The widespread adoption of computed tomography has increased the detection of lung nodules. However, deep learning methods for classification of benign and malignant nodules often fail to comprehensively integrate global and local features, and most of these methods have not been validated through clinical trials. Here we developed DeepFAN, a transformer-based model trained on more than 10,000 pathology-confirmed nodules, and conducted a multireader, multicase clinical trial (Chinese Clinical Trial Registry: ChiCTR2400084624) to evaluate its efficacy in assisting junior radiologists. DeepFAN achieved diagnostic area under the curve (AUC) values of 0.939 (95% CI 0.930-0.948) on an internal test set and 0.954 (95% CI 0.934-0.973) on a clinical trial dataset involving 400 cases across three independent medical institutions. Explainability analysis indicated higher contributions from global than local features. The average performance of 12 readers improved significantly: by 10.9% (95% CI 8.3-13.5%) for AUC, 10.0% (95% CI 8.9-11.1%) for accuracy, 7.6% (95% CI 6.1-9.2%) for sensitivity and 12.6% (95% CI 10.9-14.3%) for specificity (all P < 0.001). Nodule-level interreader diagnostic consistency improved from fair to moderate (overall κ: 0.313 versus 0.421; P = 0.019). These results indicate that DeepFAN can effectively assist junior radiologists and could help to homogenize diagnostic quality and reduce unnecessary follow-up of patients with indeterminate pulmonary nodules.

Immunotherapy shows promise for triple-negative breast cancer (TNBC), yet its effectiveness is restricted by low response rates, poor immune cell infiltration, and systemic side effects. Here, an ultrasound-responsive cerasomal nanoplatform integrating a STING agonist (SR-717@PC-iRGD) is developed for synergistic sonodynamic-immunotherapy. The nanocarrier is self-assembled from cerasome-forming lipids (CFL), porphyrin-conjugated lipids (PL), unsaturated phospholipids (DOPC), DSPC, and DSPE-PEG2000-iRGD, with SR-717 loaded in the lipid bilayer. The resulting assembly yields nanoparticles (NPs) with high SR-717 loading and exceptional stability. The siloxane shell (cerasome) confers high stability and prevents premature drug leakage, while iRGD promotes nanoparticle binding to tumor specific integrin to facilitate accumulation and retention in the tumor. Upon ultrasound irradiation, porphyrin generates reactive oxygen species (ROS) that oxidize the lipid bilayer and disrupt the cerasome, enabling on-demand SR-717 release at tumor site. The released SR-717 activates the STING pathway, driving type-I interferon production, dendritic cell maturation, and CD8+ T-cell infiltration. This strategy integrates sonodynamic therapy (SDT) with localized immune activation, addressing challenges of instability and inefficient delivery. The platform thus offers a precise and effective approach to stimulate antitumor immunity and enhance therapeutic outcomes for TNBC where no tumor targeted therapy is currently available.

To investigate the role of PANoptosis activation in anti-melanoma differentiation-associated protein 5 (MDA5) antibody-positive dermatomyositis (anti-MDA5+ DM). Transcriptomic profiling of peripheral blood mononuclear cells (PBMCs) from 40 patients with anti-MDA5+ DM and 10 healthy controls (HCs) was performed. The PANoptosis signature score was constructed using single sample gene set enrichment analysis based on the mean enrichment scores of the pyroptosis, apoptosis and necroptosis gene sets, and the clinical associations of the PANoptosis signature score in patients with anti-MDA5+ DM were evaluated. PANoptosis markers were analysed via western blotting, and the regulatory mechanism of PANoptosis activation was studied in Jurkat cells in vitro. Transcriptomic profiling demonstrated overactivation of the PANoptosis signalling pathway in patients with anti-MDA5+ DM. The PANoptosis signature score was positively correlated with inflammatory markers, type 1 interferon (IFN) signature score, disease severity and poor prognosis in patients with anti-MDA5+ DM. Z-DNA binding protein 1 ( ZBP1 ) was identified as a key PANoptosis-related gene in anti-MDA5+ DM. PANoptosis markers, including P-MLKL, GSDMD-N, cleaved caspase-8, cleaved caspase-3 and cleaved caspase-7, were significantly upregulated in T cells from patients with anti-MDA5+ DM. Functional assays demonstrated that IFN-β induced PANoptosis activation in T cells in vitro, which was significantly attenuated following ZBP1 knockdown. PANoptosis overactivation was associated with disease severity and prognosis in anti-MDA5+ DM. Our findings indicated that IFN-β triggered ZBP1-mediated PANoptosis in T cells, highlighting PANoptosis as a novel potential therapeutic target for anti-MDA5+ DM.

