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Systemic lupus erythematosus (SLE) is becoming a growing public health concern due to increasing disease and economic burdens. Epidemiological information about SLE, especially its incidence rate, is limited in developing countries. In the current study, we sought to investigate the incidence, prevalence, and economic burdens of SLE in urban China. We conducted a nationwide population-based cohort study using databases from Urban Employee Basic Medical Insurance and Urban Resident Basic Medical Insurance between 2013 and 2017, covering approximately 300 million residents in 23 provincial regions in China. Incidence rate and prevalence were standardized by age and gender to China's 2010 national census data. Additionally, we calculated the average annual costs and hospital visit rates. A total of 132,258 SLE patients were identified during the study period, with a mean age of 43.03 years (standard deviation: 15.29 years). Of these patients, 81.33% were women. In 2017, the standardized incidence rate of SLE in China was 14.09 (95% confidence interval (CI), 11.95-16.41) per 100,000 person-years, with a higher incidence in women than in men (26.41 vs. 5.92 per 100,000 person-years). Standardized prevalence in 2017 was 47.61 (41.77-53.83), 94.16 (80.67-108.69), and 17.86 (13.84-22.38) per 100,000 people in the overall, female, and male populations, respectively. The average annual rates of increase in prevalence were 21.50%, 19.72%, and 25.67% from 2013 to 2017 in the overall, female, and male populations, respectively. The age-specific incidence rates peaked at 30-49 years old in women and 40-59 years old in men. SLE incident and prevalent cases were most common in North-West China and less common in southern and eastern China. Distinct variations in incidence rates across different regions are also consistent with the varying levels of ultraviolet radiation exposure in China. Additionally, the average estimated annual per-capita cost was 1599.34 US dollars in SLE patients, with the highest costs observed in adolescent and young adult patients among different age groups. The SLE population in China is rapidly expanding, and younger at onset, especially in women, which has placed significant burdens on China's healthcare system.
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BACKGROUND: Serologically active clinically quiescent (SACQ) is a state within systemic lupus erythematosus (SLE) characterized by elevated serologic markers without clinical activity. The heterogeneity in SACQ patients poses challenges in disease management. This multicenter prospective study aimed to identify distinct SACQ subgroups and assess their utility in predicting organ damage. METHODS: SACQ was defined as a sustained period of at least 6 months with persistent serologic activity, marked by positive anti-double-stranded DNA (dsDNA) antibodies and/or hypocomplementemia, and without clinical activity. Cluster analysis was employed, utilizing 16 independent components to delineate phenotypes. FINDINGS: Among the 4,107 patients with SLE, 990 (24.1%) achieved SACQ within 2.0 ± 2.3 years on average. Over a total follow-up of 7,105.1 patient years, 340 (34.3%) experienced flares, and 134 (13.5%) developed organ damage. Three distinct SACQ subgroups were identified. Cluster 1 (n = 219, 22.1%) consisted predominantly of elderly males with a history of major organ involvement at SLE diagnosis, showing the highest risk of severe flares (16.4%) and organ damage (27.9%). Cluster 2 (n = 279, 28.2%) was characterized by milder disease and a lower risk of damage accrual (5.7%). Notably, 86 patients (30.8%) in cluster 2 successfully discontinued low-dose glucocorticoids, with 49 of them doing so without experiencing flares. Cluster 3 (n = 492, 49.7%) featured the highest proportion of lupus nephritis and a moderate risk of organ damage (11.8%), with male patients showing significantly higher risk of damage (hazard ratio [HR] = 4.51, 95% confidence interval [CI], 1.82-11.79). CONCLUSION: This study identified three distinct SACQ clusters, each with specific prognostic implications. This classification could enhance personalized management for SACQ patients. FUNDING: This work was funded by the National Key R&D Program (2021YFC2501300), the Beijing Municipal Science & Technology Commission (Z201100005520023), the CAMS Innovation Fund (2021-I2M-1-005), and National High-Level Hospital Clinical Research Funding (2022-PUMCH-D-009).
