OBJECTIVES:This study aimed to evaluate the efficacy and safety of mufemilast, a novel small-molecule selective phosphodiesterase 4 (PDE4) inhibitor, in patients with Behçet's syndrome (BS). METHODS:Patients diagnosed with BS according to the International Diagnostic (Classification) Criteria for Behçet's Disease 2013 and with active oral ulcers were eligible. Patients were randomly assigned to mufemilast (45 mg or 60 mg) or placebo, administered orally twice daily for 12 weeks. The primary endpoint was the area under the curve (AUC) for the number of oral ulcers from baseline to week 12. Safety was measured in all patients who received at least 1 dose of the study drug (ClinicalTrials: NCT04609397). RESULTS:Ninety patients were randomly assigned to the mufemilast 45 mg, 60 mg, or placebo groups (29, 31, and 30, respectively). The least-squares mean difference in AUC0-12 for oral ulcers between the 45 mg and 60 mg groups and the placebo was -104.8 (95% CI, -156.3 to -53.2; P < .001) and -142.5 (95% CI, -192.4 to -92.7; P < .001), respectively. The visual analogue pain scores and time to ulcer-free remission were significantly improved with mufemilast (P < .001). PDE4-related side effects were transient and resolved spontaneously, and discontinuation rates were low (7% for 45 mg, 7% for 60 mg, and 3% for placebo). No serious adverse events were reported related to the drug. CONCLUSIONS:Among patients with oral ulcers associated with BS, mufemilast significantly reduced the number of oral ulcers, improved pain scores, and induced significantly more ulcer-free remissions compared with placebo, with an acceptable safety profile.

BACKGROUND:Interstitial lung disease is one of the most severe pulmonary complications of connective tissue diseases and is associated with high mortality and poor prognosis due to the lack of effective therapies. Free fatty acid receptor 4, a receptor activated by long-chain unsaturated fatty acids, participates in the regulation of inflammatory responses; however, its direct role and underlying mechanisms in ILD-related pulmonary fibrosis have not yet been reported. METHODS:In this study, we investigated the effect of FFA4 on pulmonary fibrosis using both in vivo and in vitro models. A bleomycin-induced pulmonary fibrosis model was established in wild-type and FFAR4 knockout mice. In vitro, a Transwell co-culture system consisting of RAW264.7 macrophages and NIH3T3 fibroblasts was constructed. Transfection, transcriptomic analysis, dual-luciferase reporter assays, RT-qPCR, and Western blotting were performed to explore the molecular mechanisms mediated by FFA4. In addition, pharmacological interventions with the FFA4 agonist CpdA and the NF-κB inhibitor BAY11-7082 were used to evaluate the role of the NF-κB-IL-33 signaling axis in inflammation-driven fibrotic responses. RESULTS:FFA4 expression was reduced in the bleomycin-induced fibrosis model. In both the co-culture system and the mouse model, FFA4 deficiency significantly increased IL-33 expression and aggravated pulmonary fibrosis. In contrast, activation of FFA4 with CpdA reduced IL-33 expression and attenuated pulmonary fibrosis. Moreover, BAY11-7082 also suppressed IL-33 expression. Genetic deletion and pharmacological activation experiments further confirmed that the inhibitory effect of CpdA on IL-33 expression was dependent on FFA4. CONCLUSIONS:Collectively, these findings demonstrate that FFA4 restrains inflammatory signal amplification and suppresses pulmonary fibrosis progression by regulating the NF-κB-IL-33 signaling axis, suggesting that FFA4 may serve as a potential therapeutic target for pulmonary fibrosis.