PurposeThis study aimed to examine the clinical features and prognosis of patients with distal limb weakness in anti-nuclear matrix protein 2 antibody (anti-NXP2) positive myositis.Patients and methodsThis retrospective study included the medical records of patients with anti-NXP2 antibody and follow-up data. Clinical features and prognosis of patients with and without distal limb weakness were compared. The differences in groups and survival analysis were analyzed using SPSS.ResultsOf 110 enrolled adult patients with anti-NXP2 positive myositis, 53 showed distal limb weakness. The frequency of distal limb weakness in patients with anti-NXP2 positive myositis was highest when compared with other dermatomyositis (DM) patients. In the patients with anti-NXP2 positive myositis, the mean onset age of distal limb weakness group was younger, when compared with those without distal limb weakness (38.40 ± 13.36 vs. 46.26 ± 15.02 years, p = 0.005). They exhibited higher disease activity and experienced higher frequencies of severe muscle weakness, subcutaneous edema, dysphagia, and abnormal ECG (all p < 0.05). In terms of treatment, 32.1% patients with distal limb weakness received glucocorticoid (GC) pulse, which was higher than those without distal limb weakness (7.0%, p = 0.001). There were 32 patients reporting relapse. The mean dose of GC (prednisone equivalent) at relapse was higher. Nine patients with distal limb weakness died during follow-up, with infection representing the leading cause of death. The survival rate was not different in patients with and without distal limb weakness.ConclusionAdult patients with anti-NXP2 positive myositis and distal limb weakness were younger at disease onset and exhibited increased disease activity, including a higher prevalence of multi-system involvement, as well as a higher incidence of relapse during follow-up.

ObjectiveBy quantifying airway parameters on computed tomography (CT) and incorporating clinical factors such as age and prematurity, this study developed an airway prediction model for infants to provide more accurate guidance for endotracheal tube size selection and insertion depth in patients under one year of age.MethodsA retrospective analysis of data on infants under one year of age who underwent preoperative anesthesia evaluation at Peking University People's Hospital from January 2020 to December 2022. Inclusion criteria: All enrolled infants underwent chest CT examination, with the scanning range covering the level of the cricoid cartilage. The development of cricoid cartilage was associated with laryngeal cartilage maturity. Its size and ossification degree change with age, sex, height and other factors. Therefore, we collected clinical data including the age, sex, gestational age, preterm birth, height and weight at birth and visit which may affect the shortest diameter of the cricoid cartilage in children. Infants with congenital laryngeal malformations, acquired nutritional deficiencies, hormonal abnormalities such as hypothyroidism, neck tumors and a history of maternal smoking during pregnancy were excluded. Accurately measure the shortest inner diameter of the cricoid cartilage and the distance from the cricoid cartilage to the carina on CT images. SPSS 24.0 software was used for simple and multiple linear regression analysis to screen key factors. Corresponding prediction models were established for the shortest cricoid cartilage diameter and the cricoid-to-carina distance, so as to facilitate the individualized selection of tracheal tube sizes and insertion depths in clinical practice.ResultsIn this study, age and preterm birth were identified as independent influencing factors for the shortest cricoid cartilage diameter in infants [P = 0.008, 0.005(95% CI: 0.001 to 0.008); P = 0.022, −1.261 (95% CI: −2.321 to −0.201)]. The established prediction formulas were as follows: the shortest diameter of the cricoid cartilage in full-term infants (mm) = 6.119 + 0.005 × age; The shortest diameter of the cricoid cartilage in preterm infants (mm) = 6.119 + 0.005 × age-1.261. For the distance from the cricoid cartilage to the carina, age was the only significant influencing factor [P = 0.003, 35.725(95% CI: 0.016 to 0.073)], and carina formula was derived as follows: distance from the cricoid cartilage to the carina (mm) = 35.725 + 0.044 × age.ConclusionAge and preterm status were independently associated with the shortest cricoid cartilage diameter in infants under one year of age, suggesting that preterm infants may require smaller endotracheal tubes than full-term infants of the same age. Age was also associated with the cricoid-to-carina distance. These CT-based findings may support more individualized airway management in infants. However, despite internal bootstrap validation, the model remains limited by the lack of external validation, small sample size, and highly specific ophthalmologic surgical population; therefore, broader validation is required before clinical generalization.

