Tofacitinib in refractory adult-onset Still’s disease: 14 cases from a single centre in China

Hu, Qiongyi; Wang, Mengyan; Jia, Jieshuang; Teng, Jialin; Chi, Huihui; Liu, Tingting; Liu, Honglei; Cheng, Xiaobing; Ye, Junna; Su, Yutong; Sun, Yue; Zhou, Zhongzheng; Wan, Lei; Wang, Zhihong; Wang, Fan; Shi, Hui; Yang, Chengde

doi:10.1136/annrheumdis-2019-216699

Cited by 49 publications

(36 citation statements)

References 10 publications

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“…In a small study, the JAK1–JAK2 inhibitor ruxolitinib showed promising efficacy in adults with secondary HLH 115 . Other JAK inhibitors, including tofacitinib and baricitinib, have also demonstrated some efficacy in treatment-refractory sJIA or AOSD 116 , 117 .…”

Section: Mas Insights From Translational Therapiesmentioning

confidence: 99%

Immune cartography of macrophage activation syndrome in the COVID-19 era

2021

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A hyperinflammatory ‘cytokine storm’ state termed macrophage activation syndrome (MAS), culminating from a complex interplay of genetics, immunodeficiency, infectious triggers and dominant innate immune effector responses, can develop across disparate entities including systemic juvenile idiopathic arthritis (sJIA) and its counterpart adult-onset Still disease (AOSD), connective tissue diseases, sepsis, infection, cancers and cancer immunotherapy. Classifying MAS using the immunological disease continuum model, with strict boundaries that define the limits of innate and adaptive immunity, at one boundary is MAS with loss of immune function, as occurs in the ‘perforinopathies’ and some cases of sJIA–AOSD. Conversely, at the other boundary, immune hypersensitivity with gain of immune function in MHC class II-associated sJIA–AOSD and with chimeric antigen receptor (CAR) T cell therapy also triggers MAS. This provides a benchmark for evaluating severe inflammation in some patients with COVID-19 pneumonia, which cripples primary type I interferon immunity and usually culminates in a lung-centric ‘second wave’ cytokine-driven alveolitis with associated immunothrombosis; this phenomenon is generally distinct from MAS but can share features with the proposed ‘loss of immune function’ MAS variant. This loss and gain of function MAS model offers immune cartography for a novel mechanistic classification of MAS with therapeutic implications.

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Section: Mas Insights From Translational Therapiesmentioning

confidence: 99%

Immune cartography of macrophage activation syndrome in the COVID-19 era

2021

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“…In a study with 14 patients with refractory AoSD, seven of them achieved complete remission under tofacitinib, while six responded partially. This trial also showed the steroid sparing effect of tofacitinib, especially in the articular phenotype [ 106 ]. Furthermore, a reported case of AoSD complicated by MAS describes remission with tofacitinib after failure of response to tocilizumab [ 107 ].…”

Section: Treatment Managementmentioning

confidence: 99%

Adult-Onset Still’s Disease: Clinical Aspects and Therapeutic Approach

Tomaras¹,

Goetzke

Kallinich

et al. 2021

JCM

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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.

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“…A recent report from China describes the successful use of tofacitinib in 14 patients with AOSD; however, not all patients had a complete response and some were still needing to take steroids on a long-term basis. 14 The success of JAKi in patients who have failed IL-1-directed and IL-6-directed therapies might be explained by the ability of these medications to block other likely relevant cytokines such as IL-12/IL-23 and interferon-γ. However, the optimal therapeutic dose of JAKi used for AOSD might be different from what is used for licenced indication.…”

Section: Discussionmentioning

confidence: 99%

Mixed results with baricitinib in biological-resistant adult-onset Still’s disease and undifferentiated systemic autoinflammatory disease

et al. 2020

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This clinical case series describes our experience with the use of Janus kinase 1/2 inhibitor baricitinib in two patients suffering from refractory adult-onset Still’s disease (AOSD) as well as in one case suffering from AOSD-like autoinflammatory disease in the context of myelodysplastic syndrome. All patients suffered from disease non-responsive to conventional Disease-modifying antirheumatic drugs (DMARDs) as well as biological therapies including interleukin (IL)-1 and IL-6 blockade, relying instead on high daily doses of prednisolone. We also report the first case of Pneumocystis jirovecii infection following baricitinib use.

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Tofacitinib in refractory adult-onset Still’s disease: 14 cases from a single centre in China

Cited by 49 publications

References 10 publications

Immune cartography of macrophage activation syndrome in the COVID-19 era

Immune cartography of macrophage activation syndrome in the COVID-19 era

Adult-Onset Still’s Disease: Clinical Aspects and Therapeutic Approach

Mixed results with baricitinib in biological-resistant adult-onset Still’s disease and undifferentiated systemic autoinflammatory disease

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