Systemic sclerosis complicated with interstitial lung disease (SSc-ILD) is a rare autoimmune disease with a dismal prognosis. To analyze the common clinical indicators of systemic sclerosis (SSc) complicated with severe interstitial lung disease (ILD) patients based on the grading of lung high-resolution computed tomography (HRCT) and to explore the risk factors of severe (ILD). A retrospective analysis was performed on 72 newly diagnosed and treated SSc-ILD patients in the Affiliated Hospital of Xuzhou Medical University from June 2013 to August 2023. The interstitial lung disease was divided into grades 1, 2, and 3 according to the HRCT images. Grades 1 and 2 were classified as a non-severe group (42 cases), and grade 3 was a severe group (30 cases). The general data and laboratory examination results of the two groups were analyzed. A logistic regression model was established, and the receiver operating characteristic (ROC) curve was drawn to evaluate the predictive value of risk factors for severe SSc-ILD patients. Patients in the severe group had lower height (1.610 ± 0.059 vs 1.574 ± 0.078, P = 0.031), triglyceride (TG) (1.796 ± 0.743 vs 1.265 (1.04, 1.63), P = 0.026) and total protein (TP) (77.96 ± 8.784 vs 72.709 ± 8.042, P = 0.011)than those in the non-severe group. The area under the curve of height, TG, and TP combined with the diagnosis of systemic sclerosis complicated with severe interstitial lung disease was 0.839, and the sensitivity and specificity were 0.750 and 0.781, respectively. Low TG and TP may be the risk factors for SSc patients with severe interstitial lung disease. Therefore, it is promising as a potential therapeutic target for SSc-ILD and warrants appropriate follow-up in further studies of patients.

Introduction Antinuclear antibodies (ANA) are frequently positive in patients with anti-melanoma differentiation-associated gene 5-positive dermatomyositis (anti-MDA5+ DM). This study aimed to investigate the association between ANA and clinical characteristics as well as prognosis in a cohort of patients with anti-MDA5+ DM. Material and methods We conducted a systematic retrospective study of medical records from a Nanjing Medical University cohort of patients with myositis-associated interstitial lung disease (ILD). Various parameters were compared and analyzed between the ANA-positive group and the ANA-negative group. Results A total of 246 patients with anti-MDA5+ DM were enrolled in this study, with 28.5% males and 71.5% females. The median age was 53.0 years, the median disease duration was 2 months, and the median follow-up period was 12.0 months. The ANA positivity rate at baseline was 52.4% in anti-MDA5+ DM patients. The ANA-positive group showed significantly higher positivity rates of anti-Ro52 antibodies (72.9% vs. 54.7%, p = 0.003) and anti-aminoacyl-tRNA synthetase (anti-ARS) antibodies (9.3% vs. 2.6%, p = 0.033) compared to the ANA-negative group, but lower ALT levels: 39.0 (21.5, 79.3) vs. 51.3 (36.5, 95.8), p = 0.006. No significant differences were observed between the two groups in terms of overall survival, rapidly progressive interstitial lung disease (RPILD) incidence, age, disease duration, and clinical characteristics. In a subgroup analysis of the ANA-positive group, MDA5+++ patients had a higher incidence of RPILD compared to the MDA5+ group ( p = 0.028). In the ANA-negative subgroup analysis, MDA5+++ patients had a higher mortality rate and worse prognosis compared to the MDA5+ group ( p = 0.026). Multivariate Cox regression analysis showed that elevated lactate dehydrogenase (LDH) levels and the presence of rapidly progressive interstitial lung disease (RPILD) were associated with poor prognosis in ANA-negative anti-MDA5+ DM patients, with hazard ratios of 1.002 (95% CI: 1.001, 1.003, p = 0.020) and 13.694 (95% CI: 15.032, 37.267, p < 0.001), respectively. Conclusions ANA is frequently found in patients with anti-MDA5+ DM. High titers of anti-MDA5 antibodies are associated with mortality and RPILD.