OBJECTIVE:To develop and validate a prognostic model for predicting all-cause mortality in patients with antisynthetase syndrome (ASyS). METHODS:This retrospective study included 1,194 patients with ASyS from three independent institutions in China. The Cox proportional hazards (CPH) method and random survival forest (RSF) algorithm were used for developing a mortality risk prediction model in the derivation cohort (n = 763). The optimal model was simplified into a scoring system and validated in an external cohort of 431 patients from two institutions. RESULTS:The model constructed using the CPH method in the training cohort incorporated five prognostic factors: age at onset, lactate dehydrogenase, albumin, respiratory failure, and neutrophil-to-lymphocyte ratio. This model demonstrated better performance than the model developed using the RSF algorithm in the internal validation cohort and was subsequently transformed into a simplified scoring system, termed the ALARN score. The ALARN model achieved time-dependent areas under the receiver operating characteristic curves of 0.914 (95% confidence interval [CI] 0.866-0.955), 0.867 (95% CI 0.829-0.906), 0.839 (95% CI 0.789-0.885), and 0.854 (95% CI 0.789-0.909) for predicting 1-, 3-, 5-, and 10-year mortality, respectively. Moreover, patients were effectively stratified into low-, intermediate-, and high-risk groups (ALARN scores of 0-2, 3-5, and 6-8, respectively) with significantly different survival outcomes (log-rank P < 0.0001). Internal and external validation confirmed the robustness of the ALARN score in predicting overall mortality in ASyS. CONCLUSION:The ALARN score incorporates five readily available clinical parameters and provides a simple, practical tool for predicting mortality risk in patients with ASyS.

Triple-negative breast cancer (TNBC) is an aggressive malignancy characterized by poor prognosis, limited treatment options, and resistance to conventional therapies. Sonodynamic therapy (SDT) has emerged as a promising non-invasive approach that leverages ultrasound to activate sonosensitizers and generate cytotoxic reactive oxygen species (ROS). However, the therapeutic efficacy of SDT is frequently compromised by the overactivation of antioxidant pathways, notably the Keap1-Nrf2-ARE axis. In this study, we developed a multifunctional and ultrastable cerasome-based nanoplatform (ML385@PC-iRGD) that co-delivers a porphyrinbased sonosensitizer and the Nrf2 inhibitor ML385, with surface functionalization by the tumor-penetrating peptide iRGD. The cerasomes, stabilized by a siloxane surface network, exhibited excellent stability and prolonged circulation time. In vitro and in vivo studies demonstrated that ML385@PC-iRGD efficiently accumulated in tumors, enhanced cellular uptake via iRGD-mediated targeting, and triggered robust ROS production under ultrasound irradiation. Importantly, the co-delivery of ML385 suppressed Nrf2-driven antioxidant defenses, leading to amplified oxidative stress. This synergistic "ROS burst + defense blockade" strategy effectively overcome the intrinsic resistance of TNBC to oxidative therapies. Overall, our study highlights the potential of cerasome-based nanocarriers as a powerful and stable delivery system for combinatorial SDT and molecular inhibition, offering a promising therapeutic avenue for the treatment of refractory breast cancers.

