Classification Criteria Research Articles

Imaging tests as predictors of progression to rheumatoid arthritis in clinically suspect arthralgia: A systematic review & meta-analysis.

To determine and compare the diagnostic accuracy of imaging tests for the prediction of RA progression in people with inflammatory joint pain or CSA. We searched MEDLINE, Embase and Web of Science from 1987 to March 2024. Studies evaluating any imaging tests in participants with inflammatory joint pain or CSA, without clinical synovitis were eligible. Reference standards included RA classification criteria, methotrexate initiation or development of inflammatory arthritis (IA). Two authors independently extracted data and assessed validity according to QUADAS-2. We estimated summary sensitivities and specificities for each imaging characteristic and fitted bivariate and hierarchical SROC models for meta-analysis where possible. We found 39 eligible studies including 42 cohorts, of which 12 evaluated MRI (n = 2,782; 19% with RA/IA), 26 evaluated ultrasound (US)(n = 6,805; 25% with RA/IA) and 10 evaluated other imaging tests (n = 3,362; 20% with RA/IA). Summary sensitivity and specificity for US Power Doppler ≥1 in at least one joint were 37% (95%CI 18%-60%) and 90% (95%CI 82%-94%), respectively (7 studies). Summary sensitivity and specificity for MRI synovitis in at least one joint were 45% (95%CI 29%-62%) and 84% (95%CI 66%-94%), respectively (4 studies). Lack of consensus regarding positive threshold definitions limited meta-analysis for other imaging features. Evidence for MRI or US in predicting RA/IA in people with CSA is heterogeneous and of variable quality. Further studies with larger sample sizes, longer follow-up times and uniform imaging test scoring, are warranted to determine whether imaging characteristics, in combination with clinical information, can predict RA in this population. PROSPERO: https://www.crd.york.ac.uk/prospero CRD42024501243.

Determination of highly hazardous pesticides in fruits and vegetables in the Maya region of southeast of Mexico.

Highly Hazardous Pesticides (HHPs) have been identified as substances with severe adverse effects, including carcinogenicity, endocrine disruption, and reproductive toxicity. The aim of this study was to evaluate pesticide residues in fruits and vegetables from Hopelchén to provide evidence for policy recommendations on pesticide regulation. A total of 25 samples were collected and analyzed using the QuEChERS method with GC-MS/MS and LC-MS techniques. Of the 156 pesticides screened, 25 were detected, with tebuconazole, chlorantraniliprole, imidacloprid and carbendazim among the most frequent. Approximately 20% of the pesticides identified qualified as HHPs based on WHO/FAO criteria, while 60% were categorized as HHPs according to the more comprehensive criteria outlined by Pesticide Action Network International. Many of these pesticides exhibited toxicity to bees and high environmental persistence. Furthermore, 33% of the samples exceeded the European Union's Maximum Residue Limits, particularly for pesticides in papayas and bell peppers. Our findings show the presence of HHPs in the region, which represent critical hazards to ecosystem, pollinator populations, and public health. This work may contribute to the development of specific HHPs classification criteria for Mexico, thus advancing the transition toward safer, with special emphasis on vulnerable regions such as the Mayan zone in southeastern Mexico.

Initial Efficacy After Lateral Lumbar Interbody Fusion in Degenerative Lumbar Spinal Stenosis Classified as Schizas Grade A to D.

This study was to evaluate the initial efficacy of lateral lumbar interbody fusion (LLIF) in treating degenerative lumbar spinal stenosis (DLSS), encompassing a spectrum of stenosis severity from minor to extreme cases. This retrospective study analyzed the outcomes of 43 patients with DLSS, involving 55 surgical segments, who underwent LLIF. Clinical efficacy and radiographic parameters were recorded during the perioperative period and at the final follow-up. Pursuant to the Schizas' classification criteria, the 55 surgical segments were categorized into 2 groups: the Schizas grade A or B group and the C or D group. Simultaneously, the 43 patients were divided into 2 groups: the minor-moderate stenosis (MM) group and the severe-extreme stenosis (SE) group. Comparative analyses were subsequently conducted. All patients successfully underwent the LLIF procedure, without the need for additional posterior decompression surgery. Both 16MM and 27 SE group patients demonstrated significant improvements in clinical efficacy scores compared to their preoperative baselines (P < 0.001). Furthermore, there were no statistically significant differences between the MM group and the SE group in postoperative clinical metrics (P > 0.05). Both 23 Schizas grade A or B and 32 C or D group segments exhibited significant postoperative improvements in radiographic parameter values (P < 0.001), with no statistically significant differences between the 2 groups (P > 0.05). Under certain indications, patients with varying degrees of DLSS, ranging from mild to extreme, can achieve favorable outcomes through LLIF, and preoperative imaging revealing severe or extreme stenosis does not automatically disqualify patients from undergoing indirect decompression surgery.