KL130008 is a selective Janus kinase 1/2 inhibitor with encouraging preclinical efficacy. Phase I trials in healthy volunteers and patients with rheumatoid arthritis (RA) showed favorable pharmacokinetics, dose-dependent inhibition of phosphorylated signal transducer and activator of transcription 3, good tolerability, and no clinically meaningful pharmacokinetic interaction with methotrexate, supporting further evaluation in active RA. In this double-blind, placebo-controlled phase II trial at 24 centers in China, RA patients were randomized (1:1:1:1) to receive placebo or KL130008 capsules (0.5, 1 or 2 mg) once daily for 12 weeks. Week 13–24, the placebo group was switched 1:1 to KL130008 at 0.5 mg or 1 mg, while other groups continued their original doses. All patients received 7.5–25 mg methotrexate once weekly throughout 24 weeks. The primary outcome was the proportion of patients who achieved at least 20

Aims/Background Adult-onset Still’s disease (AOSD) shares similar clinical symptoms with sepsis. Thus, differentiating between AOSD and sepsis presents a great challenge while making diagnosis. This study aimed to analyse the changes in blood microbiota related to AOSD and sepsis using metagenomic next-generation sequencing (mNGS), identify potential biomarkers that distinguish AOSD from sepsis, and explore the diagnostic value of mNGS in differentiation between these two pathological conditions. Methods Clinical data of four AOSD patients and four sepsis patients treated in the Department of Rheumatology and Immunology, The Affiliated Hospital of Xuzhou Medical University between October 2021 and February 2022 were collected. The mNGS diagnostic records of these patients were analysed for microbial correlations in terms of species taxonomic structure and beta diversity by comparing blood microbiota between AOSD and sepsis. The biomarkers with the strongest capability in distinguishing the subgroups were screened using a random forest algorithm. Results There was no statistically significant differences between AOSD patients and sepsis controls in terms of gender and age (p > 0.05). A total of 91 operational taxonomic units (OTUs) were obtained. At the level of phylum, Proteobacteria, Ascomycota and Basidiomycota were present in high abundances in both groups (79.76%, 14.18% and 3.30% vs 54.03%, 32.77% and 5.81%). At the genus level, the abundances of Parainfluenzae, Aspergillus and Ralstonia were the top three highest in the AOSD group (73.88%, 10.92% and 5.48%), while Ralstonia, Aspergillus and Malassezia were ranked as the top three in the sepsis group in term of abundance (48.69%, 27.36% and 5.52%). In beta-diversity analysis, there were advances shown in visual principal coordinates analysis (PCoA) and non-metric multidimensional scaling (NMDS) between the AOSD group and sepsis group (p < 0.05), with little significant differences in the analysis of similarities (Anosim) (p > 0.05). Linear discriminant analysis effect size (LEfSe) showed that Mucoromycota, Saccharomycetes, Moraxellales, Mucorales, Xanthomonadales, Saccharomycetales, Acinetobacter, Stenotrophomonas, Yarrowia, Apophysomyces, Acinetobacter johnson, Yarrowia lipolytica, Apophysomyces variabilis and Stenotrophomonas maltophilia were more enriched in sepsis group (p < 0.05). The top five variables with the strongest capability in distinguishing between AOSD and sepsis were Acinetobacter johnsonii, Apophysomyces variabilis, Propionibacterium acnes, Stenotrophomonas maltophilia and Yarrowia lipolytica. Conclusion The blood microorganisms in AOSD were different from sepsis, and mNGS was potential to distinguish between AOSD and sepsis.

Aim: This study aimed to evaluate the long-term survival, causes of death, and prognostic factors in Chinese patients with primary Sjogren syndrome (pSS). Methods: We included patients with pSS registered in the Chinese Rheumatism Data Centre between May 2016 and December 2021, and collected baseline clinical, laboratory, and treatment data. Survival and standard mortality rates were calculated using general population mortality data. Factors related to mortality were identified using Cox proportional hazards regression. Results: Among the 8588 patients included, 274 died during a median follow-up of 4.0 years. The overall standardized mortality ratio was 1.61 (95% CI: 1.43-1.81). Overall survival rates were 98.2% at 5 years and 93.8% at 10 years. The predominant causes of death were comorbidities, including cardiovascular diseases, tumors, and infections, while the most frequent pSS-related causes of death were interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH). Male sex, older age, ILD, PAH, and high EULAR Sjogren's syndrome disease activity index (ESSDAI), thrombocytopenia, anemia, high immunoglobulin A (IgA) level, and glucocorticoid treatment independently increased the mortality risk, while using hydroxychloroquine was a protective factor. Conclusion: Mortality rates have significantly increased in Chinese patients with pSS. Comorbidities, rather than pSS-related organ damage, were the main causes of death. All-cause mortality was associated with male sex, older age, ILD, PAH, high ESSDAI, thrombocytopenia, anemia, high IgA level, and glucocorticoid treatment, whereas hydroxychloroquine use might improve the long-term survival.

