Current and emerging biological therapy in adult-onset Still’s disease

Ma, Yuning; Meng, Jianfen; Jia, Jieshuang; Wang, Mengyan; Teng, Jialin; Zhu, Dehao; Yang, Chengde; Hu, Qiongyi

doi:10.1093/rheumatology/keab485

Cited by 28 publications

(27 citation statements)

References 146 publications

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“…CD11b and CD32 are mainly expressed in neutrophils, suggesting that neutrophils contribute significantly to the onset of AOSD. Other receptors related to neutrophil activation, such as granulocyte-macrophage colony-stimulating factor receptor, are being mentioned as targets for new therapies for AOSD; however, evidence for their pathological roles in AOSD is still lacking [ 98 ].…”

Section: Neutrophils In Sjia and Aosdmentioning

confidence: 99%

An Update on the Pathogenic Role of Neutrophils in Systemic Juvenile Idiopathic Arthritis and Adult-Onset Still’s Disease

Kim

Ahn

Jung

et al. 2021

IJMS

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Neutrophils are innate immune phagocytes that play a key role in immune defense against invading pathogens. The main offensive mechanisms of neutrophils are the phagocytosis of pathogens, release of granules, and production of cytokines. The formation of neutrophil extracellular traps (NETs) has been described as a novel defense mechanism in the literature. NETs are a network of fibers assembled from chromatin deoxyribonucleic acid, histones, and neutrophil granule proteins that have the ability to kill pathogens, while they can also cause toxic effects in hosts. Activated neutrophils with NET formation stimulate autoimmune responses related to a wide range of inflammatory autoimmune diseases by exposing autoantigens in susceptible individuals. The association between increased NET formation and autoimmunity was first reported in antineutrophil cytoplasmic antibody-related vasculitis, and the role of NETs in various diseases, including systemic lupus erythematosus, rheumatoid arthritis, and psoriasis, has since been elucidated in research. Herein, we discuss the mechanistic role of neutrophils, including NETs, in the pathogenesis of systemic juvenile idiopathic arthritis (SJIA) and adult-onset Still’s disease (AOSD), and provide their clinical values as biomarkers for monitoring and prognosis.

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Section: Neutrophils In Sjia and Aosdmentioning

confidence: 99%

An Update on the Pathogenic Role of Neutrophils in Systemic Juvenile Idiopathic Arthritis and Adult-Onset Still’s Disease

Kim

Ahn

Jung

et al. 2021

IJMS

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“…Adult-onset Still's disease (AOSD) and systemic JIA (sJIA) are two autoinflammatory diseases with similar clinical presentation and treatment strategies. Interleukin-1 and interleukin-6 cytokines contribute to disease pathogeneses, and anti-IL1 and anti-IL-6 biologics have comparable efficacy in the treatment of these patients ( 22 ). JAK-inhibitors block signal transduction downstream of many cytokine receptors, so theoretically they might be more efficacious to control sJIA than classical biologics, especially in the resistant cases ( 3 ).…”

Section: Discussionmentioning

confidence: 99%

The Safety and Efficacy of Tofacitinib in 24 Cases of Pediatric Rheumatic Diseases: Single Centre Experience

et al. 2022

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JAK-inhibitors are small molecules blocking the JAK-STAT pathway that have proven effective in the treatment of different immune-mediated diseases in adults and juvenile idiopathic arthritis (JIA).Aim of StudyTo evaluate the safety and efficacy of tofacitinib in children with different rheumatic diseases.Material and MethodsWe extracted information from 24 children with the following diagnosis: JIA (n = 15), undifferentiated systemic autoinflammatory diseases (SAIDs) (n = 7), and juvenile dermatomyositis (JDM) (n = 2) who have been treated with tofacitinib for a period of longer than 6 months. The treatment outcomes were classified according to the opinion of the attending physicians as having a complete response (CR), i.e., the absence of disease activity, or a partial response (PR)—a significant improvement of symptoms and disease activity, or no response (NR)—no changes in disease activity.ResultsCR was achieved in 10/24 patients; 7/15 among JIA patients, 1/2 among JDM patients, 4/7 among SAID patients, and PR in 5/15 of JIA, 1/2 of JDM, and 3/7 of SAID patients. Three non-responders with JIA discontinued tofacitinib. Corticosteroids were successfully tapered off in 11/14 patients and discontinued in 2/14 patients. Four patients had side effects not requiring treatment discontinuation: liver enzyme elevation (n = 2), hypercholesterolemia (n = 1), lymphadenitis (n = 1).ConclusionJAK-inhibitors are effective new therapies for the treatment of multiple immune-mediated diseases. Our experience has shown the best results in patients with JIA and JIA-associated alopecia, and type I interferonopathies. More data from randomized controlled clinical trials are needed to use JAK-inhibitors safely in pediatric rheumatic diseases.

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“…Rilonacept, also known as IL-1 trap, is an IL-1a and b inhibitor that has been shown to be effective in treating both the systemic and arthritic manifestations of refractory AOSD. It is mostly used in patients who have not had much response to anakinra [ 36 ]. In a study done by Henderson et al, five refractory AOSD patients were treated with rilonacept, and three of five patients were noted to have an improvement in their symptoms.…”

Section: Discussionmentioning

confidence: 99%

“…The study also noted a decrease in the dosage of oral prednisone after 48 weeks of treatment. Sarilumab, another IL-6 receptor inhibitor, has only been used once per Ma et al [ 36 ].…”

Section: Discussionmentioning

confidence: 99%

Adult-Onset Still’s Disease: A Case Report and Review of Current Therapeutic Options

Thomas¹,

Kesarwani²,

Graber³

et al. 2022

Cureus

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Adult-onset Still's disease (AOSD) is a rare autoinflammatory disease that typically presents with a triad of fever, evanescent rash, and arthritis. There is often a delay in diagnosis of AOSD due to its nonspecific clinical presentation, which may mimic other infectious, rheumatological disorders, and malignancies. Corticosteroids have been the cornerstone for the management of AOSD for the past many years. However, with the expanding understanding of its pathogenesis, novel therapeutic options targeting various cytokines are being increasingly recognized. Herein, we present a case of AOSD that was successfully treated with tocilizumab, a monoclonal antibody against the interleukin-6 (IL-6) receptor. For the purpose of this article, we also conducted a literature search to review the current therapeutic options available for the treatment of AOSD.

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Current and emerging biological therapy in adult-onset Still’s disease

Cited by 28 publications

References 146 publications

An Update on the Pathogenic Role of Neutrophils in Systemic Juvenile Idiopathic Arthritis and Adult-Onset Still’s Disease

An Update on the Pathogenic Role of Neutrophils in Systemic Juvenile Idiopathic Arthritis and Adult-Onset Still’s Disease

The Safety and Efficacy of Tofacitinib in 24 Cases of Pediatric Rheumatic Diseases: Single Centre Experience

Adult-Onset Still’s Disease: A Case Report and Review of Current Therapeutic Options

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