Objective: Gastroparesis may be present in individuals with systemic sclerosis. In the United States, metoclopramide is the only medication approved for treating gastroparesis. Our case involves using mirtazapine therapy to help with weight loss and gastroparesis associated with systemic sclerosis. Case: A 70-year-old female with limited systemic sclerosis and sicca syndrome began experiencing weight loss, dysphagia, nausea, and abdominal fullness. Neither an esophageal dilation procedure nor six weeks of metoclopramide therapy alleviated her symptoms. However, 15 mg of mirtazapine once daily provided some symptomatic relief. A gastric emptying scan confirmed gastroparesis. The dose of mirtazapine was later increased to 30 mg once daily. With the mirtazapine therapy, the patient experienced both symptomatic improvement and weight gain benefits. Discussion/Conclusion: Mirtazapine therapy has anti depressive, appetite stimulating, anti-emetic, and prokinetic benefits. Consideration of mirtazapine therapy for patients with weight loss and gastroparesis associated with systemic sclerosis may be beneficial.

UNASSIGNED: The Pediatric Rheumatology Care and Outcomes Improvement Network (PR-COIN) is a North American learning health network focused on improving outcomes of children with juvenile idiopathic arthritis (JIA). JIA is a chronic autoimmune disease that can lead to morbidity related to persistent joint and ocular inflammation. PR-COIN has a shared patient registry that tracks twenty quality measures including ten outcome measures of which six are related to disease activity. The network\'s global aim, set in 2021, was to increase the percent of patients with oligoarticular or polyarticular JIA that had an inactive or low disease activity state from 76% to 80% by the end of 2023.
UNASSIGNED: Twenty-three hospitals participate in PR-COIN, with over 7,200 active patients with JIA. The disease activity outcome measures include active joint count, physician global assessment of disease activity, and measures related to validated composite disease activity scoring systems including inactive or low disease activity by the 10-joint clinical Juvenile Arthritis Disease Activity Score (cJADAS10), inactive or low disease activity by cJADAS10 at 6 months post-diagnosis, mean cJADAS10 score, and the American College of Rheumatology (ACR) provisional criteria for clinical inactive disease. Data is collated to measure network performance, which is displayed on run and control charts. Network-wide interventions have included pre-visit planning, shared decision making, self-management support, population health management, and utilizing a Treat to Target approach to care.
UNASSIGNED: Five outcome measures related to disease activity have demonstrated significant improvement over time. The percent of patients with inactive or low disease activity by cJADAS10 surpassed our goal with current network performance at 81%. Clinical inactive disease by ACR provisional criteria improved from 46% to 60%. The mean cJADAS10 score decreased from 4.3 to 2.6, and the mean active joint count declined from 1.5 to 0.7. Mean physician global assessment of disease activity significantly improved from 1 to 0.6.
UNASSIGNED: PR-COIN has shown significant improvement in disease activity metrics for patients with JIA. The network will continue to work on both site-specific and collaborative efforts to improve outcomes for children with JIA with attention to health equity, severity adjustment, and data quality.

OBJECTIVE: Two-dimensional shear wave elastography (2D-SWE) is a non-invasive technique for the evaluation of Sjögren\'s disease (SjD). This study investigated the diagnostic accuracy of 2D-SWE in assessing major salivary gland involvement in SjD.
METHODS: A systematic review and meta-analysis.
METHODS: Data were obtained by searching PubMed, MEDLINE, EMBASE, Scopus, Cochrane library and CNKI from 1999 to 26 September 2022, which includes randomised clinical trial of 2D-SWE for the diagnosis of SjD.
METHODS: (1) Patients (≥18 years old) diagnosed with SjD following the international classification in 2002 or 2016 American College of Rheumatology-European League Against Rheumatism classification criteria for SjD; (2) The purpose of this study was to evaluate the diagnostic value of 2D-SWE in SjD; (3) The evaluation parameters for the diagnosis of SjD can be extracted or indirectly obtained in this article, including sensitivity, specificity, true positive, false positive, false negative, true negative, diagnostic point (Young\'s modulus) and other data.
