with rheumatic disease, who developed hypogammaglobulinemia as a consequence of anti-inflammatory regimes, revealed significant differences in proportion of na&iuml, ve B cells, class-switched memory B cells and CD21low B cells among B cells as well as in CD4+ memory T cells and CD4+ T follicular cells among CD4+ T cells. Identified differences in B cell and T cell subsets, and especially in the proportion of class-switched memory B cells and CD4+ T follicular cells, display a considerable diagnostic efficacy in distinguishing PID from secondary hypogammaglobulinemia due to anti-inflammatory regimens for rheumatic disease.

Objective Extraglandular neurological manifestations of Sjögren’s syndrome are increasingly recognized, defining the disease entity of Neuro‐Sjögren. Neuropsychological assessment of patients with Sjögren’s syndrome has hitherto been performed on predominantly rheumatological cohorts. These studies revealed a wide variety of prevalence rates for cognitive impairment (22–80%), while variable cut‐off criteria for detection of cognitive impairment were applied. Attentional functions have not yet been thoroughly investigated in these patients, although they clearly represent relevant aspects of cognitive functioning in daily life. Methods We therefore conducted extensive neuropsychological assessment based on two neuropsychological test batteries [i.e., the extended German version of the Consortium to Establish a Registry for Alzheimer’s Disease Neuropsychological Assessment Battery (CERAD‐PLUS), and the test battery for attentional performance (TAP) as a well‐established assessment of attentional functions in the German‐speaking part of Europe]. Results Sixty‐four patients with Neuro‐Sjögren, who were treated at our university hospital between December 2016 and January 2019, were included. Evidence for the presence of cognitive impairment was found in 55% of patients with Neuro‐Sjögren. The degree of cognitive impairment ranged from mild (38%) to severe (17%). Attentional and mnemonic subtests showed pronounced cognitive impairment in patients with Neuro‐Sjögren. Interpretation Our results suggest that a substantial proportion of patients with Neuro‐Sjögren suffer from cognitive impairment, putatively as a corollary of attentional deficits, which might exert adverse effects on occupational abilities, other cognitive functions, and social role functioning.

Introduction The way syphilis affects the immunologic and virologic parameters of a human immunodeficiency virus (HIV) infection remains controversial. The aim of this study was to investigate the impact of syphilis infection on lymphocyte and lymphocyte subset counts as well as viral load in HIV-infected patients. Methods All HIV-infected patients attending the outpatient clinic for infectious diseases of Hannover Medical University Hospital diagnosed with syphilis between 2009 and 2016 were retrospectively evaluated for changes in total lymphocyte, B cell, CD3+ T cell, CD4+ and CD8+ T cell counts as well as in HIV viral load. These parameters were assessed at three different time points, i.e., 3–6 months before, at diagnosis and 3–6 months after treatment of syphilis. Results Eighty-four HIV-infected patients, all with early syphilis, were identified. The vast majority were men who have sex with men (MSM), and 80% were receiving antiretroviral therapy (ART). Syphilis was associated with a significant reduction in the total lymphocyte count and counts of all studied lymphocyte subsets, including CD4+ T cells, whose percentage among lymphocytes did not change. No significant changes in HIV viral load were observed at any of the studied time points. Further, antibiotic treatment of syphilis restored lymphocyte counts back to pretreatment levels. Conclusion Syphilis induces a relative non-CD4+ T cell-specific lymphopenia in HIV-infected patients. Our data suggest that serologic testing for syphilis should be considered in HIV-infected MSM in case of an otherwise unexplained drop in total lymphocyte count. Electronic supplementary material The online version of this article (10.1007/s40121-018-0219-9) contains supplementary material, which is available to authorized users.