Objective Although belimumab has been widely used in patients with systemic lupus erythematosus (SLE) globally, real-world safety data among Chinese patients are limited, particularly for children. This study assessed the safety and tolerability of belimumab in adult and paediatric patients with SLE in China in real-world clinical practice.Methods This Phase 4, multicentre, prospective, observational study enrolled patients prescribed intravenous belimumab by their physicians in tertiary hospitals, independent of a clinical study, during routine clinical visits between May 2021 and May 2022. Patients could have been receiving belimumab prior to enrolment. The primary objective was to describe the incidence of adverse events (AEs), serious AEs (SAEs), adverse drug reactions (ADRs) and AEs of special interest (AESIs) over the 24-week follow-up period. Data were collected at enrolment and approximately 4, 12 and 24 weeks post-enrolment, during routine clinical visits. AEs, ADRs and SAEs were collected as independent events. The safety population comprised patients who received >= 1 dose of belimumab and completed >= 1 follow-up visit.Results Overall, 417 patients were included in the analysis (safety population); 89.2% were female and mean (standard deviation) age was 35.5 (11.9) years. AEs were reported in 158 patients (37.9%) and were mostly mild (79.1%). The most common AEs were upper respiratory tract infections (n = 19, 4.6%) and hypokalaemia (n = 18, 4.3%; all mild). Among 22 patients (5.3%) with SAEs, four patients (1.0%) had drug-related SAEs (pneumonia, respiratory tract infection, gingivitis and decreased white blood cell and neutrophil count). ADRs were experienced by 25 patients (6.0%), most commonly urinary tract infections (n = 5, 1.2%). There were no fatal SAEs. AESIs occurred in 14 patients (3.4%), including infections of interest (n = 5, 1.2% all herpes zoster), serious selected psychiatric events (n = 3, 0.7%) and infusion-related systemic and anaphylactic reactions (n = 7, 1.7%). Of 14 paediatric patients enrolled, six experienced AEs, zero ADRs, two SAEs and one AESI.Conclusion Belimumab was generally well tolerated in adult and paediatric patients with SLE in this real-world setting in China, with no new safety signals identified. Future assessment of long-term belimumab safety in China beyond 24 weeks will extend this current body of evidence.

Based on partially linear additive individualized model, a fusion-penalized inverse probability weighted least squares method is proposed to detect the subgroup for missing data in response. Firstly, the B-spline technique is used to approximate the unknown additive individualized functions and then an inverse probability weighted quadratic loss function is established with fusion penalty on the difference of subject-wise B-spline coefficients. Secondly, minimization of such quadratic loss function leads to the estimation of linear regression parameters and individualized B spline coefficients. With a proper tuning parameter, some differences in penalty term are shrunk into zero and thus the corresponding subjects will be clustered into the same subgroup. Thirdly, a clustering method is developed to automatically determine the subgroup membership for the subjects with missing data. Fourthly, large sample properties of resulting estimates are given under some regular conditions. Finally, numerical studies are presented to illustrate the performance of the proposed subgroup detection method.