METHODS: Four authors independently screened and assessed the literature and extracted the data. RevMan V.5.3 and StataMP V.18 software were used for quality assessment and meta-analysis.
RESULTS: We included 8 studies with a total of 912 cases, including 509 patients with SjD. The high-risk bias in the quality evaluation focused on patient selection and index test. The pooled sensitivity, specificity and summary area under the curve of 2D-SWE were 0.75 (95% CI 0.62 to 0.84), 0.89 (95% CI 0.80 to 0.94) and 0.90 (95% CI 0.87 to 0.92), respectively.
CONCLUSIONS: 2D-SWE has an acceptable diagnostic accuracy for SjD patients and is an effective tool for auxiliary diagnosis of SjD.
UNASSIGNED: CRD42022365766.

BACKGROUND: Childhood-onset systemic lupus erythematosus (c-SLE) is a multifaceted autoimmune disorder predominantly affecting the musculoskeletal (MSK) system. This investigation delineated the spectrum and sequelae of MSK involvement in c-SLE patients.
METHODS: This retrospective analysis included SLE patients aged ≤ 18 years treated at a tertiary center between 2009 and 2019. Data were extracted from electronic health records.
RESULTS: The cohort comprised 321 SLE patients (mean age 13.2 ± 2.5 years, 91.3% female). MSK manifestations were observed in 134 (41.7%) individuals, with joint pain universally present, followed by joint swelling in 32.1% and morning stiffness in 9.7%. Arthritis was documented in 52 (38.8%) patients, whereas 82 (61.2%) had arthralgia. Symmetrical joint involvement was observed in 96 (71.7%) subjects. The knees, wrists, and fingers were most commonly affected, with incidences of 43.3%, 40.3%, and 33.6%, respectively. Neither erosive arthritis nor Jaccoud\'s arthropathy was detected. MSK symptoms were significantly correlated with older age at diagnosis, the presence of non-scarring alopecia, neuropsychiatric manifestations, and elevated SLE disease activity index scores at diagnosis. Over a median follow-up of 53.6 months (IQR 26.1-84.6), five patients developed septic arthritis or osteomyelitis, and avascular necrosis was identified in 16 (4.9%) patients.
CONCLUSIONS: Nearly half of c-SLE patients demonstrated MSK manifestations, chiefly characterized by symmetrical involvement of both large and small joints without evidence of erosive arthritis or Jaccoud\'s arthropathy. Avascular necrosis is a critical concern and warrants close monitoring.

Advances in DNA sequencing technologies, especially next-generation sequencing (NGS), which is the basis for whole-exome sequencing (WES) and whole-genome sequencing (WGS), have profoundly transformed immune-mediated rheumatic disease diagnosis. Recently, substantial cost reductions have facilitated access to these diagnostic tools, expanded the capacity of molecular diagnostics and enabled the pursuit of precision medicine in rheumatology. Understanding the fundamental principles of genetics and diversity in genetic variant classification is a crucial milestone in rheumatology. However, despite the growing availability of DNA sequencing platforms, a significant number of autoinflammatory diseases (AIDs), neuromuscular disorders, hereditary collagen diseases, and monogenic bone diseases remain unsolved, and variants of uncertain significance (VUS) pose a formidable challenge to addressing these unmet needs in the coming decades. This article aims to provide an overview of the clinical indications and interpretation of comprehensive genetic testing in the medical field, addressing the related complexities and implications.

Renal tubular acidosis is a well-known consequence of primary Sjogren\'s syndrome (pSS), but a rare manifestation similar to acute pancreatitis in pSS. Here, we discuss the case of a woman in her 50s, who presented to a tertiary care hospital with recurrent episodes of sudden-onset weakness in all four limbs, recurrent vomiting and epigastric pain. She had non-anion gap metabolic acidosis with hypokalaemia and was diagnosed with pSS with hypokalaemic periodic paralysis. She was also diagnosed with acute pancreatitis based on elevated amylase and lipase levels and CT findings. The article highlights the diverse spectrum of clinical manifestations of pSS, including renal and pancreatic involvements, which can be rare consequences of the disease.