Background and aims Atherosclerosis is a chronic inflammatory process of vessel walls responsible for coronary, cerebrovascular and peripheral vascular disease, which together account for the majority of non-infective global deaths. Whilst great emphasis has been placed on lifestyle factors, a growing body of evidence supports an autoimmune component to atherosclerosis. This study evaluates a novel autoantibody against MYC-associated zinc finger protein (MAZ-Ab) as a potential marker of atherosclerosis. It compares MAZ-Ab to activity on whole-body positron-emission tomography/computed tomography (PET/CT) attributable to atherosclerosis. Methods Antibody screening using protein arrays was performed in patients with angiographically-proven ischaemic heart disease. Following MAZ-Ab detection, an ELISA for large-scale testing was developed. An a priori group of unselected patients attending for unrelated 18 F-fluorodeoxyglucose (FDG) PET/CT was prospectively enrolled. Each completed a structured questionnaire under supervision and provided serum for analysis. PET/CT scans were evaluated for inflammatory arterial lesions. Whole-body arterial inflammatory burden was then correlated with ELISA optical density for MAZ-Ab. Results Protein array testing identified IgG anti-MAZ antibodies in 4/6 (67%) patients with ischemic heart disease, versus 0/10 controls. Significant positive correlations between MAZ-Ab and both increasing number of PET positive inflammatory atherosclerostic lesions ( p = 0.023) and whole-body arterial inflammatory burden ( p = 0.002) were shown. No traditional atherosclerotic risk factor correlated with MAZ-Ab. Conclusions A quantitative association between MAZ-Ab optical density on ELISA and the cumulative inflammatory burden of atherosclerosis on 18 F-FDG PET/CT could be shown. These findings provide further evidence for an autoimmune component in atherosclerosis and suggest MAZ-Abs as a potential biomarker for atherosclerotic disease.

Background: Currently, half of the population displaced worldwide is children and adolescents. Little is known on healthcare demand in underage migrants. Materials and Methods: We analyzed healthcare utilization in n = 1.411 children and adolescents living in a large German refugee reception in 2015-2016. Results: The mean age of all included refugees was 9 years (60.8% male). The majority came from the eastern Mediterranean region. During a mean camp inhabitance of 34 days, 57.6% needed primary healthcare, with a significant inverse correlation of healthcare seeking frequency with age and duration of camp inhabitance. Infants and unaccompanied minors displayed particular high demands for medical help. Discussion: Our analysis showed that pediatric primary healthcare in pediatric and adolescent refugees are most sought during the first period upon arrival with particular demand in infants, toddlers, and unaccompanied minors. Based on this data, future care taking strategies should be adapted accordingly.

Genetic studies have led to identification of an increasing number of monogenic primary immunodeficiency disorders. Monoallelic pathogenic gain-of-function (GOF) variants in NFKBIA, the gene encoding IκBα, result in an immunodeficiency disorder, typically accompanied by anhidrotic ectodermal dysplasia (EDA). So far, 14 patients with immunodeficiency due to NFKBIA GOF mutations have been reported. In this study we report three patients from the same family with immunodeficiency, presenting with recurrent respiratory tract infections, bronchiectasis and viral skin conditions due to a novel pathogenic NFKBIA variant (c.106 T > G, p.Ser36Ala), which results in reduced IκBα degradation. Immunological investigations revealed inadequate antibody responses against vaccine antigens, despite hypergammaglobulinemia. Interestingly, none of the studied patients displayed features of EDA. Therefore, missense NFKBIA variants substituting serine 36 of IκBα, differ from the rest of pathogenic GOF NFKBIA variants in that they cause combined immunodeficiency, even in the absence of EDA.

Immunodeficiency, centromeric instability and facial anomalies syndrome 2 (ICF2) is a rare autosomal recessive primary immunodeficiency disorder. So far, 27 patients have been reported. Here, we present three siblings with ICF2 due to a homozygous ZBTB24 gene mutation (c.1222 T&gt, G, p. (Cys408Gly)). Immune deficiency in these patients ranged from late-onset combined immunodeficiency (CID) with severe respiratory tract infections and recurrent shingles to asymptomatic selective antibody deficiency. Evident clinical heterogeneity manifested despite a common genetic background, suggesting the pathogenic relevance of epigenetic modification. Immunological follow-up reveals a previously unidentified gradual depletion of B and CD4+ T cells in all three presented patients with transition of a common variable immunodeficiency (CVID)-like disease to late-onset-CID in one of them. Considering all previously published cases with ICF2, we identify inadequate antibody responses to vaccines and reduction in CD27+ memory B cells as prevalent immunological traits. High mortality among ICF2 patients (20%) together with the progressive course of immunodeficiency suggest that hematopoietic stem cell transplantation (HSCT) should be considered as a treatment option in due time.

Background: Coronary heart disease is a very common comorbidity in rheumatological diseases. Risk factors predisposing to ACS are well established, but no reliable prognostic biomarkers for post-event survival or risk of reinfarction exist. Physiological levels of antibodies against anti-beta1 adrenergic[for full text, please go to the a.m. URL], 47. Kongress der Deutschen Gesellschaft für Rheumatologie (DGRh), 33. Jahrestagung der Deutschen Gesellschaft für Orthopädische Rheumatologie (DGORh), 29. Jahrestagung der Gesellschaft für Kinder- und Jugendrheumatologie (GKJR)