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OBJECTIVES: The predominant determinant of an unfavorable prognosis among Systemic Lupus Erythematosus (SLE) patients resides in the irreversible organ damage. This prospective cohort study aimed to identify the additional value of anti-nucleosome antibodies on organ damage accumulation in SLE patients. METHODS: Based on the Chinese SLE Treatment and Research group (CSTAR) registry, demographic characteristics, autoantibodies profiles, and clinical manifestations were collected at baseline. Follow-up data were collected by reviewing clinical records. RESULTS: Of 2481 SLE patients with full follow-up data, 663 (26.7%) were anti-nucleosome antibodies positive and 1668 (68.0%) were anti-dsDNA antibodies positive. 764 (30.8%) patients developed new organ damage during a mean follow-up of 4.31 ± 2.60 years. At baseline, patients with positive anti-nucleosome antibodies have a higher rate of lupus nephritis (50.7% vs 36.2%, p < .001). According to the multivariable Cox regression analysis, both anti-nucleosome (HR = 1.30, 95% CI, 1.09-1.54, p < .001) and anti-dsDNA antibodies (HR=1.68, 95% CI, 1.38-2.05, p < .001) were associated with organ damage accumulation. Anti-nucleosome (HR = 2.51, 95% CI, 1.81-3.46, p < .001) and anti-dsDNA antibodies (HR = 1.69, 95% CI, 1.39-2.06, p < .001) were independent predictors for renal damage. Furthermore, the combination of the two antibodies can provide more accurate information about renal damage in overall SLE patients (HR = 3.19, 95% CI, 2.49-4.10, p < .001) and patients with lupus nephritis at baseline (HR = 2.86, 95% CI, 2.29-3.57, p < .001). CONCLUSION: Besides anti-dsDNA antibodies, anti-nucleosome antibodies can also provide information about organ damage accrual during follow-up. The ability of co-positivity of anti-nucleosome and anti-dsDNA antibodies in predicting renal damage may lead to additional benefits in the follow-up of these patients.
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Anticuerpos Antinucleares , Lupus Eritematoso Sistémico , Nefritis Lúpica , Nucleosomas , Humanos , Femenino , Masculino , Adulto , Nucleosomas/inmunología , Estudios Prospectivos , Anticuerpos Antinucleares/inmunología , Anticuerpos Antinucleares/sangre , Nefritis Lúpica/inmunología , Lupus Eritematoso Sistémico/inmunología , Lupus Eritematoso Sistémico/complicaciones , Persona de Mediana Edad , Pronóstico , Modelos de Riesgos Proporcionales , Adulto Joven , Autoanticuerpos/sangre , Autoanticuerpos/inmunología , Sistema de Registros , China , Riñón/inmunología , Riñón/patología , Análisis Multivariante , Estudios de SeguimientoRESUMEN
Background and Objectives: MicroRNAs (miRNAs) represent a new class of biomarkers in the context of connective tissue disorders. The miRNA expression profiles in peripheral blood mononuclear cells (PBMCs) of patients with polymyositis (PM) and dermatomyositis (DM) have not been fully elucidated. The objective is to investigate miRNAs expression profile in PBMCs of patients with PM/DM. Methods: Microarray technology was used to identify differentially expressed miRNAs in PBMCs obtained from 6 untreated PM/DM patients and 3 healthy controls (HCs). TaqMan-based stem-loop real-time PCR detection was used for validation in a cohort of 34 PM/DM patients and 20 HCs. Results: Microarray analysis revealed 38 differentially expressed miRNAs (24 up-regulated and 14 down-regulated) in PM/DM patients compared to HCs. Four miRNAs (miR-320a, miR-335-3p, miR-34a-5p and miR-454-3p) were chosen for real-time PCR validation. The expression of miR-34a-5p was significantly upregulated in PM/DM group (P < 0.05). In subgroup analysis, miR-34a-5p was significantly upregulated in interstitial lung disease (ILD) group and DM group (P < 0.001). The level of SIRT1, a validated target of miR-34a, was significantly lower in PBMCs of PM/DM patients compared with HCs. Conclusions: MiR-34a-5p may potentially participate in the pathogenesis of PM/DM through SIRT1, and may serve as a potential new biomarker for PM/DM-ILD.