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UNASSIGNED: Healthcare professionals (HCPs) need to identify potentially serious musculoskeletal (MSK) presentations in children and refer them to specialists appropriately. Our aim was to develop \'pGALSplus\' (paediatric gait, arms, legs and spine plus) to support clinical assessment, aid decision-making and assess feasibility and acceptability in exemplar MSK pathologies.
UNASSIGNED: We used a three-phase mixed methods approach: phase 1, preliminary stakeholder engagement and scoping review to propose pGALSplus; phase 2, iterative development of pGALSplus involving an expert working group; and phase 3, testing the feasibility of pGALSplus in exemplar MSK conditions [JIA, mucopolysaccharidoses (MPS), muscular dystrophy (MD), developmental coordination disorder (DCD) and healthy controls (HCs)]. The final pGALSplus was derived from analysis of phase 3 data and feedback from HCPs, families and expert consensus input from an international e-survey (n = 22) and virtual event (n = 13).
UNASSIGNED: Feasibility was tested in 45 children (JIA, n = 10; MPS, n = 6; MD, n = 9; DCD, n = 10; HCs, n = 10). Overall the assessment was achievable in the target age range (2-10 years) and quick to complete [median 12 min (range 8-20)], with high acceptability from families. Expert feedback deemed pGALSplus to be very useful and of particular use to non-specialists in MSK paediatrics. The final pGALSplus comprises 26 clinical observations/skills with a colour-coding approach to aid decision-making and identification of more serious MSK presentations and additional resources to support its use in clinical practice.
UNASSIGNED: pGALSplus is a novel evidence- and consensus-based assessment building on pGALS, with high acceptability and feasibility. As community-based MSK assessment in children becomes more established, we propose that pGALSplus will facilitate and inform decision-making to promote access to specialist care.

OBJECTIVE: To determine the occurrence of breakthrough COVID-19 infections (BIs) in patients with systemic lupus erythematosus (SLE) compared with patients with other rheumatic autoimmune diseases (rAIDs), patients with non-rheumatic autoimmune diseases (nrAIDs), and healthy controls (HCs).
METHODS: The study was based on data from 7035 fully vaccinated respondents to the online COVAD questionnaire with SLE (N = 852), rAIDs (N = 3098), or nrAIDs (N = 414), and HCs (N = 2671). BI was defined as COVID-19 infection occurring in individuals vaccinated with ≥ 2 doses (or 1 dose of J&J) ≥ 14 days after vaccination and not after 6 months since the last vaccine dose. Data were analysed using linear and logistic regression models.
RESULTS: A total of 91/852 (10.7%) SLE patients reported at least one BI. The frequency of BIs in SLE patients was comparable to that among HCs (277/2671; p = 0.847) and patients with nrAID (39/414; p = 0.552) but higher than that among patients with other rAIDs (235/3098; p = 0.005). No demographic factors or treatments were associated with BIs in SLE patients (p ≥ 0.05 for all). Joint pain was more frequent in SLE patients than in HCs (odds ratio [OR]: 3.38; 95% confidence interval [CI]: 1.89-6.04; p < 0.001) or nrAID patients (OR: 2.44; 95% CI: 1.04-5.75; p = 0.041). Patient with SLE did not report a higher frequency of hospitalisation or need for advanced treatment for COVID-19 infection compared with disease controls and HCs, respectively.
CONCLUSIONS: COVID-19 vaccination conferred similar protection against COVID-19 infection in terms of frequency and severity in patients with SLE to that reported by healthy individuals.

BACKGROUND: Rheumatology in Germany is facing major challenges. The need for rheumatological care is increasing and can no longer be met in some regions for capacity reasons. Too many people with an inflammatory rheumatic disease (IRD) have to forego appropriate care or receive it too late. The 4th new edition of the memorandum of the German Society for Rheumatology and Clinical Immunology (DGRh) provides information on rheumatological care in Germany. It was produced under the leadership of the DGRh together with the Professional Association of German Rheumatologists (BDRh), the Association of Acute Rheumatology Clinics (VRA), the German Rheumatism League (DRL) and the German Rheumatism Research Center (DRFZ).
