Zeitschrift fur Rheumatologie最新文献

Centromere-antibody-positive limited cutaneous systemic sclerosis (LCSSc) is a common disease. It generally takes a benign course, but can still considerably reduce quality of life. Our patient-a woman born in 1987 who was first diagnosis 2017-suffered from pain and declining hand function due to sclerodactyly and calcinosis. Methotrexate (MTX) and analgesics were not effective. After commencing off-label treatment with filgotinib, the patient reported rapid improvement. An X-ray after 8 months showed a reduction in calcinosis load. Filgotinib is well tolerated. Sclerodactyly has resolved, and hand function continues to improve. The clinical and radiological response merit further enquiry of Janus kinase (JAK) inhibitors in centromere-antibody-positive LCSSc.

Our case study highlights the challenges in treating chronic non-bacterial osteitis/osteomyelitis (CNO) of the jaw and supports the current recommendations for diagnosis and treatment, which prioritize newly defined drug treatment strategies. The implementation of regular reevaluations-including magnetic resonance imaging (MRI) if necessary-is essential for the desired treat-to-target strategy. The patient care should be carried out in centers with an interdisciplinary approach and experience in the treatment of CNO.

Over the past 25 years, the results of treatment in pediatric rheumatology have greatly improved due to enormous developments in medical therapies. Today, reaching inactive disease or remission has become a realistic aim. Controlling inflammation and avoiding damage still remain the most important issues. Scores and tools for the measurement of disease activity have mostly been designed to be applicable in studies. Priorities may be different in a patient's perspective. This paper illustrates the different perspectives from a patient's and physician's view, thus, trying to describe the requirement, potentials, and limitations of shared decision-making in children, adolescents and young adults suffering from rheumatic diseases.

Janus kinase inhibitors (JAKis) are highly effective drugs for treating inflammatory rheumatic diseases. The randomized ORAL surveillance trial showed that patients with rheumatoid arthritis (RA) aged 50 years and older with at least 1 more cardiovascular risk factor had an increased risk of malignant diseases with the JAKi tofacitinib compared to tumor necrosis factor inhibitors; however, this result has not been replicated in subsequent studies. An analysis of the German RABBIT register now revealed an overall slightly increased cancer risk in RA patients ever treated with JAKi compared to those ever treated with biological (b) disease-modifying antirheumatic drugs (DMARDs). During or after 2285 JAKi treatment episodes and 4259 bDMARD treatment episodes, 88 and 135 malignant diseases occurred, respectively, resulting in a rate of 11.6 (95% confidence interval, CI: 9.3-14.3) events for JAKis and 8.9 (95% CI: 7.4-10.5) events for bDMARDs per 1000 patient years. The adjusted hazard ratio was 1.40 (95% CI: 1.09-1.80), which is comparable to the results from the ORAL surveillance study. Particularly affected by the observed risk increase were patients aged 60 years and older, those with high disease activity and those who had already received ≥ 3 treatments with conventional synthetic DMARDs. Other above-average affected groups included smokers, male patients and patients with an RA duration of > 10 years. When treating patients with an increased risk, clinicians should carefully consider together with them whether this observed risk outweighs the potential risks of discontinuing or withholding a highly effective treatment such as JAKis, bearing in mind that inadequate disease control is also associated with an increased risk of cancer. When in doubt, the results from RABBIT should not discourage physicians from prescribing a JAKi treatment to patients who need it. It could be a way forward to ensure that a close cancer screening is carried out for risk patients treated with JAKis.

Immunoglobulin G4 (IgG4)-related diseases are a heterogenous group of chronic inflammatory systemic disorders characterized by fibrosing inflammation with infiltration of IgG4-positive plasma cells. They can affect nearly any organ system. Typical manifestations include autoimmune pancreatitis, sclerosing cholangitis, lymphadenopathy, retroperitoneal fibrosis, and inflammatory orbitopathy as well as involvement of the salivary and lacrimal glands. Each manifestation may present in isolation or in combination with others. Diagnosis requires careful exclusion of malignant or other inflammatory conditions, as IgG4-related diseases can mimic a wide range of disease entities. A multimodal approach combining laboratory findings, histopathological evaluation and radiological imaging is essential for establishing the diagnosis. A structured diagnostic algorithm and close interdisciplinary collaboration are crucial to avoid misdiagnosis and enable appropriate treatment.

Background: Regulatory safety recommendations regarding Janus kinase inhibitors (JAKi) led to a marked decline in new prescriptions in Germany. In the meantime, professional medical societies have also issued differentiated recommendations.

Objectives: The aim was to investigate the RHADAR (RheumaDatenRhePort) registry to check whether JAKi prescriptions recovered thereafter.

Materials and methods: A retrospective analysis of new prescriptions for JAKi, tumor necrosis factor inhibitors (TNFi), and interleukin‑6 receptor inhibitors (IL-6Ri) in patients with rheumatoid arthritis between 1 April 2020, and 30 September 2024. The primary endpoint was the proportion of JAKi among all new prescriptions of these three drug classes per half-year. The treatment line of JAKi use was additionally assessed.

Results: A total of 3492 treatment initiations were recorded in the registry during the observation period (TNFi: n = 1770, 50.7%, JAKi: n = 1269, 36.3%, IL-6Ri: n = 453, 13.0%). The proportion of JAKi initially rose from 29.5% (Q2-Q3/2020) to 46.9% (Q2-Q3/2021), followed by a decline to 24.4% (Q2-Q3/2023) after the regulatory safety communications. Since Q4/2023, a renewed increase has been observed (32.8%), though not yet reaching previous peak levels. JAKi were increasingly shifted from early to advanced treatment lines (≥ 3rd line).

Conclusion: Following the decline in JAKi prescriptions due to European Medicines Agency safety recommendations, a renewed increase has been observed. Since then, JAKi have been predominantly used in later treatment lines, indicating a more differentiated approach to this drug class.

Protocols concerning classification, monitoring and treatment were developed for the oligoarticular form of juvenile idiopathic arthritis (JIA) as part of a consensus process. The group of authors formulated 23 statements and circulated them in an online survey to medical members of the Society for Paediatric and Adolescent Rheumatology (GKJR). A total of 80 of the 124 paediatric and adolescent rheumatologists took part in the survey, which corresponds to just under 65% of the paediatric and adolescent rheumatologists who were active at the time. In a final online meeting, comments from the survey were incorporated into the statements and then agreed upon by the group of authors. For newly occurring oligoarticular JIA, 20 statements and a summary consensus treatment protocol were developed to optimise the treatment of persistent oligoarticular JIA.