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BACKGROUND: Melanoma differentiation-associated gene 5 (MDA5), as a cytoplasmic sensor for viral double-stranded RNAs, has received increasing attention in recent years. Although considerable headway has been made on the functional role of MDA5 in antiviral immunity and autoimmune disease, the available literature is insufficient to assess the vast field. METHODS: This study performed a bibliometric analysis to investigate current hotspots in the global scientific output of MDA5 over the past two decades. Related publications and recorded information from 2002 to 2022 in the Web of Science Core Collection (WoSCC) database were retrieved. VOSviewer and CiteSpace were used for quantitative evaluation and visualization. RESULTS: A total of 2267 original articles and reviews were obtained, and the annual number of publications related to MDA5 was increasing rapidly. China has published the most papers, while the USA was the most influential country with the most citations and the highest H-index. The Chinese Academy of Sciences, the United States Department of Health and Human Services, and the Journal of Virology were the most prolific research affiliation, funding source, and journal, respectively. Fujita T (Kyoto University) was the most productive author with the highest H-index and had close cooperation with Kato H and Yoneyama M. The keywords "RIG-I," "MDA5," "innate immunity," "double-stranded-RNA," and "recognition" had the highest frequency, while "dermatomyositis" as well as "autoantibody" seemed to be the emerging hotspots. CONCLUSION: This study comprehensively demonstrated the research frontiers of MDA5 and will provide a useful resource for scholars to conduct future decisions. KEY POINTS: We conducted the first in-depth survey of the research frontiers on melanoma differentiation-associated gene 5 (MDA5) over the past two decades via bibliometric analysis. We found that many early breakthroughs have been made in the mechanism of MDA5-mediated antiviral immune responses, and the role of MDA5 in autoimmune and autoinflammatory diseases has raised the recent concern. We identified that the virus infection-associated pathogenesis and effective therapeutic strategy of anti-MDA5 antibody-positive dermatomyositis will remain the hotspots in the future.
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Enfermedades Autoinmunes , Helicasa Inducida por Interferón IFIH1 , ARN Viral , Humanos , Autoanticuerpos/inmunología , Enfermedades Autoinmunes/inmunología , Enfermedades Autoinmunes/virología , Bibliometría , China , Virus ARN Bicatenario/inmunología , Helicasa Inducida por Interferón IFIH1/inmunología , ARN Bicatenario/inmunología , ARN Viral/inmunología , Estados UnidosAsunto(s)
Cardiomiopatías , Miositis , Humanos , Miositis/complicaciones , Miositis/diagnóstico , FibrosisRESUMEN
OBJECTIVE: To investigate the health-related quality of life (HR-QoL), work productivity and activity impairment and associated factors among patients with idiopathic inflammatory myopathy (IIM). METHODS: This was an observational, cross-sectional study. The 189 ambulatory patients with IIM were recruited from May 2019 to May 2022. HR-QoL was measured by the European Quality of Life 5-Dimension (EQ-5D) questionnaire. The Work Productivity and Activity Impairment (WPAI) questionnaire was used to evaluate work productivity and activity impairment. The IIM-related parameters were assessed by the 8-item Manual Muscle Test (MMT-8), Myositis Disease Activity Assessment visual analogue scale (MYOACT), Myositis Damage Index (MDI), Disease Activity Score (DAS) and Physician/Patient Global Assessment (PhGA/PtGA). Quantile regression and ordinal logistic regression were performed to identify the factors, considering EQ-5D or WPAI scores as dependent variables, respectively. RESULTS: Of the 189 IIM patients enrolled, 60% had DM, 13% had PM and 27% had clinical amyopathic DM. The median EQ-5D score was 1.00 (95% CI 0.73, 1.00), 28% were employed and 45% of overall work was impaired due to health problems. EQ-5D values were positively associated with MMT-8 and negatively with MYOACT, DAS, MDI-global and PhGA/PtGA. For the WPAI, activity impairment was associated with a lower MMT-8 score, older onset age and higher PhGA only in 25th-75th percentile. Increased PtGA was associated with increased activity and overall working productivity impairment in most quantiles (P<0.05). CONCLUSION: Multiple disease characteristics were associated with reduced HR-QoL or working productivity impairment in patients with IIM, especially for PtGA.
