To develop new classification criteria for systemic lupus erythematosus (SLE) jointly supported by the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR).This international initiative had four phases. 1) Evaluation of antinuclear antibody (ANA) as an entry criterion through systematic review and meta-regression of the literature and criteria generation through an international Delphi exercise, an early patient cohort, and a patient survey. 2) Criteria reduction by Delphi and nominal group technique exercises. 3) Criteria definition and weighting based on criterion performance and on results of a multi-criteria decision analysis. 4) Refinement of weights and threshold scores in a new derivation cohort of 1,001 subjects and validation compared with previous criteria in a new validation cohort of 1,270 subjects.The 2019 EULAR/ACR classification criteria for SLE include positive ANA at least once as obligatory entry criterion; followed by additive weighted criteria grouped in 7 clinical (constitutional, hematologic, neuropsychiatric, mucocutaneous, serosal, musculoskeletal, renal) and 3 immunologic (antiphospholipid antibodies, complement proteins, SLE-specific antibodies) domains, and weighted from 2 to 10. Patients accumulating ≥10 points are classified. In the validation cohort, the new criteria had a sensitivity of 96.1% and specificity of 93.4%, compared with 82.8% sensitivity and 93.4% specificity of the ACR 1997 and 96.7% sensitivity and 83.7% specificity of the Systemic Lupus International Collaborating Clinics 2012 criteria.These new classification criteria were developed using rigorous methodology with multidisciplinary and international input, and have excellent sensitivity and specificity. Use of ANA entry criterion, hierarchically clustered, and weighted criteria reflects current thinking about SLE and provides an improved foundation for SLE research.
Einleitung: Es ist bekannt, dass Clostridium (C.) perfringens und dessen Toxine eine Rolle in der Pathogenese des AHDS spielen und betroffene Hunde Veränderungen der intestinalen Mikrobiota aufweisen.Derzeit wird AHDS symptomatisch therapiert, wobei der Einfluss von Probiotika auf das intestinale Mikrobiom sowie den klinischen Verlauf bei Hunden mit AHDS bisher nicht untersucht wurde.Material und Methoden: 25 Hunde
Patients with antiphospholipid syndrome (APS) are prone to thromboembolism. So far, the only predictive parameters for further complications are their number in patient's history and perhaps the titre of aPL. Derived from clinical investigation of stroke and obvious analogies between cerebrovascular ischemia (CVI) in patients with carotid artery disease (CAD) and patients with APS, a novel non-invasive method is introduced using transcranial Doppler (TCD) long-term monitoring to detect high energy ultrasonic signals (so called ‘microemboli’) in the cerebral vasculature. In patients with CAD, these microemboli proved to correlate with past and impending symptoms of CVI permitting therapeutic stratification by their detectability. In SLE and APS, this technique enabled identification of very similar signals in cerebral bloodstream of APS patients. Microemboli were highly associated with the history of CVI and the titre of aPL. Detection of microemboli offers new possibilities in risk estimation, therapeutic stratification and in studying pathophysiology of APS.
Response to 'What rheumatologist should know about Fabry disease' by Moiseev et al In their correspondence 1 to our study on the prevalence of Fabry disease (FD) within a national cohort of early undifferentiated arthritis patients in Germany, 2 Moiseev et al raise additional issues of importance for clinical rheumatologists pertaining to the change of symptoms in FD over time.Retrospective studies analysing the medical history of patients with confirmed FD raised concerns that FD may be overlooked in rheumatological practice.In particular, Lidove et al reported that 9 of 40 patients were mistakenly diagnosed with a rheumatic condition in adulthood prior to the diagnosis of FD. 3 This is well in line with the data reported by Moiseev et al in their correspondence.They observed erroneous rheumatological diagnoses in 28 of 107 confirmed FD cases across all age groups.4][5] For instance, Lidove et al report a mean age at diagnosis of 37.2 years with a range up to 71 years in their study focusing on musculoskeletal complaints of FD patients. 3Moreover, a cross-sectional study from the Fabry Outcome Survey comparing older (>50 years) to younger patients with confirmed FD showed that the mean age at first diagnosis was >50 years in the older FD patients, representing 34% of the 1934 patients included. 6Additionally, patients diagnosed at an older age or with a presumably late clinical onset may differ phenotypically from patients diagnosed at a younger age. 6Although acroparesthesias or Fabry crises tend to be a more prominent feature at a younger age and to diminish over time, 5 7 joint complaints remain a significant burden for patients with 40%-50% of patients>50 years of age reporting joint pain or swelling in a survey by Ivleva et al. 7 In summary, FD patients often display musculoskeletal complaints and the correct diagnosis is typically delayed by years.In our opinion, these data provided a strong rationale for our study in which we intended to explore the prevalence of FD patients in a national cohort of early undifferentiated arthritis in adult patients (n=798, aged 56.5±14.3years, range 18-87).We did not find a mutation considered pathogenic under the inclusion criterion of at least one swollen joint.Hence, our study reassures us that we do not systematically miss the diagnoses of FD in our adult early arthritis patients employing current standard of practice procedures.However, rheumatologists and even paediatricians may not generally be familiar with signs and symptoms of FD. 8 We are therefore delighted that our study raised the interest of Moiseev et al who comprehensively summarised clinical features of FD and shared data on the musculoskeletal complaints of their FD cohort in their comment.
