Adult-onset Still’s disease (AoSD) is a rare systemic autoinflammatory disease characterized by arthritis, spiking fever, skin rash and elevated ferritin levels. The reason behind the nomenclature of this condition is that AoSD shares certain symptoms with Still’s disease in children, currently named systemic-onset juvenile idiopathic arthritis. Immune dysregulation plays a central role in AoSD and is characterized by pathogenic involvement of both arms of the immune system. Furthermore, the past two decades have seen a large body of immunological research on cytokines, which has attributed to both a better understanding of AoSD and revolutionary advances in treatment. Additionally, recent studies have introduced a new approach by grouping patients with AoSD into only two phenotypes: one with predominantly systemic features and one with a chronic articular disease course. Diagnosis presupposes an extensive diagnostic workup to rule out infections and malignancies. The severe end of the spectrum of this disease is secondary haemophagocytic lymphohistiocytosis, better known as macrophage activation syndrome. In this review, we discuss current research conducted on the pathogenesis, diagnosis, classification, biomarkers and complications of AoSD, as well as the treatment strategy at each stage of the disease course. We also highlight the similarities and differences between AoSD and systemic-onset juvenile idiopathic arthritis. There is a considerable need for large multicentric prospective trials.

Background: Pulmonary affection (PA) is associated with a substantial increase in morbidity and mortality in patients with idiopathic inflammatory myopathies (IIM). However, the underlying immune mechanisms of PA remain enigmatic and prompt deeper immunological analyses. Importantly, the Janus-faced role of natural killer (NK) cells, capable of pro-inflammatory as well as regulatory effects, might be of interest for the pathophysiologic understanding of PA in IIM. Methods: To extend our understanding of immunological alterations in IIM patients with PA, we compared the signatures of NK cells in peripheral blood using multi-color flow cytometry in IIM patients with (n = 12, of which anti-synthetase syndrome = 8 and dermatomyositis = 4) or without PA (n = 12). Results: We did not observe any significant differences for B cells, CD4, and CD8 T cells, while total NK cell numbers in IIM patients with PA were reduced compared to non-PA patients. NK cell alterations were driven by a particular decrease of CD56^dim NK cells, while CD56^bright NK cells remained unchanged. Comparisons of the cell surface expression of a large panel of NK receptors revealed an increased mean fluorescence intensity of NKG2D⁺ on NK cells from patients with PA compared with non-PA patients, especially on the CD56^dim subset. NKG2D⁺ and NKp46⁺ cell surface levels were associated with reduced vital capacity, serving as a surrogate marker for clinical severity of PA. Conclusion: Our data illustrate that PA in IIM is associated with alterations of the NK cell repertoire, suggesting a relevant contribution of NK cells in certain IIMs, which might pave the way for NK cell-targeted therapeutic approaches.

Plant-derived nutraceuticals are proposed as new key instruments to represent a profound “back to basics” shift in medical treatment. Data accumulated over the past ten years suggest that curcumin, the major active compound of the turmeric plant, has anti-inflammatory properties. It has yet to be determined whether the anti-inflammatory profile of curcumin is potent enough to justify the application of this substance as a nutritional supplement for patients with rheumatic diseases. To address this question, the most relevant in vitro studies that investigate the mechanism of action of curcumin were reviewed in this article. In addition, a total of 18 animal and human trials were evaluated. The pleiotropic, anti-inflammatory and immunomodulatory effects of curcumin were observed in animal studies. In addition, human trials demonstrated promising findings. In these studies, curcumin was able to reduce the expression of proinflammatory cytokines, lower the level of the C-reactive protein and improve clinical parameters. A limiting factor of the application of curcumin is the inconsistent bioavailability of the substance. Therefore, new formulations have been developed to improve the pharmacodynamic profile of curcumin. The future acceptance of the substance is dependent on new controlled clinical trials with a standardised formulation of curcumin administered as well as standard of care.

Myositis is a rare and an extremely heterogeneous autoimmune disease, that causes muscle weakness. Currently, “idiopathic inflammatory myopathies (IIM)“ is the preferred umbrella-term used to describe the disease complexity within individuals. IIM include dermatomyositis, polymyositis, inclusion body myositis, autoimmune necrotizing myopathy, overlap myositis and antisynthetase syndrome. Research activity concerning myositis was very intense over the past ten years and led to new diagnostic approach as well as to novel therapeutic strategies. Correct classification is the key for successful management. One single treatment regime for every possible organ involvement in all different forms of IIM is still not existing. Idiopathische inflammatorische Myopathien sind seltene heterogene Autoimmunerkrankungen, die mit einer Muskelschwäche einhergehen. Kommen zur Muskelschwäche Haut- oder Allgemeinsymptome wie Fatigue und Fieber hinzu oder andere Auffälligkeiten, sollten Behandelnde aufmerksam werden. Dieser Beitrag stellt die wesentlichen Erkenntnisse zu Diagnose und Therapie der Erkrankung mit ihren komplexen unterschiedlichen Unterformen vor. Publication Date: 02 July 2020 (online) © Georg Thieme Verlag KG Stuttgart · New York

Zusammenfassung: Auf dem Sektor der Kollagenosen ist der wissenschaftliche Fortschritt bei der Myositis in den letzten 15–20 Jahren bemerkenswert. Durch kontinuierliche Forschung und intensive Vernetzung der Myositis-Experten ist es gelungen, neue Untergruppen zu identifizieren und somit für die Prognose wichtige Organmanifestationen rechtzeitig zu erkennen. Vor dem Hintergrund dieser Neuerungen verfolgt diese Übersichtsarbeit sowohl das Ziel, möglichst alle Facetten der Erkrankung zu präsentieren, als auch die moderne Einteilung der idiopathischen inflammatorischen Myopathien zu erläutern. Außerdem werden die neuen Klassifikationskriterien vorgestellt, die die Kriterien von Bohan und Peter aus dem Jahr 1975 abgelöst haben. Im Artikel werden ihre Stärken und Schwächen sowie ihr Optimierungspotenzial diskutiert.