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Miositis , Calidad de Vida , Humanos , Miositis/complicaciones , Eficiencia , Medición de Resultados Informados por el Paciente , Estudios TransversalesRESUMEN
Diabetes mellitus (DM) is a chronic metabolic disorder that affects multiple organs and systems, including the pulmonary system. Pulmonary dysfunction in DM patients has been observed and studied for years, but the underlying mechanisms have not been fully understood. In addition to traditional mechanisms such as the production and accumulation of advanced glycation end products (AGEs), angiopathy, tissue glycation, oxidative stress, and systemic inflammation, recent studies have focused on programmed cell deaths (PCDs), especially the non-apoptotic ones, in diabetic pulmonary dysfunction. Non-apoptotic PCDs (NAPCDs) including autophagic cell death, necroptosis, pyroptosis, ferroptosis, and copper-induced cell death have been found to have certain correlations with diabetes and relevant complications. The AGE-AGE receptor (RAGE) axis not only plays an important role in the traditional pathogenesis of diabetes lung disease but also plays an important role in non-apoptotic cell death. In this review, we summarize novel studies about the roles of non-apoptotic PCDs in diabetic pulmonary dysfunction and focus on their interactions with the AGE-RAGE axis.
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Muerte Celular Autofágica , Diabetes Mellitus , Humanos , Productos Finales de Glicación Avanzada/metabolismo , Receptor para Productos Finales de Glicación Avanzada/metabolismo , ApoptosisRESUMEN
INTRODUCTION: Baricitinib, a JAK1/JAK2 inhibitor, is approved for treatment of moderate-to-severe rheumatoid arthritis (RA) in China. This single-arm, prospective, multi-center, post-marketing safety study (PMSS) evaluated the safety and effectiveness of baricitinib in Chinese patients. METHODS: This study included adult patients with moderate-to-severe active RA who received baricitinib over periods of approximately 12 and 24 weeks. The primary endpoint was safety, defined as week 12 adverse event (AE)/serious AE incidence. Secondary endpoints were week 24 safety and effectiveness (disease activity score with 28 joints/C-reactive protein [DAS28-CRP] and simplified/Clinical Disease Activity Index [SDAI/CDAI]). RESULTS: Safety analyses included 667 patients (female, 82.3%; mean age, 53.3 years; mean RA duration, 86.9 months); 106/667 (15.9%) were 65-74 years old and 19/667 (2.8%) were ≥ 75 years old; 87.0% received baricitinib 2 mg QD. Total exposure was 262.1 patient-years (PY). At week 12, AEs had occurred in 214 (32.1%; exposure-adjusted incidence rate [EAIR], 172.5 per 100 PY) patients (serious AEs: 22 [3.3%; EAIR, 15.0]). At week 24, AEs had occurred in 250 (37.5%; EAIR, 125.9) patients (serious AEs: 28 [4.2%; EAIR, 10.9]). Two patients (0.3%) died (of pneumonia and unknown cause); EAIR for death, 0.77. Serious infection occurred in 1.2% of patients (EAIR, 3.1). Hepatotoxicity occurred in 3.4% of patients (EAIR, 9.0). No patients met potential Hy's law laboratory criteria (alanine/aspartate aminotransferases ≥ 3 × upper limit of normal (ULN) and total bilirubin ≥ 2 × ULN). Malignancy occurred in one patient. No patients experienced venous thromboembolism (VTE) or major adverse cardiovascular events (MACE). At week 24, 52.4%, 27.5%, and 27.6% of patients achieved remission per DAS28-CRP, SDAI, and CDAI, respectively. CONCLUSIONS: This PMSS investigated the safety and effectiveness of baricitinib in clinical practice in China. No VTE/MACE or new safety signals were reported and there was promising effectiveness, supporting the use of baricitinib in Chinese patients with moderate-to-severe active RA. TRIAL REGISTRATION: EU PAS Register: EUPAS34213.