Dermatomyositis (DM) is a heterogeneous disease characterized by skin rash and muscle weakness. Although tongue disorders have been documented in idiopathic inflammatory myopathies (IIM), biopsy-confirmed tongue myositis remains exceedingly rare, and no reports have been published in patients with DM. This study describes two rare cases of anti-PM-Scl 75 antibody-positive dermatomyositis with tongue involvement and dysphagia. In case 1, muscle weakness and skin rash improved after the initial therapy. However, the patient developed tongue atrophy, severe pharyngeal dysphagia (Functional Oral Intake Scale (FOIS) level = 3) and dysarthria (Frenchay Dysarthria Assessment scale (FDA) score = 13) during the tapering of glucocorticoid. Escalating glucocorticoids and adding rituximab improved her swallowing (FOIS level = 6) and speech (FDA score = 23) function, though a limited improvement of tongue atrophy. Case 2 presented with severe skin rash, muscle weakness and elevated CK levels, accompanied by tongue redness and swelling, mild pharyngeal dysphagia (FOIS level = 5) and dysarthria (FDA score = 20) at disease onset. With the treatment of glucocorticoid, tacrolimus and intravenous immunoglobulin (IVIG), the patient achieved complete remission. Tongue myositis may occur in DM and could serve as a potential indicator of disease activity. Early recognition may be beneficial and biological agents warrant further investigation in refractory cases. Not applicable.

ObjectiveThis study aimed to describe the MRI features of lower limbs (thighs and calves) in patients with anti-NXP2 antibody positive myositis, and explore their relationship with clinical manifestations and prognosis.MethodsAdult patients with anti-NXP2 antibody who underwent both thigh and calf MRI examinations simultaneously were enrolled between 2017 and 2023. The MRI features and medical records of patients were reviewed. Statistical analysis was conducted by SPSS 21.0.ResultsA total of 48 patients (29 females and 19 males) were included in the study. There were fifteen and seven patients with subcutaneous edema on the thigh and calf MRI, respectively. The incidence of fascia edema in the thigh was higher than that in the calf (50.0% vs. 22.9%, p = 0.006). All patients experienced varying degrees of thigh muscle edema, with 41 cases (85.4%) showing calf muscle edema on MRI. The primarily affected group in the thigh was the anterior muscle, while the anterior and posterior groups of the calf were equally affected. In addition, the inflammation score of muscle MRI was positively correlated with disease activity (r = 0.316, p = 0.029), the level of muscle enzymes, serum ferritin (r = 0.439, p = 0.002), D-dimer (r = 0.410, p = 0.004), and NSE (r = 0.420, p = 0.006). Patients with diffuse pattern on MRI generally exhibited higher disease activity. However, there was no difference in the frequency of relapse and mortality between patients with and without diffuse lesions on MRI.ConclusionLower limb MRI of patients with anti-NXP2 antibody provided useful information in evaluating the extent and distribution of lesions. In addition, the degree of muscle edema on MRI was significantly correlated with various clinical features.