Systemic disease activity measured with ESSDAI varies largely over 5 years in a prospective, longitudinal cohort of patients with Sjögren’s disease

ObjectivesThe objectives are to evaluate variation in systemic disease activity (European Alliance of Associations For Rheumatology (EULAR) Sjögren’s Syndrome Disease Activity Index (ESSDAI)) over time at group and individual patient...

Secondary cryofibrinogenemia is related to more severe microangiopathic involvement in systemic sclerosis: results from a retrospective observational study.

The aims of this study were to investigate the prevalence of cryofibrinogenemia in a cohort of patients with systemic sclerosis (SSc) regardless of clinical manifestations, who were admitted to our hospital and determine the associations among CF positivity, disease features and ongoing therapies. This was a monocentric and retrospective study. The inclusion criteria were a diagnosis of SSc (according to the ACR/EULAR 2013 classification criteria), regular administration of i.v. prostanoids, and CF testing between February 2020 and February 2022. Data on demographic, clinical, and immunological features and ongoing treatments were retrospectively collected. Categorical data were compared with the chi-square test or Fisher's exact test, while quantitative variables comparisons were carried out with Student's t test or Mann‒Whitney test. In total, 101 SSc patients were ultimately enrolled. The majority of patients were female (92.1%) and had the limited cutaneous form of SSc (81.2%). CF positivity was observed in 69.3% of the patients, whereas only 9% presented cryoglobulins and CF. CF positivity was negatively associated to RNAP3 antibodies (p = 0.027). No direct associations with specific clinical phenotypes were observed. No associations with immunosuppressive treatments were identified, however a positive association with nifedipine administration (p = 0.040), and a negative association with endothelin receptor antagonists (ERAs) plus phosphodiesterase-5 (PDE5) inhibitors regimen (p = 0.031) were observed. Macitentan administration was also associated to CF cryocrit ≥ 1% (p = 0.045). Among patients who were not treated with ERAs, an estimated pulmonary artery systolic pressure ≥ 30mmHg was significantly associated with CF positivity (p = 0.025). Moreover, a cryocrit ≥3% was associated with a relative risk of 3.44 (95% CI 1.26-9.39, p = 0.016) for digital amputation and 5.17 (95% CI 1.18-22.6, p = 0.029) for death. Isolated CF is a frequent phenomenon observed in SSc patients and is associated with a higher administration of vasoactive drugs, probably identifying a SSc clinical phenotype with a more severe microvascular involvement. Moreover, a higher cryocrit is associated with an increased risk of death and digital amputations. Screening SSc patients for CF would represent an opportunity to provide better therapeutic approaches by anticipating ERA administration in an earlier phase, thereby preventing the manifestation of severe microvascular involvement. Key Points • Cryofibrinogen is a cryoprotein that can cause microangiopathic damage. • Isolated cryofibrinogenemia is common in patients with systemic sclerosis. • SSc patients should be tested for cryofibrinogen because a high cryocrit (≥ 3%) is associated with death and/or digital amputation due to necrosis. • Cryofibrinogen is associated with indirect markers of pulmonary arterial hypertension in patients not treated with endothelin receptor agonists (ERAs). • ERAs could play a role in preventing cryofibrinogen damage.

Predictive Value of the DETECT Algorithm for Pulmonary Arterial Hypertension in Systemic Sclerosis: Findings from an Italian Observational Study.