METHODS: The memorandum describes the current state and development of the following areas: number of people with IRD, outpatient, inpatient and rehabilitative care structures, number of specialists in rheumatology, education and training, quality of care, health economic aspects and digital care concepts. Proposals for health policy measures to safeguard rheumatological care are presented.
RESULTS: Prevalence: approximately 1.8 million adults in Germany have an IRD. The prevalence is increasing, due to changes in the demographic structure of the population, improved diagnostics, treatment and longer survival. Care structures: outpatient specialist care (ASV) for rheumatic diseases is developing as a cross-sectoral care model for hospital outpatient clinics and rheumatology practices. Hospitals have been able to be certified as rheumatology centers since 2020, which enables structural developments. Specialists in rheumatology: as of 31 December 2023, there were 1164 specialists in rheumatology working in Germany. This included 715 physicians accredited to work in practices for national health assurance patients, 39% of whom were employees. In hospitals, 39% of doctors worked part-time. At least 2 rheumatology specialists per 100,000 adults are needed, i.e. around 1400, in order to provide adequate care. This means that there is a shortage of around 700 rheumatology specialists in the outpatient sector alone. Of all working specialists, 30% are currently aged 60 years old and over. Medical training: only 10 out of 38 (26%) state universities have an independent chair in rheumatology. In addition, 11 rheumatology departments are subordinate to a nonrheumatology chair. In the rheumatology-integration into student training (RISA) III study, only 16 out of 36 faculties fulfilled the recommended minimum number of compulsory hours of student rheumatology teaching. Continuing education in rheumatology: the annual postgraduate training qualifications do not cover the demand for rheumatology specialists, which is additionally increasing due to intensified workload, reduced capacities through retirement, and part-time work. Quality of care: since the introduction of highly effective medication patients with IRD have a much better chance of achieving remission of their disease. With early initiation of targeted therapy, the lives of many patients are hardly restricted at all: however, waiting times for a first rheumatological visit often last more than 3 months. Quality target is a first consultation within the first 6 weeks after the onset of symptoms. Models for early consultation, delegation of medical services, structured patient training and digital care concepts have been positively evaluated but are not covered financially.
RESULTS: the total annual costs for inflammatory joint diseases alone amount to around 3 billion euros. The direct costs have significantly risen since the introduction of biologics, while the indirect costs for sick leave, disability and hospitalization have fallen.
CONCLUSIONS: The core demands of this memorandum are a significant and sustainable increase in the number of further training positions in the outpatient and inpatient sector, the creation of chairs or at least independent departments for rheumatology at all universities and the further implementation of new and cross-sectoral forms of care. This will ensure modern needs-based rheumatological care for all patients in the future.
UNASSIGNED: HINTERGRUND: Die Rheumatologie in Deutschland steht vor großen Herausforderungen: der Bedarf an rheumatologischer Versorgung steigt und kann aus Kapazitätsgründen bereits jetzt in einigen Regionen nicht mehr gedeckt werden. Zu viele Menschen mit einer entzündlich-rheumatischen Erkrankung (ERE) müssen auf eine angemessene Versorgung verzichten oder erhalten diese zu spät. Die 4. Neuauflage des Memorandums der Deutschen Gesellschaft für Rheumatologie und Klinische Immunologie e. V. (DGRh) informiert über die rheumatologische Versorgung in Deutschland. Es wurde unter Führung der DGRh mit dem Berufsverband Deutscher Rheumatologen (BDRh), dem Verband Rheumatologischer Akutkliniken (VRA), der Deutschen Rheuma-Liga (DRL) und dem Deutschen Rheuma-Forschungszentrum (DRFZ) erstellt.
METHODS: Das Memorandum beschreibt den aktuellen Stand und die Entwicklung folgender Bereiche: Anzahl der Personen mit ERE, ambulante, stationäre und rehabilitative Versorgungsstrukturen, Anzahl an Fachärzt:innen für Rheumatologie, Aus- und Weiterbildung, Versorgungsqualität, gesundheitsökonomische Aspekte und digitale Versorgungskonzepte. Vorschläge für gesundheitspolitische Maßnahmen zur Sicherung der rheumatologischen Versorgung werden dargestellt.