Interferons (IFN) are implicated in the pathogenesis of anti-MDA5 dermatomyositis (anti-MDA5-DM), but the presence of anti-IFN antibodies remains unclear. This study aims to assess serum levels of anti-IFN-α antibodies in anti-MDA5-DM patients and explore their clinical associations. Serum samples from 176 anti-MDA5-DM patients and 55 healthy controls were analyzed for anti-IFN-α antibody levels using an in-house ELISA assay, with immunoblot validation in a subset. Associations between anti-IFN-α antibodies and disease activity or prognosis were assessed. The prevalence of anti-IFN-α antibodies in anti-MDA5-DM patients was 17.6

OBJECTIVE:The coronavirus disease pandemic brought unknown challenges to patients with idiopathic inflammatory myopathy, who are often heavily immunosuppressed and have comorbidities. We aimed to investigate the outcomes and risk factors of coronavirus disease in Chinese patients with idiopathic inflammatory myopathy during the Omicron wave. METHODS:This observational study included patients with idiopathic inflammatory myopathy who visited the China-Japan Friendship Hospital. Data on baseline characteristics and coronavirus disease-related information were collected through medical records and surveys, and subsequently analysed. RESULTS:Overall, 204 patients with idiopathic inflammatory myopathy were identified; dermatomyositis was the most common idiopathic inflammatory myopathy subtype. Data were collected from 185 patients with idiopathic inflammatory myopathy who tested positive for severe acute respiratory syndrome coronavirus 2 via polymerase chain reaction or antigen tests; of these, 20 experienced a severe course of the disease, and 9 died. All patients with severe coronavirus disease had idiopathic inflammatory myopathy-associated interstitial lung disease, and the most common antibodies observed in patients with mortality were anti-aminoacyl tRNA synthetase and anti-MDA-5 antibodies. Furthermore, 45.0% of patients in the severe disease group took > 15.0 mg of prednisone daily before infection, a significantly higher proportion than that in the non-severe disease group. Advanced age, mechanics' hands, dyspnoea, chronic cough and fever during the course of myositis, low lymphocyte count, low serum albumin level, and high D-dimer and ferritin levels before infection were prominent in patients with severe coronavirus disease. Albumin levels below 35.0 g/L and ferritin levels above 306.8 ng/mL were independent risk factors of severe coronavirus disease. CONCLUSION:Omicron did not worsen the overall outcomes of coronavirus disease for patients with idiopathic inflammatory myopathy; however, specific risk factors were identified, highlighting the need for targeted management strategies.

The clinical application of artificial intelligence (AI) models based on breast ultrasound static images has been hindered in real-world workflows due to operator-dependence of standardized image acquisition and incomplete view of breast lesions on static images. To better exploit the real-time advantages of ultrasound and more conducive to clinical application, we proposed a whole-lesion-aware network based on freehand ultrasound video (WAUVE) scanning in an arbitrary direction for predicting overall breast cancer risk score. The WAUVE was developed using 2912 videos (2912 lesions) of 2771 patients retrospectively collected from May 2020 to August 2022 in two hospitals. We compared the diagnostic performance of WAUVE with static 2D-ResNet50 and dynamic TimeSformer models in the internal validation set. Subsequently, a dataset comprising 190 videos (190 lesions) from 175 patients prospectively collected from December 2022 to April 2023 in two other hospitals, was used as an independent external validation set. A reader study was conducted by four experienced radiologists on the external validation set. We compared the diagnostic performance of WAUVE with the four experienced radiologists and evaluated the auxiliary value of model for radiologists. The WAUVE demonstrated superior performance compared to the 2D-ResNet50 model, while similar to the TimeSformer model. In the external validation set, WAUVE achieved an area under the receiver operating characteristic curve (AUC) of 0.8998 (95

This study developed an end-to-end deep learning (DL) model using non-enhanced MRI to diagnose benign and malignant pelvic and sacral tumors (PSTs). Retrospective data from 835 patients across four hospitals were employed to train, validate, and test the models. Six diagnostic models with varied input sources were compared. Performance (AUC, accuracy/ACC) and reading times of three radiologists were compared. The proposed Model SEG-CL-NC achieved AUC/ACC of 0.823/0.776 (Internal Test Set 1) and 0.836/0.781 (Internal Test Set 2). In External Dataset Centers 2, 3, and 4, its ACC was 0.714, 0.740, and 0.756, comparable to contrast-enhanced models and radiologists (P > 0.05), while its diagnosis time was significantly shorter than radiologists (P < 0.01). Our results suggested that the proposed Model SEG-CL-NC could achieve comparable performance to contrast-enhanced models and radiologists in diagnosing benign and malignant PSTs, offering an accurate, efficient, and cost-effective tool for clinical practice.