Background/Objectives: Pulmonary arterial hypertension (PAH) is a complication of systemic sclerosis (SSc), and several screening algorithms have been proposed for the early detection of PAH in SSc. This study aimed to evaluate the predicting values of the DETECT algorithm for SSc-PAH screening in patients with SSc undergoing right heart catheterization (RHC) based on 2015 ESC/ERS echocardiographic criteria in a real-life setting. Methods: Patients fulfilling the 2013 ACR/EULAR classification criteria for SSc and with available data for PAH screening with the DETECT algorithm and the 2015 ESC/ERS echocardiographic criteria were retrospectively enrolled from January to June 2017 and then followed for 5 years. Baseline and annual clinical, laboratory, and instrumental data were collected. Results: A total of 33 out of 131 (25%) patients were selected based upon the ESC/ERS echocardiographic criteria, but 30 (23%) underwent RHC, while 51 (39%) patients with SSc were positive based on the DETECT algorithm. PAH diagnosis was confirmed in 28/30 cases (93.3%). The DETECT algorithm showed lower specificity and positive predictive value (PPV) (p < 0.0001) but higher sensitivity and negative predictive value (NPV) (p < 0.0001) than ESC/ERS criteria. Notably, patients with SSc with a negative DETECT screening at baseline had a low probability of developing PAH during a 5-year follow-up (OR 0.15, 95% CI 0.10-0.60-p < 0.0001). Conclusions: The DETECT algorithm has proven to be an easy, fast, and inexpensive tool for screening PAH in patients with SSc. Overall, a low probability of PAH using DETECT is highly predictive of a good prognosis.

Factors associated with renal involvement in adult immunoglobulin a vasculitis: what is it?

ABSTRACT Objective The objective of this study is to examine the potential of specific parameters in determining renal involvement in adult patients diagnosed with Immunoglobulin A vasculitis (IgAV). Methods The patients’ records with IgAV meeting the EULAR/PRINTO/PRES classification criteria who were diagnosed between January 2017 and January 2022 were retrospectively reviewed. The Birmingham Vasculitis Activity Score (BVAS) version 3 was used to assess initial disease activity. A comparison was conducted between the data of patients with and without renal involvement. To investigate factors associated with renal involvement, the significant parameters were used to develop a forward stepwise multivariate regression model. Receiver Operating Characteristic (ROC) curve analysis was used to evaluate the predictive ability of significant parameters for renal involvement. Results The study included 85 patients, of whom 22 (25.9%) had renal involvement. Patients with renal involvement had a median age of 60.5 years and a median follow-up of 56.3 months. The median BVAS and ferritin/albumin ratio (FAR) values were significantly higher in patients with renal involvement (p < 0.001). Multivariate regression analysis identified initial BVAS and FAR values as factors associated with renal involvement. ROC analysis demonstrated that the initial BVAS and FAR values were associated with renal involvement, with AUCs of 0.882 and 0.817, respectively (p < 0.001). Conclusions The initial BVAS above 7.5, and baseline FAR above 24.97 are factors associated with renal involvement. The results of this study may provide the basis for identifying individuals at high risk for renal involvement and, consequently, for more rigorous monitoring.

Clinical and laboratory characteristics of Sjögren's syndrome-associated autoimmune liver disease: a real-world, 10-year retrospective study.

To investigate the clinical and laboratory features of Sjögren's syndrome-associated autoimmune liver disease (SS-ALD) patients and identify potential risk and prognostic factors. SS patients with or without ALD, who visited Tongji Hospital between the years 2011 and 2021 and met the 2012 American College of Rheumatology (ACR) classification criteria for Sjögren's syndrome, were retrospectively enrolled. The clinical and laboratory data of the enrolled patients, including autoimmune antibodies, were collected and analyzed with principal component analysis, correlation analysis, LASSO regression, and Cox regression. A total of 117 SS-ALD patients were confirmed out of 568 SS patients. Compared to SS-non-ALD patients (n = 451), SS-ALD patients exhibited more severe involvement of the hepatic and hematologic systems, albeit with less pronounced typical SS symptoms. Disease activity was higher in SS-ALD patients, as indicated by elevated ESR, CRP, and IL-6 levels, particularly in the SS-overlap subgroup. Furthermore, SS-AIH patients without AIH-specific autoantibody testing or with negative testing results had higher AST and ALT levels than those who were autoantibody-positive. Our predictive model, incorporating IgG, IgM, AST, GGT, ALT, and C4, effectively identified ALD complications in SS patients, achieving an AUC of 0.924. Additionally, a grimmer prognosis was associated with higher baseline AST and ALT levels. SS-ALD patients often manifest with an insidious onset and atypical SS symptoms, yet frequently exhibit severe systemic involvement, intense inflammatory and immune responses, and a poor prognosis. To improve the clinical outcomes in SS-ALD patients, regular monitoring, early identification, and active treatment should be applied. Key Points • The study provided a detailed profile of clinical and laboratory features of SS-ALD and SS-non-ALD patients, contributing to a predictive model of ALD complications in SS patients • SS-ALD patients manifested with an insidious onset but exhibited severe systemic involvement, robust inflammatory and immune responses, and poor prognosis • SS-AIH patients without available testing for AIH-specific autoantibodies or with negative results demonstrated worse liver function, thus routine screening for autoimmune liver antibodies is recommended in SS patients • More severe baseline liver function status was associated with poorer therapeutic responses to routine medications, so early detection and timely intervention are essential for SS patients.

