Objective:This study aims to investigate the clinical characteristics and identify risk factors associated with progressive pulmonary fibrosis (PPF) in individuals diagnosed with primary Sjögren's syndrome (pSS). Methods:A retrospective case-control study was conducted from individuals with pSS-associated interstitial lung disease (pSS-ILD) registered in the Chinese Rheumatism Data Center between June 2010 and October 2023. Participants were categorized into two groups: those with PPF (pSS-PPF) and those without PPF (pSS-non-PPF). Comparative analyses were performed on clinical manifestations, laboratory parameters, pulmonary function, and treatment history between the two groups. Results:Sixty-six individuals with pSS-ILD were included, of whom 29 met the criteria for PPF. Compared to pSS-non-PPF group, the pSS-PPF group demonstrated a higher rate of expectoration (48.3% vs 16.2%, p = 0.005) and crackles on auscultation (41.4% vs 13.5%, p = 0.01), but lower rates of parotid gland enlargement (3.4% vs 32.4%, p = 0.003), and arthritis (6.9% vs 27%, p = 0.035). Additionally, the incidence rate of the subjects suffering xerophthalmia and xerostomia in the PPF group was lower (24.1% vs 2.7%, p = 0.023). Pulmonary function testing showed significantly reduced forced vital capacity percentage predicted (83.6±15.6 vs 91.3±14.6, p = 0.042) and diffusing capacity of the lung for carbon monoxide percentage predicted (DLCO%, 54.2±21.5 vs 68.5±13.6, p = 0.003) in the PPF group. Multivariate logistic regression identified a baseline DLCO% < 60% as an independent risk factor for PPF. Parotid gland enlargement and arthritis were potentially protective. The predictive model demonstrated good performance, with an area under the curve of 0.821 (95% CI: 0.716~0.925, p < 0.001). The sensitivity was 58.6% and the specificity was 91.7%. Conclusion:A baseline DLCO% < 60% is an independent predictor of PPF in individuals with pSS. The developed predictive model shows strong discriminatory ability, while further validation in larger cohorts is warranted.

Primary Sjögren’s syndrome (pSS) was one of the pivotal causes of pulmonary arterial hypertension (PAH) among connective tissue diseases (CTDs) with adverse outcomes, but genetic studies of pSS-PAH are quite limited. Studies have identified genetic predisposition in both familial PAH (fPAH) and idiopathic PAH (iPAH) cases. Therefore, the aim of this study was to explore the genetic susceptibility of PAH in pSS. Forty-three pSS-PAH patients, 92 pSS‐non‐PAH patients, and 105 healthy controls (HC) were obtained. PAH was diagnosed according to the 2015 European Society of Cardiology (ESC)/European Respiratory Society (ERS) guidelines, and ruled out by echocardiogram as estimated PAP < 40 mmHg. Single-nucleotide polymorphism (SNP) sites related to PAH and pSS were selected and then compared between pSS-PAH and pSS-non-PAH groups. In pSS-PAH group, 97.7

OBJECTIVE:Early personalized identification of SSc patients at risk of scleroderma renal crisis (SRC) can help provide better treatment and improve outcomes. This study aimed to create and validate a new multi-predictor Nomogram to predict SRC risk and compare it to an existing model. METHODS:A retrospective multicentre observational study was conducted using clinical data from SSc patients with SRC registered in the Chinese Rheumatism Data Center (CRDC) database. Each SSc patient with SRC was matched with four SSc patients without SRC, registered consecutively afterward, as controls. Differences between the two groups were analysed using Student's t-test, Mann-Whitney U test, χ2 test, or Fisher's exact test. Key risk factors were identified using univariate and multivariate logistic regression, as well as LASSO regression. The Nomogram's performance was assessed with receiver operating characteristic curves, calibration plots, decision curve analysis (DCA), and bootstrap resampling for internal and external validation. Net reclassification improvement (NRI) and integrated discrimination improvement (IDI) were used to compare models. RESULTS:The Nomogram incorporated predictive factors such as myocardial involvement, SSc subtype, anaemia, platelet count, and disease duration. The area under the ROC curve showed strong discrimination in both the training and validation datasets. Calibration curves and the Hosmer-Lemeshow test indicated good agreement between predicted and actual outcomes. DCA demonstrated greater clinical net benefit. The NRI and IDI results showed significant improvement over the previous model. CONCLUSION:A Nomogram with improved predictive performance compared with the previous one was developed in a larger sample size in China.