Objective We here investigate the relationship between the appearance of skin rash, complement, and the risk of systemic lupus erythematosus and lupus nephritis. Methods All data were collected from 71 patients with SLE (without LN) and 200 patients with LN treated at our hospital from August 2018 to August 2023. The latter group was further categorized into a high eGFR group (eGFR ≥ 60 ml/min; 100 cases) and a low eGFR group (eGFR < 60 ml/min; 100 cases). Basic clinical characteristics such as gender, age, fever, joint pain, rash, hair loss, along with laboratory indicators including cystatin C, complement C3, complement C4, and anti-dsDNA titers were gathered. The differences in clinical characteristics and hematological indicators between the SLE group and the LN group, as well as between the high eGFR group and the low eGFR group patients, were compared. Binary logistic regression analysis was employed to identify independent risk factors for the progression from SLE to LN and independent risk factors for the deterioration of renal function in LN. Correlation studies were conducted to elucidate the relationship between independent factors and the disease. The predictive value of risk was assessed using ROC curves. Results Compared to the SLE group, the absence of skin rash and low complement C3 levels were significantly associated with the occurrence of LN. Multifactor analysis revealed that both skin rash (OR: 0.231, P < 0.001) and complement C3 (OR: 0.080, P < 0.001) were influencing factors for the development of lupus nephritis, while SLEDAI scores consistently showed no statistical significance. The combination of no skin rash and low complement C3 levels had an area under the curve (AUC) of 0.708 in relation to LN, with a diagnostic sensitivity of 0.83 and specificity of 0.70, demonstrating good predictive efficacy. In the progression of LN, the absence of skin rash and low levels of complement C3 and C4 were significant in differential and correlation analyses; however, in multifactor analysis, low levels of complement C3 and C4 showed no statistical significance with p-value > 0.05. SLEDAI scores remained statistically insignificant, possibly due to treatment-induced differences. Conclusion The absence of skin rash and low levels of complement C3 are risk factors for the occurrence of LN, and their combined predictive diagnostic value is higher. Disease activity may not necessarily be the sole factor for further deterioration of kidney function.

Rheumatoid arthritis-associated interstitial lung disease (RA-ILD) is characterized by a high incidence and mortality rate, highlighting the need for biomarkers to detect ILD early in RA patients. Previous studies have shown the protective effects of Interleukin-22 (IL-22) in pulmonary fibrosis using mouse models. This study aims to assess IL-22 expression in RA-ILD to validate foundational experiments and explore its diagnostic value. The study included 66 newly diagnosed RA patients (33 with ILD, 33 without ILD) and 14 healthy controls (HC). ELISA was utilized to measure IL-22 levels and perform intergroup comparisons. The correlation between IL-22 levels and the severity of RA-ILD was examined. Logistic regression analysis was employed to screen potential predictive factors for RA-ILD risk and establish a predictive nomogram. The diagnostic value of IL-22 in RA-ILD was assessed using ROC. Subsequently, the data were subjected to 30-fold cross-validation. IL-22 levels in the RAILD group were lower than in the RA-No-ILD group and were inversely correlated with the severity of RA-ILD. Logistic regression analysis identified IL-22, age, smoking history, anti-mutated citrullinated vimentin antibody (MCV-Ab), and mean corpuscular hemoglobin concentration (MCHC) as independent factors for distinguishing between the groups. The diagnostic value of IL-22 in RA-ILD was moderate (AUC = 0.75) and improved when combined with age, smoking history, MCV-Ab and MCHC (AUC = 0.97). After 30-fold cross-validation, the average AUC was 0.886. IL-22 expression is dysregulated in the pathogenesis of RA-ILD. This study highlights the potential of IL-22, along with other factors, as a valuable biomarker for assessing RA-ILD occurrence and progression.