Cerebrovascular risk in rheumatoid arthritis patients: insights from carotid artery atherosclerosis in the Paracelsus 10,000 study

Rheumatoid arthritis (RA) is a chronic autoimmune disease marked by systemic inflammation. While RA primarily affects the joints, its systemic effects may lead to an increased cerebro- and cardiovascular risk. Atherosclerosis of the carotid arteries is a significant risk factor for cerebrovascular events and serves as a surrogate marker for cardiovascular risk. This study explores the link between RA and carotid artery atherosclerosis with data from the Paracelsus 10,000 Study. Baseline assessments were conducted on individuals randomly selected from Salzburg and its surrounding regions. Participants diagnosed with RA based on ACR-EULAR classification criteria and who underwent carotid artery ultrasound were included. Data were gathered from a total of 9729 participants, among whom 299 were diagnosed with RA. Carotid arteries were examined using ultrasound imaging. The primary endpoint was the difference in the prevalence of plaque presence between the RA and non-RA groups. One univariate (Model I) and three multivariate analyses were conducted, with adjustments in Model II incorporating SCORE 2, while Model III accounted for metabolic syndrome, age and sex. Additionally, Model IV included further adjustments for high-sensitivity C-reactive protein (hs-CRP). Plaque presence was defined as the ultrasound detection of plaque formation larger than 0 mm2, regardless of whether it was unilateral or bilateral. Additional assessments included carotid stenosis, intima-media thickness (IMT) and total plaque area (TPA). RA patients had a higher prevalence of plaque (50%) compared to non-RA individuals (38%). The odds ratio (OR) for plaque presence in RA patients versus non-RA individuals was 1.64 (95% CI 1.30–2.06). This association persisted after adjusting for SCORE2, with an adjusted odds ratio (aOR) of 1.65 (95% CI 1.26–2.15). The association remained significant when adjusting for metabolic syndrome, age and sex (aOR = 1.32, 95% CI 1.02–1.72) and also in Model IV, which included further adjustment for hs-CRP (OR = 1.33, 95% CI 1.02–1.74). The findings underscore an increased risk of cerebrovascular disease associated with RA. This study highlights the importance of thorough cerebrovascular and cardiovascular risk assessments, along with proactive management, for RA patients to reduce this risk. Recognizing the substantial impact of RA on stroke and cerebrovascular disease is important for enhancing patient care strategies. Carotid ultrasound appears to be an effective method for atherosclerosis screening in RA patients.

Unbiased identification of cell identity in dense mixed neural cultures.

Induced pluripotent stem cell (iPSC) technology is revolutionizing cell biology. However, the variability between individual iPSC lines and the lack of efficient technology to comprehensively characterize iPSC-derived cell types hinder its adoption in routine preclinical screening settings. To facilitate the validation of iPSC-derived cell culture composition, we have implemented an imaging assay based on cell painting and convolutional neural networks to recognize cell types in dense and mixed cultures with high fidelity. We have benchmarked our approach using pure and mixed cultures of neuroblastoma and astrocytoma cell lines and attained a classification accuracy above 96%. Through iterative data erosion, we found that inputs containing the nuclear region of interest and its close environment, allow achieving equally high classification accuracy as inputs containing the whole cell for semi-confluent cultures and preserved prediction accuracy even in very dense cultures. We then applied this regionally restricted cell profiling approach to evaluate the differentiation status of iPSC-derived neural cultures, by determining the ratio of postmitotic neurons and neural progenitors. We found that the cell-based prediction significantly outperformed an approach in which the population-level time in culture was used as a classification criterion (96% vs 86%, respectively). In mixed iPSC-derived neuronal cultures, microglia could be unequivocally discriminated from neurons, regardless of their reactivity state, and a tiered strategy allowed for further distinguishing activated from non-activated cell states, albeit with lower accuracy. Thus, morphological single-cell profiling provides a means to quantify cell composition in complex mixed neural cultures and holds promise for use in the quality control of iPSC-derived cell culture models.