BACKGROUND AND OBJECTIVE:Anti-melanoma differentiation-associated gene 5-positive dermatomyositis (MDA5 + DM) exhibits the worst prognosis among all subtypes of idiopathic inflammatory myopathies, with substantial heterogeneity in patient outcomes. This study aimed to investigate prognostic factors for MDA5 + DM and develop a scoring system to determine mortality risk. METHODS:This retrospective study included 621 patients with MDA5 + DM. Variables were selected using univariable Cox regression and LASSO regression. Predictive models for mortality risks were constructed using machine learning-based algorithms. A simplified scoring system was established based on the optimal model with thorough validation to ensure predictive accuracy. RESULTS:Seven variables emerged as key factors associated with mortality in MDA5 + DM and incorporated into the mortality risk prediction model: ferritin, lactate dehydrogenase, age at onset, CD8+ T-cell count, C-reactive protein, albumin, and lung computed tomography pattern of NSIP + OP. Among six models, the Cox proportional hazards model demonstrated superior discriminative ability and clinical utility and was translated into a simplified scoring system 'FLATCAN'. This model achieved a concordance index of 0.815 and time-dependent area under the receiver operating characteristic curves for predicting 3-, 6-, and 12-month mortality of 0.895, 0.855, and 0.850, respectively. Patients were effectively stratified into low-, intermediate-, and high-risk groups using the FLATCAN score. Further internal cross-validation, time-point splitting, and rapidly progressive interstitial lung disease-based splitting confirmed the FLATCAN score's robust predictive ability. CONCLUSION:The FLATCAN score provides an easy-to-use tool for predicting mortality risk in patients with MDA5 + DM and may facilitate improved risk stratification-based patient management.

Interstitial lung disease (ILD) is the most prominent clinical feature of antisynthetase syndrome (ASyS), with a subset of patients developing progressive pulmonary fibrosis (PPF), which is associated with a poor prognosis. However, the prevalence, clinical characteristics, and predictive factors of PPF in patients with ASyS remains unclear. This longitudinal cohort study retrospectively enrolled 643 patients with ASyS-associated ILD. Clinical data, outcomes, and serum biomarkers were analyzed in patients with PPF. Receiver operating characteristic curves, least absolute shrinkage and selection operator, and logistic regression were used to identify key biomarkers associated with PPF. Fibrotic ILD was observed in 51.2

Myosteatosis is associated with poor outcomes in various liver diseases. However, standardized methods for assessing, defining, and diagnosing myosteatosis in the context of liver diseases remain unclear. Furthermore, the underlying mechanisms by which myosteatosis leads to pathophysiological progression and adverse health outcomes remain elusive. Therefore, in this review, we elaborate on the currently available measures, definitions, and diagnostic criteria of myosteatosis in the existing literature. We thoroughly clarify the recent evidence and data regarding the possible involvement of myosteatosis in the progression and deterioration of various liver diseases and resulting complications, including liver cirrhosis, chronic viral hepatitis, non-alcoholic/metabolic-associated fatty liver disease, primary sclerosing cholangitis, liver transplantation, and hepatocellular carcinoma. Additionally, it synthesizes insights from basic research on the pathogenesis of myosteatosis, which involves multifactorial mechanisms, including insulin resistance, mitochondrial dysfunction, and chronic inflammation. Finally, from an operational and pragmatic perspective, several regimens, including physical, nutritional, and pharmacological therapies, have been discussed as potential treatments for myosteatosis.