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BACKGROUND: Janus kinase (JAK) inhibitors have been proven to be effective and safe in various autoimmune diseases. However, there is still a lack of comprehensive evidence regarding their efficacy and safety in systemic and cutaneous lupus erythematosus. METHODS: We searched for systemic and cutaneous lupus erythematosus patients who were treated with JAK inhibitors in PubMed, Embase, Web of Science, and the Cochrane Library until February 28, 2023. The quality of clinical trials was assessed using the Cochrane risk-of-bias tool. Meta-analysis was conducted when at least three studies had comparable measures of outcome. If meta-analysis was not feasible, a descriptive review was carried out. RESULTS: We included 30 studies, consisting of 10 randomized controlled trials and 20 case series or reports, with a total of 2,460 patients. JAK inhibitors were found to be more effective than placebo in systemic lupus erythematosus (SLE) based on the percentage of achieving SLE Responder Index (SRI)-4 response (RR = 1.18; 95% CI 1.07 to 1.31; p = 0.001), British Isles Lupus Assessment Group -based Composite Lupus Assessment (BICLA) response (RR = 1.16; 95% CI 1.02 to 1.31; p = 0.02), Lupus Low Disease Activity State (LLDAS) (RR = 1.28; 95% CI 1.07 to 1.54; p = 0.008), and Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2 K) remission of arthritis or rash (RR = 1.09; 95% CI 1.00 to 1.18; p = 0.04), particularly in treating musculoskeletal and mucocutaneous involvement. However, the effect of JAK inhibitors on cutaneous lupus erythematosus was uncertain. JAK inhibitors and placebo had a similar incidence of adverse events (RR = 1.01; 95% CI 0.97 to 1.04; p = 0.65). CONCLUSION: JAK inhibitors could be a potential treatment option for systemic and cutaneous lupus erythematosus, particularly in treating cutaneous and musculoskeletal lesions of SLE. JAK inhibitors had a safe profile.
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Artritis , Inhibidores de las Cinasas Janus , Lupus Eritematoso Cutáneo , Lupus Eritematoso Sistémico , Humanos , Inhibidores de las Cinasas Janus/efectos adversos , Lupus Eritematoso Sistémico/tratamiento farmacológico , Lupus Eritematoso Cutáneo/tratamiento farmacológico , Piel , Resultado del TratamientoRESUMEN
ABSTRACT: Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease with high prevalence and possible poor prognosis. Though the pathogenesis of pSS has not been fully elucidated, B cell hyperactivity is considered as one of the fundamental abnormalities in pSS patients. It has long been identified that Janus kinases-signal transducer and activator of transcription (JAK-STAT) signaling pathway contributes to rheumatoid arthritis and systemic lupus erythematosus. Recently, increasing numbers of studies have provided evidence that JAK-STAT pathway also has an important role in the pathogenesis of pSS via direct or indirect activation of B cells. Signal transducer and activator of transcription 1 (STAT1), STAT3, and STAT5 activated by various cytokines and ribonucleic acid contribute to pSS development, respectively or synergically. These results reveal the potential application of Janus kinase inhibitors for treatment of pSS, which may fundamentally improve the quality of life and prognosis of patients with pSS.
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OBJECTIVES: This study aimed to investigate the clinical characteristics, outcomes, and associated factors of patients with systemic lupus erythematosus-associated diffusive alveolar hemorrhage (SLE-DAH) stratified by infection status in a national representative cohort. METHODS: This single-center retrospective study included 124 consecutive patients with SLE-DAH in a tertiary care center between 2006 and 2021. The diagnosis of DAH was made based on a comprehensive evaluation of clinical manifestations, laboratory and radiologic findings, and bronchoalveolar lavage. Demographics, clinical features, and survival curves were compared between patients with bacterial, non-bacterial, and non-infection groups. Univariate and multivariate logistic regression analyses were performed to determine the factors independently associated with bacterial infection in SLE-DAH. RESULTS: Fifty-eight patients with SLE-DAH developed bacterial infection after DAH occurrence, thirty-two patients developed fungal and/or viral infection, and thirty-four patients were categorized as non-infection. The bacterial infection group have a worse prognosis (OR 3.059, 95%CI 1.469-6.369, p = 0.002) compared with the other two groups, with a mortality rate of 60.3% within 180 days after DAH occurrence. Factors independently associated with bacterial infections in SLE-DAH included hematuria (OR 4.523, 95%CI 1.068-19.155, p = 0.040), hemoglobin drop in the first 24 h after DAH occurred (OR 1.056, 95%CI 1.001-1.115, p = 0.049), and anti-Smith antibody (OR 0.167, 95%CI 0.052-0.535, p = 0.003). Glucocorticoid pulse therapy and cyclophosphamide were administered in more than 50% of patients regardless of their infectious status. According to clinical experience at our hospital and in previous studies, we recommended a comprehensive management algorithm for SLE-DAH based on infection stratification. CONCLUSION: Infection, especially bacterial infection, is a severe complication and prognostic factor of SLE-DAH. Comprehensive management strategies, including diagnosis, evaluation, treatment, and monitoring, based on infection stratification may fundamentally improve outcomes of patients with SLE-DAH. Key Points ⢠Bacterial infection is an important, but neglected, prognosis factor of systemic lupus erythematosus (SLE)-associated diffusive alveolar hemorrhage (DAH). ⢠Hematuria, hemoglobin drop, and anti-Smith antibody can independently predict bacterial infections in SLE-DAH. ⢠We put forward a comprehensive management algorithm based on infection stratification for SLE-DAH.