Causes of Sleep Disturbance in Early ASAS Spondyloarthritis: A Retrospective Long-Term Experience.

Introduction: Sleep disturbance (SD) in the second half of the night due to inflammatory pain was included in the 2009 ASAS classification criteria of Spondyloarthritis (SpA), even though its definition is uncertain. Aim: We aimed to investigate SD in early-SpA (e-SpA) patients at T1 (2010-2013), comparing them to long-term SpA (l-SpA) patients at T2 (2023-2024) after at least 10 years of follow-up. Methods: At T1, in e-SpA and l-SpA cases, SD, classified as "difficulty in initiating sleep" (DIS), "difficulty in maintaining sleep" (DMS) and "early awakening" (EA), was compared to clinical parameters (ASDAS-CRP, BASDAI, m-HAQ-S, BASMI, MASES, 68/66 joint count, tenderness of sacroiliac joints, fatigue [FACIT] and HADS for anxiety [A] and depression [D]). At T2, e-SpA patients were re-evaluated using the Pittsburgh Sleep Quality Index (PSQI). Results: At T1, 45% of 166 SpA patients had SD; in e-SpA patients (60), SD correlated with sacroiliac pain (DMS) BASDAI, FACIT and HADS-D (EA); in l-SpA patients (106), it correlated with HADS-A (DIS), BASDAI and FACIT (DMS). At T2, e-SpA patients showed a high PSQI in 51.5% of cases, correlated with T2-ASDAS-CRP and T2-BASDAI. Moreover, T1-ASDAS-CRP was predictive of T2-PSQI. Conclusions: SD is more specific for inflammatory pain in e-SpA and might be influenced by disease activity also in long-term disease.

Systemic Inflammatory Immune Index (SII) Predicts Disease Activity in Systemic Lupus Erythematosus (SLE) Patients: A Cross-Sectional Study

Background: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by widespread inflammation and diverse clinical manifestations. The systemic inflammatory immune index (SII), calculated as platelet count * neutrophil count/lymphocyte count, has emerged as a potential marker of systemic inflammation in various conditions. This study aimed to investigate the relationship between SII and disease activity in SLE patients. Methods: We conducted a cross-sectional study involving 60 SLE patients diagnosed according to the 2019 EULAR/ACR classification criteria. Demographic and clinical data were collected, and disease activity was assessed using the Mexican Systemic Lupus Erythematosus Disease Activity Index (MEX-SLEDAI). Blood samples were analyzed to determine SII values. Statistical analysis included Spearman's correlation to assess the relationship between SII and MEX-SLEDAI scores. Results: The study population predominantly consisted of women (98.3%), with a median age of 29 years. A strong positive correlation was observed between SII and MEX-SLEDAI scores (r = 0.931, p &lt; 0.001). Patients with higher SII values exhibited significantly greater disease activity. Conclusion: Our findings suggest that SII is a promising predictor of disease activity in SLE patients. This readily available index may aid clinicians in assessing disease severity and tailoring treatment strategies. Further research is warranted to validate these findings and explore the utility of SII in monitoring disease progression and treatment response.

Revealing a novel GI-19 lineage infectious bronchitis virus sub-genotype with multiple recombinations in South Korea using whole-genome sequencing.

Infectious bronchitis (IB), caused by the infectious bronchitis virus (IBV), is a highly contagious chicken disease, causing economic losses worldwide. New IBV strains and variants continue to emerge despite using inactivated and live-attenuated vaccines to prevent or control IB. In this study, the S1 genes of 46 IBV strains, isolated from commercial chicken flocks between 2003 and 2024 in Korea were sequenced and genetically characterized. The IBV isolates belonged to Korean group II (K-II), which was included in the GI-19 lineage. The K-II was divided into five sub-genogroups (a-e) based on phylogenetic tree analysis results and nucleotide identification of the S1 gene. Of these, K-IId was the most common genotype in Korea; however, eight novel isolates belonging to the K-IIe sub-genotype were discovered. The nucleotide and amino acid identities of the other four K-II sub-genotypes and the eight isolates were 84.42-95.89% and 84.02-95.86%, respectively. The complete genomes of the eight K-IIe isolates were obtained using next-generation sequencing. Various recombination patterns were observed despite the high homology of the S1 gene among the eight IBV strains. Among the eight K-IIe isolates, six were recombinants, exhibiting recombinations between K-IIe and K-IIc, K-IIe and K-IIa, and with the live vaccine strain. Most recombination breakpoints were detected in the nsp2 region of the ORF1a, S2, and M genes. The present study proposed new classification criteria for the K-II belonged to the GI-19 lineage prevalent in South Korea and revealed the recombination patterns of recently identified novel isolates, providing important information on novel viral sub-genotype strains and IBV evolution.