The development of a procedure for the measurement of insertion depth angles of cochlear implant electrode arrays based on flat-panel computed tomography (FPCT) and the application of this technique to in vivo postoperative images.The knowledge of the insertion depth angle of electrode arrays is relevant for the preservation of low-frequency residual hearing and for optimizing speech coding strategies. Until now, the angular position of electrodes was derived from 2-dimensional radiographs.In the present study, 3-dimensional (3D) radiographs provided by FPCT were used to determine the insertion depth according to angular electrode positions with higher accuracy. For this purpose, a new evaluation procedure was designed and applied to radiographs of 15 cochlear implant patients.In contrast to 2-dimensional radiographs, the obtained 3D images show all 3 semicircular channels and therefore allow the determination of a clear reference, which is required for precise insertion angle measurements. Furthermore, the presented FPCT radiographs visualize distinct electrodes. Despite the constant length of the implanted electrode arrays, we have found a considerable variation of measured insertion depth angles, which is consistent with published observations on the variability and the gender dependence of the size of human cochleae.FPCT provides 3D high-resolution radiographic data that enable the determination of the insertion depth angle with high accuracy and, potentially, an angle determination of individual electrodes. Therefore, this low-dose technique is especially appropriate for postoperative investigations after cochlea implantation.
Electronic health records are increasingly used and frequently required from various regulatory authorities. Apart from their day-to-day use by health care professionals for routine clinical practice and/or the improvement of quality of care processes, patients with chronic inflammatory disease may become increasingly involved in the data retrieval process by self-monitoring and providing patient-reported (outcome) data. Among key features of electronic health records are automated scoring, visualisation of validated measures, and long-term systematic patient-centered data collection in a structured and standardised manner. Data derived from electronic health records are increasingly incorporated into patient-centered research, registries, and other secondary uses. Thus, electronic health records offer opportunities to improve knowledge and to create new process flows in rheumatology health care. The article summarises some of these opportunities in patient care, as well as an overview of secondary use scenarios. In addition, the article focuses on patients' active involvement in the disease management process via health information applications, reports on patients' perspectives, as well as some legal and regulatory matters concerning electronic health records.
This article reports a case of guttural pouch bleeding which was managed successfully by using intravascular embolisation systems to occlude the damaged vessels. Percutaneous catheterisation of the common carotid artery allowed angiographic visualisation of the main head arteries: A. carotis externa, A. carotis interna and A. occipitalis, which showed no abnormalities angiographically. Originating from the A. occipitalis, one artery sent smaller, extensively branching and tortuous vessels to the guttural pouch area. This branching was interpreted as a sign of inflammatory hypervascularization. The artery was occluded by positioning of a detachable balloon distally of the origin of the two small vessels. Insertion of two coils in the A. occipitalis proximally of the origin of this artery completed the embolisation. Occlusion of the distal part was necessary to avoid blood supply to the lesion from the contralateral arterial system via the circle of Willis. The technique used allowed occlusion of a selected head artery without direct surgical intervention in this area. There is no need to remove the implants. After catheterisation, no more episodes of epistaxis occurred. The causing diphtheroid inflammation in the guttural pouch was treated by local infusions of iodine-solutions and resolved completely within 24 days.