This study aims to investigate the characteristics, risk factors, and outcomes of digital gangrenes in SSc patients, and to identify whether vasculitis is one of the causes for digital gangrene. A retrospective case–control study was performed from February 2003 to April 2021. Forty-three SSc patients with digital gangrene admitted to Peking Union Medical College Hospital were included. One-hundred forty-six age- and sex-matched SSc patients without gangrene were selected as controls during the same period. Univariate and multivariate logistic regression analysis was used to determine risk factors. Among 43 SSc patients with gangrene, 93.0

Background This study aims to establish a reliable prediction model of progressive fibrosing interstitial lung disease (PF-ILD) in patients with systemic sclerosis (SSc)-ILD, to achieve early risk stratification and to help better in preventing disease progression.Methods 304 SSc-ILD patients with no less than three pulmonary function tests within 6–24 months were included. We collected data at baseline and compared differences between SSc patients with and without PF-ILD. Least absolute shrinkage and selection operator regularisation regression and multivariable Cox regression were used to construct the prediction model, which were presented as nomogram and forest plot.Results Among the 304 patients with SSc-ILD included, 92.1% were women, with a baseline average age of 46.7 years. Based on the 28 variables preselected by comparison between SSc patients without PF-ILD group (n=150) and patients with SSc PF-ILD group (n=154), a 9-variable prediction model was constructed, including age≥50 years (HR 1.8221, p=0.001), hyperlipidemia (HR 4.0516, p<0.001), smoking history (HR 3.8130, p<0.001), diffused cutaneous SSc subtype (HR 1.9753, p<0.001), arthritis (HR 2.0008, p<0.001), shortness of breath (HR 2.0487, p=0.012), decreased serum immunoglobulin A level (HR 2.3900, p=0.002), positive anti-Scl-70 antibody (HR 1.9573, p=0.016) and usage of cyclophosphamide/mycophenolate mofetil (HR 0.4267, p<0.001). The concordance index after enhanced bootstrap resampling adjustment was 0.874, while the optimism-corrected Brier Score was 0.144 in internal validation.Conclusion This study developed the first prediction model for PF-ILD in patients with SSc-ILD, and internal validation showed favourable accuracy and stability of the model.

Background The presence of circulating antinuclear antibodies (ANAs) is a hallmark of immune dysregulation in patients with systemic sclerosis (SSc). Objective A variety of ANAs are associated with unique sets of disease manifestations and are widely used in clinical practice in SSc. This study aimed to investigate the clinical features of SSc patients negative for ANAs in a Chinese Rheumatism Data Center (CRDC) multicenter cohort in China. Methods Patients were prospectively recruited between April 2008 and June 2019 from 154 clinical centers nationwide, and all cases fulfilled the 2013 ACR/EULAR classification criteria for systemic sclerosis. Results for antinuclear antibodies were intensively collected. Demographic, clinical, and laboratory data were compared between ANA-positive SSc patients and those negative for ANAs. Results Antinuclear antibodies were detected in 2129 of 2809 patients enrolled in the study; 4.2% of patients were negative. There were more males among ANA-negative SSc patients (29/60 vs. 294/1746, p < 0.001). The incidence of certain critical organ involvement, including gastroesophageal reflux (5.6% vs. 18.5%, p = 0.002), interstitial lung disease (65.2% vs. 77.9%, p = 0.015), and pulmonary arterial hypertension (11.5% vs. 29.0%, p = 0.006) was significantly lower in ANA-negative patients than in ANA-positive patients. The proportion of abnormal erythrocyte sedimentation rate (32.4% vs. 47.6%, p = 0.013) and IgG elevation (14.3% vs. 37.0%, p = 0.003), an indicator of disease activity, was significantly lower in ANA-negative patients than in ANA-positive patients. Conclusion Antinuclear antibodies are strongly associated with the clinical manifestations of systemic sclerosis, with ANA-negative SSc patients tending to exhibit relatively milder disease.

