Zeitschrift fur Rheumatologie最新文献

The pandemic led to a global disruption of public life unprecedented in modern times due to an infectious disease, which certainly caused additional special burdens for patients with chronic diseases as well as for personnel in the healthcare system. The German Society of Rheumatology and Clinical Immunology (DGRh), with its Executive Board and two ad hoc commissions, responded promptly to the complex challenges posed by the pandemic for rheumatological care in Germany with provision of a comprehensive and professionally sound range of information and provided concrete assistance in many situations. The diverse activities of the DGRh in the context of the pandemic led to national and international attention and consideration of its committees and recommendations in national committees and guidelines.

Coronavirus disease 2019 (COVID-19) has influenced the world over the last 3 years. Although the risk of a severe course is low in children, it can be influenced by chronic rheumatic diseases or treatment with immunosuppressive drugs or immunomodulatory medication. The German register for biologics in pediatric rheumatology (BIKER) documented systematic data from 68 centers on the occurrence, presentation and outcome of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infections in children with rheumatic diseases. Between March 2020 and December 2022, a total of 927 SARS-CoV‑2 infections in 884 patients could be reported and analyzed in pediatric patients with rheumatic diseases. Juvenile idiopathic arthritis (JIA) was the most frequent diagnosis (716 infections) followed by genetic autoinflammation (103 infections), systemic autoimmune diseases (78 infections), idiopathic uveitis (25 infections) and vasculitis (5 infections). Only four patients were treated as inpatients. A 3.5-year-old female patient died during the first wave from encephalopathy and respiratory failure. The patient was treated with methotrexate (MTX) and steroids for systemic JIA. Genetic tests revealed a previously unknown congenital immune defect. No other patient had to be ventilated or treated on the intensive care unit. A case of uncomplicated pediatric inflammatory multisystem syndrome (PIMS) was registered in a patient with JIA treated with MTX. At the time of the infection over 60% of the patients were treated with standard disease modifying antirheumatic drugs (DMARD) and/or biologics. Although the patients treated with MTX showed a slightly longer duration of symptoms, the antirheumatic treatment did not appear to have a negative influence on the severity or outcome of the SARS-CoV‑2 infection.

At the beginning of the coronavirus disease 2019 (COVID-19) pandemic in December 2019 there was no available evidence regarding the management of immunosuppressive or immunomodulatory treatment and the potential outcomes of severe acute respiratory syndrome coronavirus type 2 (SARS-CoV-2) infections in inflammatory rheumatic diseases (IRD). As a result, the Justus Liebig University of Giessen, Germany, in collaboration with the German Society for Rheumatology, established the German COVID-19 register ( www.covid19-rheuma.de ). The COVID-19 register enabled for the first time a systematic documentation and evaluation of viral infections in patients with IRD. The data collection started as early as March 2020. Currently, the register is one of the largest global registers in the field of COVID-19 and IRD. As of 18 December 2023 the register has recorded more than 7100 cases. The first scientific findings on SARS-CoV‑2 infections in IRD patients were generated from the register in 2020, showing an association between disease activity of IRD, certain comorbidities, such as cardiovascular diseases and treatment with rituximab, with an unfavorable course. The contents and construction of the database of the register were designed at the conception to allow collaboration and data exchange with other national and international registers (e.g., EULAR COVID-19 register, COVID-19 global rheumatology alliance and the Lean European open survey on SARS-CoV‑2 infected patients). In addition, other registers and surveys were initiated. A vaccination register documents the tolerability and possible adverse reactions to COVID-19 vaccination in IRD patients. The data resulted in numerous publications and formed the basis for national and international recommendations for action in the care and vaccination of IRD patients during the COVID-19 pandemic. In summary, the German COVID-19 register has made a significant contribution to the understanding of the course of COVID-19 in IRD patients and has facilitated international collaboration for a better understanding of COVID-19 and IRD.

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) continues to play a major role as a severe and potentially fatal airway infection, especially in vulnerable patient groups. In view of the thin data situation on the influence of treatment and response to vaccination, at the beginning of the corona pandemic it was a major challenge to predict the tolerability and effectiveness in patients with inflammatory rheumatic diseases under immunomodulation or immunosuppression. In the meantime, numerous studies have addressed the questions of response and tolerability, at least for the COVID-19 vaccination. Even in the first months of the vaccination campaign, a small study on a single center cohort could show that apart from patients with B‑cell depletion, all included patients showed a seroconversion after the first two vaccinations. This resulted in neither an increased occurrence of exacerbations of the underlying disease nor new autoimmune phenomena. Various studies have since then confirmed these data.

This article summarizes the current guidelines and recommendations published by the European Alliance of Associations for Rheumatology (EULAR), the Kidney Disease Improving Global Outcomes (KDIGO) and the American College of Rheumatology (ACR). In addition to glucocorticoids (GC), treatment with biologics is nowadays an established option to treat Anti-Neutrophil Cytoplasmic Antibody (ANCA)-associated vasculitis (AAV). Rituximab (RTX) is used for remission induction and maintenance in organ-threatening and non-organ-threatening granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). For eosinophilic GPA (EGPA) anti-interleukin 5 (IL5) strategies are an important component of treatment for remission induction and maintenance of refractory or relapsing non-organ-threatening diseases in conjunction with GC. The dosing of GC for remission induction in GPA and MPA is now lower than was previously used and additionally, avacopan is approved as a new GC-sparing medication for GPA and MPA over 52 weeks. Conventional strategies, such as cyclophosphamide (CYC) are important for remission induction in severe or refractory organ-threatening disease for all AAVs. The use of methotrexate (MTX) and azathioprine (AZA) is becoming less prominent. The most important unanswered questions in the treatment of AAVs are with respect to the duration of remission maintenance treatment and the individualized treatment guidance based on biomarkers.

Background: Vaccinations represent an easily accessible, safe, and important method for preventing infections. Patients with primary immunodeficiencies (PID) are more susceptible to infections and should receive an extended spectrum of immunizations in many countries.

Methods: Between January 2019 and May 2020, vaccination certificates of 70 patients with PID from the regions of Würzburg and Hanover in Germany were evaluated. The patients were additionally surveyed regarding their attitude towards vaccinations and the communication with their physicians. Medical records were analyzed.

Results: Of the 70 patients, 54 (77%) suffered from common variable immunodeficiency, 30 (43%) were diagnosed with accompanying autoimmunity, 62 (89%) had an increased susceptibility to infections, and 56 (80%) were on immunoglobulin substitution therapy. Seven patients (10%) had neither a vaccination certificate nor were they able to recollect of their last vaccination. Only 55 (79%) and 43 (61%) patients stated that their rheumatologist or immunologist had recommended an influenza and a pneumococcal vaccination, respectively. When asked about their overall trust in vaccinations on a scale of 0 to 10 (0 = very low, 10 = very high), the mean value was 7.8. The most common vaccination was against tetanus in 63 (90%) patients, 49 (70%) had received vaccination against pneumococci, and 39 (56%) had received an influenza vaccination. Interestingly, 26 patients (37%) were vaccinated against measles, even though this is contraindicated in most PID patients.

Conclusion: Our data suggest that vaccination rates in this at-risk population are insufficient. Healthcare providers should emphasize vaccinations routinely when caring for these patients.