Epidemiology of Antisynthetase Syndrome and Risk of Malignancy in a Population-based Cohort (1998-2019).

Population-based epidemiology studies about antisynthetase syndrome (ASSD) are lacking. Our aims were to determine the incidence and prevalence of ASSD and assess malignancy risk among patients following ASSD diagnosis. A retrospective, population-based cohort of adults with incident ASSD residing in Olmsted County, Minnesota, in 1998-2019 was assembled. Fulfillment of ASSD Solomon et al. classification criteria and clinical data were collected by manual chart review. Patients were followed until death, migration from the area, or December 31, 2019. Malignancy was defined by physician diagnosis in the medical record. Incidence rate was age- and sex-adjusted to the 2010 U.S. white population. Point prevalence rate was obtained on Jan 1, 2015. 13 patients with ASSD were identified (7 [54%] female, 13 [100%] Caucasian, median age 44.9 years [IQR: 41.9-58.3]). The age- and sex-adjusted incidence of ASSD was 0.56 (95% CI: 0.25-0.87) per 100,000 population. Incidence was highest in the 50-59 age group. Age- and sex-adjusted prevalence was 9.2 per 100,000 (95% CI: 3.4-15.0). 2 of 13 (15%) were diagnosed with malignancy within the follow-up interval; none within 3 years of ASSD diagnosis. At median 11.9 (IQR: 7.0-13.4) years of follow-up, 12/13 (92%) of patients were alive. Antisynthetase syndrome is rare, with incidence of 0.56 per 100,000 population and prevalence of 9 per 100,000. In this cohort, incidence was similar between males and females, and highest in persons ages 50-59 years. None of the patients developed malignancy within 3 years of ASSD diagnosis.

The Relationship Between Fibromyalgianess and Clinical Features, Disease Activity in Patients With Systemic Lupus Erythematosus.

Fibromyalgia (FM) is a chronic syndrome characterised by widespread pain, fatigue, and symptoms such as sleep disturbances, cognitive impairment, and mood disorders. FM prevalence is notably higher among systemic lupus erythematosus (SLE) patients compared with the general population, often leading to diagnostic challenges. Misinterpreting FM as SLE activity can result in overtreatment. This study aimed to evaluate fibromyalgianess and its relationship with the clinical and immunological characteristics of SLE patients using comprehensive scoring methods for better diagnostic accuracy. This cross-sectional study included 50 SLE patients meeting the 2019 EULAR/ACR classification criteria. Patients with coexisting autoimmune diseases or severe systemic conditions were excluded. Clinical data, SLEDAI scores, and fibromyalgianess severity were assessed using the Polysymptomatic Distress Scale (PSDS). Patients were categorised into groups based on fibromyalgia diagnostic criteria: widespread pain and SLE-FM. Statistical analysis was performed using SPSS, with p<0.05 considered significant. Among 50 patients (45 female, 5 male; mean age 42.04±12.5), 24% had fibromyalgianess, and 18% experienced widespread pain. Female patients exhibited significantly higher PSDS scores (p<0.05). While NSAID use was associated with increased PSDS scores (p<0.001), no significant relationship was found between fibromyalgianess and SLEDAI scores or organ involvement. Fibromyalgianess in SLE patients primarily reflects heightened pain sensitivity and symptom severity rather than disease activity. Incorporating fibromyalgianess assessment into routine SLE management may prevent diagnostic and therapeutic pitfalls and improve treatment outcomes. Multidisciplinary approaches, including pharmacological and non-pharmacological strategies, are essential for effective care.

Pulmonary Arterial Hypertension Incidence in Scleroderma Patients Treated with Bosentan for Digital Ulcers: Evidence from the Italian SPRING Registry.