BackgroundThe prognosis of anti-melanoma differentiation-associated gene 5 positive dermatomyositis (anti-MDA5+DM) is poor and heterogeneous. Rapidly progressive interstitial lung disease (RP-ILD) is these patients’ leading cause of death. We sought to develop prediction models for RP-ILD risk in anti-MDA5+DM patients.MethodsPatients with anti-MDA5+DM were enrolled in two cohorts: 170 patients from the southern region of Jiangsu province (discovery cohort) and 85 patients from the northern region of Jiangsu province (validation cohort). Cox proportional hazards models were used to identify risk factors of RP-ILD. RP-ILD risk prediction models were developed and validated by testing every independent prognostic risk factor derived from the Cox model.ResultsThere are no significant differences in baseline clinical parameters and prognosis between discovery and validation cohorts. Among all 255 anti-MDA5+DM patients, with a median follow-up of 12 months, the incidence of RP-ILD was 36.86%. Using the discovery cohort, four variables were included in the final risk prediction model for RP-ILD: C-reactive protein (CRP) levels, anti-Ro52 antibody positivity, short disease duration, and male sex. A point scoring system was used to classify anti-MDA5+DM patients into moderate, high, and very high risk of RP-ILD. After one-year follow-up, the incidence of RP-ILD in the very high risk group was 71.3% and 85.71%, significantly higher than those in the high-risk group (35.19%, 41.69%) and moderate-risk group (9.54%, 6.67%) in both cohorts.ConclusionsThe CROSS model is an easy-to-use prediction classification system for RP-ILD risk in anti-MDA5+DM patients. It has great application prospect in disease management.

IntroductionTo identify the clinical characteristics and risk factors for cutaneous ulcers in patients with anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis (anti-MDA5+ DM) combined with rapidly progressive interstitial lung disease (RPILD).Material and methodsWe conducted a retrospective cohort study on the medical records of patients enrolled from the Nanjing Medical University Myositis-associated ILD cohort (NMMI). The clinical characteristics of patients in the ulcer-positive group were compared with those in the ulcer-negative group by chi-square or Fisher’s exact test. Univariate and multivariate logistic regression analyses were used to assess risk factors for the development of cutaneous ulcers.ResultsA total of 246 patients with anti-MDA5+ DM were retrospectively enrolled in the study, including 176 females (176/246, 71.54%) and 70 males (70/246, 28.46%), with a female-to-male ratio of 2.51 : 1. Among the 246 patients, a total of 88 cases (88/246, 35.77%) with anti-MDA5+ DM combined with RPILD were further studied, including 55 females (55/88, 62.5%) and 33 males (33/88, 37.5%), with a female-to-male ratio of 1.67 : 1. Twelve patients (12/88, 13.64%) had cutaneous ulcers. In terms of clinical characteristics, patients in the ulcer-positive group had significantly more proximal muscle involvement (83.33% vs. 38.16%, p = 0.003) and more heliotrope rash (83.33% vs. 43.42%, p = 0.010) than in the ulcer-negative group. In univariate analysis, cutaneous ulcers were associated with proximal muscle involvement (OR = 8.103; 95% CI: 1.657–39.625; p = 0.010) and heliotrope rash (OR = 6.515; 95% CI: 1.336–31.773; p = 0.020). In multivariate analysis, cutaneous ulcers were associated with proximal muscle involvement (OR = 6.436; 95% CI: 1.274–32.524; p = 0.024), and proximal muscle involvement was an independent risk factor for cutaneous ulcers.ConclusionsWe confirmed the association between cutaneous ulcers and proximal muscle involvement and heliotrope rash in patients with anti-MDA5+ DM combined with RPILD. Proximal muscle involvement is an independent risk factor for cutaneous ulcers.

Objective To explore the effectiveness and safety of belimumab in Chinese adult lupus nephritis (LN) patients under a real-world setting. Methods For this retrospective cohort study, 30 LN patients received belimumab plus standard of care (SoC) due to a new onset or a relapse of LN from June 2021 to December 2022. And 30 matched patients only treated with SoC after propensity score matching (PSM) at the same period were also enrolled. After 24-week follow-ups, renal complete remission (CR), disease activity index, 24 h urine protein level, dose of prednisone and incidence of adverse events were compared. Results No statistically significant differences existed in baseline demographics, complement C3/C4, anti-dsDNA autoantibody titer, 24 h urine protein level or disease activity index. At Week 24, 21/30 patients achieved renal CR and it was higher than that of SoC group (21 vs 12, P=0.037). As compared with SoC group, belimumab group had lower levels of 24 h urine protein[346.50(183.75，571.00) mg vs 611.50(360.00，1450.00) mg], SLEDAI-2K (SLE disease activity index, SLEDAI-2K) score[(3.93±2.79) vs (5.70±3.14)] and daily prednisone dose [(11.67±4.34) mg/d vs (22.42±9.23) mg/d](P＜0.05). Furthermore, the incidence of adverse events between two group was comparable (P>0.05). Conclusions Belimumab plus SoC may alleviate LN, boost the rate of renal CR, lower 24 h urine protein levels, improve disease activity and reduce the intake of prednisone.

