Background Nucleotide‐binding oligomerization domain‐like receptor family, pyrin domain containing 3 (NLRP3)‐associated autoinflammatory disease (NLRP3‐AID) is a rare, heterogeneous disease entity associated with mutations in NLRP3. Biologic therapy for NLRP3‐AID yields diverse results. Objectives We aimed to evaluate the clinical features and outcomes of Chinese adult patients with NLRP3‐AID who were treated with tumour necrosis factor (TNF)‐α inhibitors. Methods Five patients with NLRP3‐AID were diagnosed and treated with TNF‐α inhibitors at Peking Union Medical College Hospital between 2017 and 2020 and were followed up for 6 to 12 months. All patients were systematically studied for treatment outcomes, including clinical manifestations and inflammatory markers. Results All five adult NLRP3‐AID patients were Chinese Han, and four patients were males. The mean age at disease onset was 4.2 ± 4.1 years, and the mean time of diagnosis delay was 19.8 ± 6 years. All patients received TNF‐α inhibitors with or without methotrexate/prednisone. During follow‐up, all patients achieved remarkable clinical remission of skin lesions and polyarthritis and showed improvements in acute‐phase reactants, inflammatory cytokines, patient visual analogue scale, physician global assessment and 36‐item Short Form (SF‐36). Conclusions Early diagnosis and effective therapy for NLRP3‐AID are essential for avoiding irreversible organ damage. TNF‐α inhibitors might serve as a therapeutic alternative for patients with NLRP3‐AID who have unsatisfactory responses or no access to interleukin‐1 inhibitors.

Systemic autoinflammatory diseases (SAIDs) are a group of rare diseases characterized by recurrent or continuous inflammation, typically accompanied by genetic variants. Good responses to anti-TNF therapy were observed in SAIDs patients. However, the mechanisms underlying the disease flare and the response to TNF blocking therapy have not been fully elucidated. Here, single-cell RNA sequencing technology was used to describe the transcriptomic profile of PBMCs and PMNs in two SAID patients both before and after anti-TNF treatment. Interferon responses were involved in the disease flare. After anti-TNF therapy, clinical symptoms were alleviated while TNF and IL-1 were unexpectedly increased, indicating that these inflammatory cytokines are not positively correlated with disease activity. Trajectory analysis showed that inhibition of macrophage differentiation, rather than reduction of the inflammatory cytokines, as the potential mechanism of anti-TNF treatment response in SAIDs.

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In recent years, the coal chemical industry project which can replace the petrochemical products is an investment hot spot. The Syngas-purification Unit is a main part of the coal chemical industry. Its role is: purify the syngas obtained from the gasification reaction (absorb the hydrogen sulfide and carbon dioxide). Yet, there is no research and the summary about the corrosion, failure mechanism and risk level of this unit. In this paper, the syngas-purification equipment of Sinopec Qilu Corporation were quantified by RBI analysis, and investigated the corrosion of the syngas-purification equipment, and discussed the device typical failure mechanism, and proposed the recommendations of the corrosion and on-line monitoring, etc. In last few years, the production of clean energy and alternative oil chemical products from the new coal chemical projects become of great interest[1]. Because the purification of the syngas is necessary, the syngas-purification unit is one of the main equipment of coal chemical industry. In the country, there is no systematic study or summary about the corrosion, failure mechanism and risk level of the device. The RBI analysis of the Syngas-purification unit is necessary, and has some guiding significance for the routine maintenance and periodic inspection of the device. This paper introduces the corrosion and the typical failure mechanism of Syngas-purification unit followed by the risk assessment results. Then the paper puts forward the suggestion on line monitoring.