Bosentan (BOS) is approved for treating pulmonary arterial hypertension (PAH) and preventing digital ulcers (DU) in systemic sclerosis (SSc). Our study aimed to evaluate whether BOS prescribed for DU could reduce the incidence of PAH in a large SSc cohort from the SPRING registry. Patients with SSc from the SPRING registry, meeting ACR/EULAR 2013 classification criteria with data on PAH onset, DU status, BOS exposure, and at least a one-year follow-up between 2015 and 2020, and no known PAH at baseline were included. PAH was diagnosed with right heart catheterization during the follow-up, and its incidence rate (IR) was calculated. Kaplan-Meier curves were determined, and multivariate regression identified PAH risk factors. Among 727 eligible patients with SSc, followed for a median of 2.0 years, 54 (7.4%) developed PAH [IR 3.71 per 100 patients/years]. Patients with DU who were never exposed to BOS had a higher incidence of PAH [IR 4.90 per 100 patients/years] compared to those exposed to BOS, whose rates matched those without DU and who were never exposed to BOS. Risk factors independently associated with PAH development included DU (HR 1.85), age (HR 1.05), modified Rodnan Skin Score (mRSS) >4 (HR 2.07), ILD (HR 2.29), and acetylsalicylic acid treatment (HR 1.78). In our cohort, DU were confirmed as a leading risk factor for PAH development, and BOS use for DU prevention may reduce this risk. Only patients with DU who were not on BOS had an increased PAH incidence.

Experimental investigation of the precursor characteristics and early warning of coal burst based on quantitative analysis of acoustic emission signals

Coal burst is one of the most frequent and destructive dynamic disasters encountered during underground mining engineering. However, the understanding of quantitative precursor characteristics of coal burst is still in its infancy, rendering it difficult to provide effective early warning of disaster. In this study, to quantitatively study precursor characteristics and warning signs of coal burst, the coal burst experiments were carried out on coal-rock combination with a crack. The acoustic emission (AE) technique was employed to quantitatively analyse the precursor information during coal burst process. Testing results indicated that coal burst process is classified into three stages based on evolution in AE energy, i.e., early incubation stage, late incubation stage and occurrence stage. The first significant increase in AE energy could be identified as the beginning of the late incubation stage of coal burst, accompanying by the phenomenon of macro-failure initiation. AE signals during the whole process could be classified as five types according to their dominant frequency and amplitude characteristics, i.e., HF-HA, LF-HA, EHF-LA, HF-LA and LF-LA respectively. The dramatic increase in number proportion of HF-HA and LF-HA signals is highly correlated with occurrence of coal burst. In addition, a comprehensive classification criterion for the coal burst prediction was proposed under a quantitative analysis of three AE parameters, i.e., first energy index (FEI), coal burst risk indicator based on AE energy (CRIE) and frequency spectrum (CRIF−A). The findings in this study could facilitate accurate coal burst prediction.

Are there atypical sites of IgG4 related disease in head and neck region? Personal experience and literature review.

Immunoglobulin G4-related disease (IgG4-RD) is a complex systemic fibroinflammatory condition with different clinical manifestations affecting multiple organ systems. Despite its rarity, the disease presents diagnostic and therapeutic challenges due to its mimicry of malignancies and other immune-mediated disorders. The 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria for IgG4-Related Disease is the current state of art to confirm the diagnosis of IgG4-RD even in the absence of histological analysis. However, this classification excludes atypical sites, focusing on the more typical ones, even in case of histological confirmation. In the ENT field, several localizations of this disease have been described. We report two clinical cases at the Otolaryngology Unit of IRCCS San Martino Hospital, Genoa affected by IgG4-RD arising in atypical sides of the head and neck region. Additionally, we perform a clinical review of the current literature. The review provides an extensive overview of IgG4-RD, encompassing epidemiology, clinical manifestations, diagnostic approaches, and therapeutic strategies. We discuss the evolution of diagnostic criteria, emphasizing the necessity of interdisciplinary collaboration among clinicians, radiologists, and pathologists for accurate diagnosis. Diagnostic imaging plays a crucial role, with characteristic radiological patterns aiding in the identification of affected organs. However, definitive diagnosis often requires histopathological confirmation, highlighting the importance of biopsy in challenging cases. We also focus on the treatment of IgG4-RD which poses significant challenges, with glucocorticoids remaining the cornerstone of therapy. Emerging steroid-sparing agents such as rituximab and Dupilumab, show promising results in refractory or recurrent disease. IgG4-RD is a multisystemic fibroinflammatory disease that can potentially affect any part of the body. The 2019 ACR/EULAR 3-stage classification criteria for IgG4-RD considers only a few head and neck sites. Therefore, it is of paramount importance that neurosurgeons, head and neck surgeons, and oral and maxillofacial pathologists are familiar with the clinicopathological manifestations of IgG4-RD in these sites to avoid misdiagnosis and inappropriate treatment, which can lead to a decrease in patients' quality of life. To our knowledge, there are no risk factors or genetic predispositions. Further studies are needed to elucidate the pathophysiology of IgG4-RD with the aim of providing a targeted therapy that could spare steroid-related effects and reduce relapses.