Introduction:The diagnostic accuracy of traditional imaging examination in predicting ypT stage of rectal cancer after neoadjuvant therapy is significantly reduced, which would affect patients’ subsequent treatment choices. This study aimed to investigate the use of endorectal shear wave elastography (SWE) for diagnosing ypT0 stage of rectal cancer after neoadjuvant chemoradiotherapy (nCRT).Methods:Sixty patients with rectal cancer were prospectively recruited in this study. Data on endorectal ultrasound (ERUS) and SWE parameters were collected before nCRT and 6–8 weeks after nCRT. Postoperative pathological results were the gold standard for evaluating the diagnostic accuracy of SWE and ERUS in predicting the ypT0 stage of rectal cancer after nCRT. Receiver operating characteristic (ROC) curve analysis was used to determine the cut-off values of the SWE parameters that best corresponded to the ypT0 stage and analyze the sensitivity, specificity, and accuracy.Results:The diagnostic accuracies of using ERUS to predict the ypT and ypT0 stages of rectal cancer after nCRT were 58.1% (18/31) and 64.3% (9/14), respectively. The ROC curve was constructed with the lesion’s Emean, Emean corrected (EC), Emean difference (ED), Emean corrected differencede (ECD), Emean descendding rate (EDR) and Emean corrected descendding rate (ECDR) values after nCRT, the cut-off values of diagnosing the ypT0 stage were 64.40 kPa, 55.45 kPa, 72.55 kPa, 73.75 kPa, 50.15%, and 55.93%, respectively; the area under the curve (AUC) for diagnosing the ypT0 stage was 0.924, 0.933, 0.748, 0.729, 0.857 and 0.861, respectively. The EC value showed the best diagnostic performance.Conclusion:SWE could improve the accuracy of conventional ERUS in diagnosing the ypT0 stage of rectal cancer after nCRT. It is expected to become a new method to help predict pathological complete responses in clinical practice and provide new evidence for the watch-and-wait approach.

Abstract Objective To investigate the association of serum anti-Jo-1 antibody levels with the disease activity and prognosis in anti-Jo-1-positive patients with antisynthetase syndrome (ASS). Methods This study included 115 anti-Jo-1-positive patients with ASS who were admitted to China-Japan Friendship Hospital between 2009 and 2019. Anti-Jo-1 antibody serum levels at initial admission and follow-up were determined by enzyme-linked immunosorbent assay (ELISA). Global and organ disease activity was assessed at baseline and follow-up according to the International Myositis Assessment and Clinical Studies guidelines. Results Among enrolled patients, 70 (60.9%) patients initially presented with interstitial lung disease (ILD), and 46 (40%) patients presented with with muscle weakness at initial admission. At baseline, patients with ILD had lower levels of anti-Jo-1 antibodies than those without ILD (p = 0.012). Baseline anti-Jo-1 antibody levels were higher in patients with muscle weakness, skin involvement, and arthritis (all p < 0.05) compared to those without these manifestations. Baseline anti-Jo-1 antibody levels were positively correlated with skin visual analogue scale (VAS) scores (r = 0.25, p = 0.006), but not with disease activity in other organs. However, changes in anti-Jo-1 antibody levels were significantly positively correlated with the changes in PGA (β = 0.002, p = 0.001), muscle (β = 0.003, p < 0.0001), and pulmonary (β = 0.002, p = 0.013) VAS scores, but not with skin and joint VAS scores. Older age of onset (hazard ratio [HR] 1.069, 95% confidence interval [CI]:1.010–1.133, p = 0.022) and higher C-reactive protein (CRP) levels (HR 1.333, 95% CI: 1.035–1.717, p = 0.026) were risk factors for death. Conclusion Anti-Jo-1 titers appear to correlate more with disease activity changes over time rather than with organ involvement at baseline, which provides better clinical guidance for assessing the disease course using anti-Jo-1 levels.