UNASSIGNED: Prävalenz: Etwa 1,8 Mio. Erwachsene in Deutschland haben eine ERE. Die Prävalenz steigt aus verschiedenen Gründen: Veränderungen der Altersstruktur der Bevölkerung, verbesserte Diagnostik und Therapie mit längerem Überleben. Versorgungsstrukturen: Neben der regulären kassenärztlichen Versorgung hat sich die ambulante spezialfachärztliche Versorgung (ASV) als sektorenübergreifendes Versorgungsmodell etabliert. Krankenhäuser können sich seit 2020 als rheumatologische Zentren zertifizieren lassen, was strukturelle Weiterentwicklungen ermöglicht. Fachärzt:innen (FÄ) für Rheumatologie: Zum 31.12.2023 waren in Deutschland 1164 FÄ für Rheumatologie berufstätig. Vertragsärztlich waren dies 715 FÄ, davon 39 % angestellt. In Krankenhäusern waren 39 % der FÄ in Teilzeit tätig. Für eine bedarfsgerechte ambulante Versorgung benötigen wir mindestens 2 FÄ für Rheumatologie pro 100.000 Erwachsene, das sind rund 1400. Es fehlen also allein im ambulanten Bereich zum jetzigen Zeitpunkt etwa 700 FÄ für Rheumatologie. Von allen berufstätigen FÄ sind 30 % derzeit 60 Jahre und älter. Ärztliche Ausbildung: Nur 10 von 38 (26 %) staatlichen Universitäten verfügen über einen eigenständigen rheumatologischen Lehrstuhl. Darüber hinaus sind 11 rheumatologisch geführte Abteilungen einem nicht-rheumatologischen Lehrstuhl untergeordnet. Nur 16 von 36 Fakultäten erfüllten in der RISA III-Studie die empfohlene Mindestzahl an Pflichtstunden studentischer rheumatologischer Lehre. Rheumatologische Weiterbildung: Die jährlichen Weiterbildungsabschlüsse für Rheumatologie decken nicht den Bedarf an rheumatologischen FÄ, der durch steigende Arbeitsbelastung, reduzierte Kapazitäten durch Pensionierung und zunehmende Teilzeittätigkeit noch zunimmt. Versorgungsqualität: Rheuma-Betroffene haben seit Einführung hochwirksamer Medikamente eine deutlich bessere Aussicht auf eine Remission ihrer Erkrankung. Bei frühzeitiger adäquater Therapie ist die Lebensführung vieler Betroffener kaum noch eingeschränkt. Die Wartezeit auf eine rheumatologische Erstvorstellung beträgt aber oft mehr als 3 Monate. Qualitätsziel ist eine Vorstellung innerhalb der ersten 6 Wochen nach Symptombeginn. Frühsprechstunden, Delegation ärztlicher Leistungen, strukturierte Patientenschulungen und digitale Versorgungskonzepte wurden positiv evaluiert, sind aber nicht finanziell gedeckt. Kosten: Die jährlichen Gesamtkosten allein für entzündliche Gelenkerkrankungen belaufen sich auf etwa 3 Mrd. €. Die direkten Kosten sind seit Einführung der Biologika deutlich gestiegen, während indirekte Kosten für Krankschreibung, Erwerbsunfähigkeit und stationäre Aufenthalte gesunken sind.
UNASSIGNED: Kernforderungen dieses Memorandums sind: die deutliche und nachhaltige Steigerung der Zahl von Weiterbildungsstellen im ambulanten und stationären Bereich, die Schaffung von Lehrstühlen oder mindestens eigenständigen Abteilungen für Rheumatologie an allen Universitäten sowie die weitere Umsetzung neuer und sektorenübergreifender Versorgungsformen. Dies stellt eine bedarfsgerechte, moderne rheumatologische Versorgung für alle Betroffenen auch in Zukunft sicher.