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Enfermedades Pulmonares , Lupus Eritematoso Sistémico , Humanos , Estudios Retrospectivos , Hematuria , Alveolos Pulmonares , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/tratamiento farmacológico , Lupus Eritematoso Sistémico/diagnóstico , Hemorragia/etiología , Hemorragia/diagnóstico , Enfermedades Pulmonares/etiología , Enfermedades Pulmonares/diagnóstico , HemoglobinasRESUMEN
BACKGROUND: To estimate the annual direct costs and cost-drivers associated with systemic lupus erythematosus (SLE) patients in China. METHODS: A multi-center, cross-sectional study was conducted based on the CSTAR registry. The information on demography and expenditures for outpatient and inpatient visits due to SLE were collected using online questionnaires. These patients' medical records were from the database of the Chinese Rheumatology Information System (CRIS). The average direct costs and 95% confidence interval were estimated using the bootstrap method with 1000 bootstrap samples by resampling with replacement. The cost-drivers were identified using multivariate regression models. RESULTS: A total of 1778 SLE patients from 101 hospitals participated in our study, with 92.58% as females, a mean age of 33.8 years old, a median duration of SLE of 4.9 years, 63.8% in an active disease state, 77.3% with two organs or more damaged, and 8.3% using biologics as treatment. The average annual direct cost per patient was estimated at CNY 29,727, which approximates to 86% for direct medical costs. For moderate to severe disease activities, the use of biologics, hospitalization, treatment of moderate or high dose glucocorticoids, and peripheral vascular, cardiovascular, and/or renal system involvements were found to substantially increase the direct costs, while health insurance slightly decreased the direct costs of SLE. CONCLUSIONS: This study provided reliable insight into financial pressures on individual SLE patients in China. The efforts focusing on preventing flare occurrences and limiting disease progression were recommended to further reduce the direct cost of SLE.
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Productos Biológicos , Lupus Eritematoso Sistémico , Femenino , Humanos , Adulto , Estudios Transversales , Lupus Eritematoso Sistémico/terapia , Gastos en Salud , Sistema de Registros , Costos de la Atención en Salud , Estudios RetrospectivosRESUMEN
OBJECTIVES: Catastrophic antiphospholipid syndrome (CAPS) is a life-threatening form of antiphospholipid syndrome (APS) with high mortality. We try to develop a predictive model to achieve early recognition of CAPS. METHODS: Data of APS patients referred into Peking Union Medical College Hospital from May 2013 to October 2021 was collected. A binary logistic regression method was used to identify predictors of CAPS, coefficient B was assigned with score value in the development of prediction model, and risk-stratification was based on the calculated scores using the model. RESULTS: Twenty-seven CAPS (11.9%) occurred in 226 APS patients. CAPS was more likely to occur in male secondary APS patients with a history of hypertension, hyperlipidaemia, and arterial thrombosis, presented with haematological, nephrological and immunological abnormalities simultaneously. Hypertension history (OR 5.091, 95% CI 1.119-23.147), anaemia (OR 116.231, 95% CI 10.512-1285.142), elevated LDH (OR 59.743, 95% CI 7.439-479.815) and proteinuria (OR 11.265, 95% CI 2.118-59.930) were independent predictors for CAPS, and the scores were 1, 3, 3 and 2 points, respectively. The risk scores were divided into high-risk (6-9) and low risk (0-5), the risk for CAPS were 54.1% and 0.6%, with sensitivity of 0.963 and specificity of 0.886. The Nagelkerke's R2 (0.739) and the Omnibus test (χ2 =109.231, df=4, p=0.000) indicated the model has a good fit. The AUC of 0.971 indicated good discrimination. The calibration curve in internal validation showed good calibration of this predictive model. CONCLUSIONS: A predictive model of CAPS was developed with hypertension, anaemia, elevated LDH and proteinuria. This model could help identify CAPS in high-risk patients, achieve early recognition and intervention to improve prognosis.