Anti-C1q antibodies in IgG4-related disease are common and associated with renal involvement and cutaneous small-vessel vasculitis.

To evaluate the prevalence and clinical associations of anti-C1q antibodies in IgG4-related disease (IgG4-RD), focusing on renal involvement and cutaneous small-vessel vasculitis (CSVV). We enrolled patients who met the revised 2020 Comprehensive Diagnostic Criteria and/or the 2019 ACR/EULAR Classification Criteria for IgG4-RD. Variables included demographics, organ involvement, clinical phenotypes, disease activity, serum biomarkers, follow-up duration, remission, and relapses. Anti-C1q antibodies were measured using a quantitative enzyme-linked immunosorbent assay (cut-off <10 U/ml). Seventy patients with a mean age of 52.1 years were included. 34 (48.6%) were male. Anti-C1q antibodies were positive in 74.3%, with a median level of 19.8 U/ml. Patients with active disease had higher anti-C1q antibody levels than inactive patients (p= 0.03). Renal involvement was more frequent in anti-C1q positive patients (p= 0.01). Six patients (8.6%) had CSVV, and all had positive anti-C1q levels. All exhibited palpable purpura and one patient had urticarial-like lesions. These patients had multi-organ involvement, and most had high IgG, IgG1, IgG4, and hypocomplementemia. Skin biopsies in three patients showed leukocytoclastic vasculitis with lymphocytic and eosinophilic infiltrates. Anti-C1q antibody levels correlated negatively with levels of C3 and C4, and positively with levels of IgG1, IgG4, and serum free light chains. Anti-C1q positivity did not predict relapse-free survival. This study is the first to evaluate anti-C1q antibodies in IgG4-RD, finding a high prevalence, particularly in patients with renal involvement and CSVV. The results support the hypothesis that immune complex-mediated complement activation contributes to IgG4-RD pathogenesis.

Validation of the 2022 ACR/EULAR classification criteria for giant cell arteritis in Koran patients with giant cell arteritis.

We applied the 2022 American College of Rheumatology (ACR)/European Alliance of Association for Rheumatology (EULAR) criteria to Korean patients previously diagnosed with giant cell arteritis (GCA) according to the 1990 ACR criteria and validated its clinical efficiency. Nine patients with GCA were included in this study. The proportion of patients meeting each item of the 1990 ACR criteria and the 2022 ACR/EULAR criteria were assessed. fluorodeoxyglucose-positron emission tomography (FDG-PET), and temporal artery biopsy were performed in eight, and four of the nine patients, respectively. The median age was 65.0 years, and 77.8% of the patients were women. Seven (77.8%) patients had polymyalgia rheumatica. All nine patients were reclassified as having GCA according to the 2022 ACR/EULAR criteria. Among the 10 items of the 2022 ACR/EULAR criteria, the item contributing the most to the reclassification was elevated acute-phase reactant levels (100%), followed by new temporal headache (77.8%), and FDG-PET activity throughout the aorta (77.5%). Positive temporal artery biopsy findings were observed in 75.0% of the patients; however, only four patients underwent biopsy, which was insufficient to assess its efficacy. An item of sudden visual loss did not make a considerable contribution to the reclassification of GCA because of its rare frequency (11.1%). In this study, for the first time, we demonstrated a concordance rate of 100% between the two criteria in Korean patients previously diagnosed with GCA. Moreover, we also clarified the major contributors to the reclassification according to the 2022 ACR/EULAR criteria.