ObjectiveInterstitial lung disease (ILD) is a common extramuscular complication contributing to significant morbidity and mortality in patients with dermatomyositis (DM) who are positive for antimelanoma differentiation–associated gene 5 antibody (anti-MDA5+). We conducted this study to investigate the association of anti-Ro52 antibodies with clinical characteristics and prognosis in patients with anti-MDA5+ DM.MethodsWe assessed a cohort of 246 patients with anti-MDA5+ DM. To calculate hazard ratios and 95% CIs for rapidly progressive ILD (RP-ILD) and death while controlling for potential confounders, variables selected by univariate Cox regression analysis were included in a multivariate Cox regression model with the stepwise forward-selection method. A 2-tailed analysis withP< 0.05 was considered to be statistically significant.ResultsA total of 246 patients with anti-MDA5+ DM were enrolled; 70 patients were male, and the patient group had an average age of 53.1 (12.4) years. Anti-Ro52 was present in 64.2% (158/246) patients. Patients with anti-MDA5+ DM who were positive for anti-Ro52 had a higher rate of RP-ILD (log-rankP< 0.001) and a higher mortality rate (log-rankP= 0.01). For patients with anti-MDA5+ DM who were positive for anti-Ro52, those with a short disease course and high inflammation were at increased risk of RP-ILD and death. The appearance of active rash was an independent protective factor of death.ConclusionAnti-Ro52 antibodies were highly prevalent in patients with anti-MDA5+ DM, and their coexistence correlated with a higher rate of RP-ILD and mortality. Patients with a short disease course, with increased inflammation, and without rash were more likely to have a poor prognosis.

Abstract Objectives: To explore the Association between human herpes simplex virus(HSV) type 1 or type 2 infection and the risk of rheumatoid arthritis(RA), and what is this relationship. Methods: We evaluated the associations of HSV-1/2 antibody levels with the risk of RA among U.S. adults from the National Health and Nutrition Examination Survey (NHANES), 2001-2016. We developed four independent multivariate logistic regression models to evaluate the association between HSV-1/2 infection and the risk for RA the population. Results: Finally, we analyzed 1346, 1343 and 1343 subjects, respectively. In all models, HSV-1 infection significantly reduced the prevalence of RA in adults aged 18-49 years, with the lowest odds ratio (OR) (after weighting: OR 0.73, 95% CI 0.72, 0.73), whereas HSV-2 infection was positively associated with an increased prevalence of RA in population aged 18-49 years, with the highest value of the OR (after weighting: OR 1.69, 95% CI 1.69, 1.69), after correction for confounders such as age, sex, race, education level, marital status, smoking,alcohol, diabetes, hypertension, hyperlipidemia and missing values or removing the effect of the interaction between the two viruses, these connections still exist. Conclusion: In summary, these findings indicated that HSV-1 infection can reduce the prevalence of RA in adults, while HSV-2 infection is positively associated with the prevalence of RA in adults. However, our findings need more powerful to prove these associations through rigorously designed prospective studies.

This study aimed to develop nomogram prediction models to differentiate between adult-onset Still’s disease (AOSD) and sepsis. We retrospectively collected laboratory test data from 107 hospitalized patients with AOSD and sepsis at the Affiliated Hospital of Xuzhou Medical University. Multivariate binary logistic regression was used to develop nomogram models using arthralgia, WBC, APTT, creatinine, PLT, and ferritin as independent factors. The performance of the model was evaluated by the bootstrap consistency index and calibration curve. Model 1 had an AUC of 0.98 (95