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Síndrome Antifosfolípido , Hipertensión , Humanos , Masculino , Síndrome Antifosfolípido/complicaciones , Síndrome Antifosfolípido/diagnóstico , Síndrome Antifosfolípido/epidemiología , Estudios de Cohortes , Pueblos del Este de Asia , Factores de Riesgo , Proteinuria/etiología , Proteinuria/complicacionesRESUMEN
Objective: To investigate the efficacy of conventional rehabilitation alone and conventional rehabilitation combined with aerobic training on muscle strength and function, health condition, and quality of life for patients with stable idiopathic inflammatory myopathy (IIM). Methods: This is a historical retrospective cohort study, in which the medical records of patients with IIM, who received the combination of conventional rehabilitative therapy and aerobic training (combined training group [CTG]), from February 2015 to December 2017 were reviewed. Patients with IIM who received conventional therapy alone were matched based on their age, gender, and disease activity as the control group (CG). Scores obtained on manual muscle testing of eight designated muscles (MMT8) was the primary outcome measure, and scores on the myositis Functional Index-2 (FI-2), Health Assessment Questionnaire (HAQ), and 36-item Short Form Medical Outcomes Study Questionnaire (SF-36) at 12 weeks during training were the secondary outcomes. Results: Fifty-six patients (28 in the CTG and 28 in the CG) were included in this analysis. Patients in both groups had improved MMT8, FI-2, HAQ, and SF-36 scores after 12 weeks of physical therapy. The CTG had a significantly higher score on the MMT8 and HAQ than the CG in the 12th week. The FI-2 scores were significantly higher in the CTG for the four items (P < 0.05) of hip flexion, step test, heel lift, and toe lift. SF-36 scores of the CTG were also higher than those of the CG for the five items (P < 0.05) of physical functioning, general health, vitality, social functioning, and mental health. Conclusions: Physical exercise training including conventional rehabilitation and aerobic training improved muscle function, health condition, and quality of life. Conventional rehabilitative training combined with aerobic training achieved better improvement compared with conventional rehabilitation training alone.
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Objectives: Studies concerning myocardial involvement (MI) in patients with anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis/clinically amyopathic dermatomyositis (anti-MDA5 Ab+ DM/CADM) are scarce. We aimed to characterize MI in our anti-MDA5 Ab+ DM/CADM cohort and to investigate its association with prognosis. Methods: In this single-center retrospective study, anti-MDA5 Ab+ hospitalized DM/CADM patients who underwent transthoracic echocardiography (TTE) were enrolled. Myocardial involvement was diagnosed according to abnormal cardiac structure and function detected by TEE. Clinical features and cardiac examination findings of patients with MI were analyzed. Clinical features, laboratory findings, complications, and treatments were compared between MI and non-MI, deceased, and survival patients. Logistic regression analysis was used to explore the independent risk factors for the occurrence of MI and prognostic factors for these patients. Results: Seventy-six hospitalized patients with anti-MDA5 Ab+ DM/CADM were enrolled. Twelve (15.8%) patients were diagnosed with MI. Of the 12 patients, three underwent cardiac magnetic resonance imaging (CMR) and late gadolinium enhancement (LGE) were noted for them. TEE revealed that eight (66.7%) patients had left atrial and/or ventricular enlargement, three (25.0%) had cardiac hypertrophy, six (50.0%) had diffuse ventricular wall dyskinesia, and seven (58.3%) had diastolic dysfunction. Six (50.0%) patients with MI developed heart failure (HF) during treatment. Of the 12 patients, one patient died of HF caused by myocarditis, three died of infection, and four died of exacerbation of rapidly progressive interstitial lung disease (RP-ILD). Logistic regression analysis revealed that dysphagia (OR 3.923, 95% CI 1.085, 14.181), NT-proBNP >600 pg/ml (OR 18.333, 95% CI 1.508, 222.875), and increased peripheral white blood cells (OR 1.201, 95% CI 1.003, 1.438) were risk factors for the occurrence of MI, but plasma albumin (OR 0.892, 95% CI 0.796, 0.999) was a protective factor. Both MI (OR 5.984, 95% CI 1.174, 30.496) and RP-ILD (OR 11.875, 95% CI 2.796, 50.411) were independent risk factors for the mortality of these anti-MDA5 Ab+ DM/CADM patients. Conclusion: Myocardial involvement is not rare and is an independent poor prognostic factor of anti-MDA5 Ab+ DM/CADM patients. Cardiac abnormality screening is necessary for them.
