<scp>NXP2</scp>‐positive dermatomyositis complicated with refractory skin edema: Successful treatment with tocilizumab

Lu, Zihan; Chen, Yanan; Xue, Jing; Liu, Lei

doi:10.1111/dth.14712

Cited by 6 publications

(3 citation statements)

References 10 publications

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“…Therefore, instead of simply comparing IL-6 with healthy individuals, we specifically studied IL-6 in RP-ILD. Studies have been found successful tocilizumab treatment of RP-ILD in anti-MDA5 antibody-positive DM patients [ 33 ]; we have also successfully treated an NXP-2 antibody-positive DM patient with tocilizumab [ 34 ]. The levels of serum IL-6 were reported to be higher in the RP-ILD subset than in the non-ILD subset or the chronic ILD subset in IIM, but there was no significance [ 35 ].…”

Section: Discussionmentioning

confidence: 99%

Promising Neutrophil-Associated Biomarkers in Lung Diseases of Patients with Antisynthetase Syndrome and Dermatomyositis

Liu

Wang

Zhang

et al. 2022

Journal of Immunology Research

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Objectives. Antisynthetase syndrome (ASS) and dermatomyositis (DM) are serious autoimmune diseases, with lungs being the most frequently involved organ and sometimes fatal. This study is aimed at clarifying the role of neutrophil-associated biological markers in suggesting ASS and DM-associated respiratory infections and interstitial lung diseases. Methods. We carried out a retrospective review of the medical records of 46 cases of ASS and DM diagnosed at the Second Hospital of Zhejiang University College of Medicine, between January 2017 and December 2020. Serum myeloperoxidase (MPO), neutrophil elastase (NE), α1 anti-trypsin (AAT), and interleukin-6 (IL-6) were also detected. Results. Gottron’s sign is characteristic of dermatomyositis, while polyarthritis is more characteristic of ASS. Pulmonary function is worse in ASS than in DM patients. Patients with ASS and DM had abnormal lymphocyte and neutrophil counts compared to healthy subjects, but not in relation to lung function and rapid progression of interstitial lung disease (RP-ILD). Elevated serum NE, MPO, and IL-6 levels are suggestive of respiratory infections, whereas decreased circulating IL-6 is predictive of RP-ILD. Conclusion. Our study identified the neutrophil-associated biomarkers MPO, NE, and IL-6 as promising indicators with different suggestive roles in respiratory infections and interstitial lung diseases in patients with ASS and DM.

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Section: Discussionmentioning

confidence: 99%

Promising Neutrophil-Associated Biomarkers in Lung Diseases of Patients with Antisynthetase Syndrome and Dermatomyositis

Liu

Wang

Zhang

et al. 2022

Journal of Immunology Research

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“…According to the literature, Kondo M et al [19] successfully treated a 32-year-old female with refractory dermatomyositis complicated with RA by tocilizumab, which not only had a significant effect on joint swelling and pain, but also alleviated the symptoms of rash and myalgia, and the muscle enzymes were also reduced to normal. Lu Z et al [20] reported a case of NXP2-positive dermatomyositis that was significantly reduced by tocilizumab treatment. Fei Teng et al [21] reported that tocilizumab was successfully applied to a 63-year-old female with anti-MDA5-positive rapidly progressive pulmonary interstitial lesions, and the patient was intubated to assist breathing due to pulmonary lesions.…”

Section: Discussmentioning

confidence: 99%

Case study of tocilizumab in the treatment of dermatomyositis-associated interstitial lung disease

2024

FMSR

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Dermatomyositis (DM) complicated by rapidly progressive interstitial lung disease (RP-ILD) is a type of connective tissue disease (CTD) with rapid disease progression and high mortality.Traditional treatment regimens such as glucocorticoids, cyclophosphamide, and calcineurin inhibitors are available, and some of them are not effective. Tocilizumab is an IL-6 receptor (IL-6R) antagonist that is widely used to improve the symptoms and signs of autoimmune diseases as a commonly used drug in the treatment of connective tissue diseases such as rheumatoid arthritis and Takayasu's arteritis. Related studies have shown that it has a certain efficacy in alleviating the acute response of connective tissue disease-associated interstitial lung disease (CTD-ILD). This may be related to the increased levels of IL-6, KL-6, and serum ferritin in ILD. In this paper, we report two cases of successful treatment of dermatomyositis complicated with interstitial lung disease with tocilizumab, and review the literature to explore the diagnosis and treatment experience of DM ILD and further explore its potential mechanism.

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“…Various case reports have highlighted the efficacy of Tocilizumab (an anti-IL6R) in the treatment of refractory DM, ASS and anti-MDA5+ ADM, both with chronic fibrosing- and RP-ILD ( 60 – 63 ). However, a recent phase IIb trial comparing Tocilizumab to a placebo in the treatment of DM and PM patients failed to meet its primary endpoint ( 64 ).…”

Section: Introductionmentioning

confidence: 99%

Targeting intracellular pathways in idiopathic inflammatory myopathies: A narrative review

Rocca

Ferro²,

Baldini³

et al. 2023

Front. Med.

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In recent decades, several pieces of evidence have drawn greater attention to the topic of innate immunity, in particular, interferon (IFN) and Interleukin 6 in the pathogenesis of idiopathic inflammatory myopathies (IIM). Both of these molecules transduce their signal through a receptor coupled with Janus kinases (JAK)/signal transducer and activator of transcription proteins (STAT). In this review, we discuss the role of the JAK/STAT pathway in IIM, evaluate a possible therapeutic role for JAK inhibitors in this group of diseases, focusing on those with the strongest IFN signature (dermatomyositis and antisynthetase syndrome).

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NXP2‐positive dermatomyositis complicated with refractory skin edema: Successful treatment with tocilizumab

Cited by 6 publications

References 10 publications

Promising Neutrophil-Associated Biomarkers in Lung Diseases of Patients with Antisynthetase Syndrome and Dermatomyositis

Promising Neutrophil-Associated Biomarkers in Lung Diseases of Patients with Antisynthetase Syndrome and Dermatomyositis

Case study of tocilizumab in the treatment of dermatomyositis-associated interstitial lung disease

Targeting intracellular pathways in idiopathic inflammatory myopathies: A narrative review

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