Left ventricular diastolic dysfunction (LVDD) is a common manifestation of cardiac involvement in systemic sclerosis (SSc), which is associated with increased mortality, but little is known about the risk factors. The aim is to determine the frequency and potential predictors of SSc-LVDD. We conducted a prospective multi-center cohort study, enrolling 784 SSc patients assessed by echocardiography between April 2008 and June 2019. Diagnosis of systemic sclerosis was according to the 2013 American College of Rheumatology (ACR)/the European League Against Rheumatism (EULAR) classification criteria. Data were compared between patients with and without LVDD, while univariate and multivariate regression analysis was performed to determine the factors independently associated with LVDD. LV diastolic dysfunction was present in 246/784 (31.4%) of the subjects. There were no significant differences in gender, BMI, or disease duration between the two groups. Around 40% of the patients in the SSc-LVDD group and in the SSc-non LVDD group had diffused cutaneous involvements. Factors independently associated with LV diastolic dysfunction in multivariable analysis included age at onset (OR 1.053, 95%CI 1.021–1.086, p = 0.001), pulmonary arterial hypertension (OR 3.057, 95%CI 1.468–6.367, p = 0.003), positivity of anti-RNP antibody (OR 2.455, 95%CI 1.049–5.745, p = 0.038), increased WBC count (OR 1.156, 95%CI 1.037–1.287, p = 0.009), elevated levels of uric acid (OR 1.003, 95%CI 1.000–1.006, p = 0.036), and triglyceride (OR 1.515, 95%CI 1.106–2.077, p = 0.010). LV diastolic dysfunction was prevalent in the SSc population. Advanced onset age, PAH, positive anti-RNP antibody, increased WBC count, and adverse metabolic status were independent risk factors for SSc-related LVDD.

Pulmonary arterial hypertension (PAH) is a severe complication and leading cause of mortality in patients with primary Sjogren’s syndrome (pSS). This study was to investigate the overall survival rates and the utility of red blood cell distribution width (RDW) as a potential prognostic factor of pSS-PAH. This cohort study retrospectively enrolled 55 patients with pSS-PAH who were followed up at the Department of Rheumatology of Peking Union Medical College Hospital (PUMCH) between August 2007 and May 2017. The patients were stratified according to the level of RDW (≤ 15.0 and > 15.0%). Baseline demographics, laboratory results, pulmonary function conditions, hemodynamic assessments, and treatment regimens were analyzed. Cox proportional hazards regression analysis was used to identify whether RDW level is a factor related to adverse outcome. A total of 55 patients were recruited, with an average age of 38.9 ± 9.3 years. Fifty-four were female (98.2%), and the average duration at the time of PAH diagnosis was 25.5 ± 33.2 months. Higher RDW levels were found in patients who deceased in follow-up (13.8 ± 2.6 vs 16.5 ± 1.6%, p = 0.003) and with higher NYHA classes (13.8 ± 1.8 vs 16.5 ± 2.9%, p < 0.001). Patients with RDW > 15% had a significantly worse overall survival than patients with RDW ≤ 15% (3-year survival rate 59.5 vs. 88.7% log-rank p = 0.015). Cox regression analysis identified RDW > 15% as a prognostic factor for adverse outcome (HR 1.786, 95% CI 1.137–2.803, p = 0.012). RDW can serve as a potential negative prognostic factor of pSS-PAH.

Tumor-induced osteomalacia (TIO) is a rare disease that behaves benignly. Very few reports about the features of the responsible tumors according to anatomical locations have been presented.In this retrospective study of 53 patients with TIO-associated tumors in the foot/ankle, tibia and femur, we compared preoperative, postoperative, and follow-up courses, including alkaline phosphatase, phosphorus, and fibroblast growth factor 23, to compare the characteristics of TIO-associated tumors in these 3 locations (level of evidence: therapeutic level III).Patients in the foot/ankle group had longer disease courses and therefore a significantly higher complication rate (P<.001). All TIO-associated tumors in the foot/ankle group involved soft tissue (P=.021), whereas most lesions in the tibia group involved bone, and therefore had much higher concentrations of alkaline phosphatase (P=.020). Additionally, serum phosphorus took much longer to normalize after surgery in the foot/ankle group than that in the other 2 groups (P=.004). Consequently, symptom remission was much better in the tibia and femur groups (P=.008). Moreover, the Ki 67 index in TIO-associated tumors was significantly higher in the foot/ankle group (P<.001) and the recurrence rate in this group was markedly higher (P=.002).The TIO-associated tumors in the foot/ankle are characteristically of occult onset, more soft-tissue involvement, and more readily recurrence. More knowledge and examinations are necessary to enable early diagnosis, radical treatments, and minimize recurrence. New therapies are welcomed and needed.

It seemed to be common sense that malnutrition was associated with osteoporosis, but there were few studies with detailed data proving that. Additionally, the association between BMI and osteoporosis was still under discussion. In our study of 138 patients, we first confirmed the association between nutrition and osteoporosis with propensity score matching method reducing the confounding bias, then discovered that body mass index (BMI) and 25OHD level acted as two crucial factors of nutrition risk-mediated osteoporosis. Moreover, a new BMI classification was proposed in our article to do more help for nutrition management and anti-osteoporosis treatment for the old in China. Consequently, nutrition is important to bone health, with BMI and 25OHD level playing key roles.