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Dermatomiositis , Enfermedades Pulmonares Intersticiales , Autoanticuerpos , Medios de Contraste , Dermatomiositis/diagnóstico , Progresión de la Enfermedad , Gadolinio/uso terapéutico , Humanos , Helicasa Inducida por Interferón IFIH1 , Enfermedades Pulmonares Intersticiales/diagnóstico , Estudios RetrospectivosRESUMEN
BACKGROUND: Antiphospholipid syndrome (APS) is an autoimmune disease characterized by persistent antiphospholipid antibodies (aPLs) positivity with a wide manifestation spectrum. A risk stratification is needed for management guidance and prognosis assessment. We aimed to identify phenotypes among aPL-positive patients and assess the prognosis of each phenotype. METHODS: This was a single-center, prospective cohort study of aPL-positive patients presented to Peking Union Medical College Hospital from 2012 to 2020. Demographic characteristics, aPL-related manifestations, cardiovascular risk factors, and antibodies profiles were recorded. The primary endpoint was defined as a combination of newly onset thrombosis, major bleeding events, non-criteria manifestations, and all-cause death. Hierarchical cluster analysis and Kaplan-Meier survival analysis were performed. RESULTS: Four clusters among 383 patients (70.2% female; mean age 37.7 years) were identified. Cluster 1 (n = 138): patients with systemic lupus erythematosus (SLE) and non-criteria manifestations; cluster 2 (n = 112): patients with multiple cardiovascular risk factors; cluster 3 (n = 83): female patients with obstetric morbidity; cluster 4 (n = 50): patients with isolated lupus anticoagulant (LA) positivity. Non-criteria manifestations were found aggregated with SLE from cluster analysis of variables. Cluster 3 showed the best outcome, while cluster 2 suffered highest frenquency of newly onset arterial thrombosis. CONCLUSIONS: We identified 4 clinical phenotypes of aPL-positive patients. Non-criteria manifestations may indicate underlying SLE, for which immunosuppressive therapy besides anticoagulation may be necessary. Patients with isolated LA positivity suffered similar risks with secondary APS and patients with multiple cardiovascular risk factors. Attention should be paid to male patients, and the screening of cardiovascular risk factors should never be ignored.
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Síndrome Antifosfolípido , Lupus Eritematoso Sistémico , Trombosis , Anticuerpos Antifosfolípidos/análisis , Síndrome Antifosfolípido/complicaciones , Síndrome Antifosfolípido/diagnóstico , Síndrome Antifosfolípido/epidemiología , Estudios de Cohortes , Femenino , Humanos , Inhibidor de Coagulación del Lupus , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/tratamiento farmacológico , Masculino , Embarazo , Pronóstico , Estudios ProspectivosRESUMEN
OBJECTIVE: The effectiveness and safety of sirolimus for SLE treatment have been shown in some uncontrolled studies. However, a comparison of sirolimus with other classic immunosuppressants has not been reported. We conducted the study to compare the effectiveness and safety of sirolimus versus tacrolimus for SLE treatment. METHODS: A real-world cohort study was conducted. Patients with clinically active SLE who were prescribed sirolimus or tacrolimus were enrolled. Propensity score matching was used to ensure equivalent disease conditions and background medications. SLE disease activity indices, serological parameters, steroid doses, modification of other immunosuppressants, renal effectiveness and adverse events were compared between the two groups at 3-month, 6-month, 9-month and 12-month follow-up visits. RESULTS: Data from 52 patients in each of the sirolimus and tacrolimus groups were analysed. Indices regarding the effectiveness of sirolimus, including Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) scores, physician's global assessment (PhGA) scores, and proportion of patients with SLEDAI-2K reduction of ≥4 and PhGA increase of <0.3, were equivalent to those of tacrolimus at all follow-up timepoints (all p≥0.05). Greater improvements in complement levels were observed in the sirolimus group at 3 and 6 months. Higher percentages of patients with prednisone doses ≤7.5 mg/day were observed in the sirolimus group at all timepoints. Seventeen adverse events in the sirolimus group were recorded. None was severe or led to drug discontinuation. CONCLUSIONS: Overall, sirolimus was as effective as tacrolimus in the treatment of SLE. Sirolimus had better effects on serological improvement and glucocorticoid tapering. Sirolimus was well tolerated in patients with SLE.