bluuebunny/crossref_metadata_2025_split huggingface dataset:
The Neutrophil-to-Lymphocyte Ratio as a Surrogate Marker for Disease Activity in Systemic Lupus Erythematosus: A Correlation Study Using the SLE-DAS
Authors: Wong Ming Yan , Mok Chi Chiu 
Published: November 2024

Background: Systemic lupus erythematosus (SLE) is a multisystem autoimmune disorder mainly affecting women of childbearing age. Mortality and organ damage of SLE is mainly due to uncontrolled disease activity and treatment-related toxicities. Thus, assessment of disease activity is important during routine clinical practice. In recent years, neutrophil-to-lymphocyte ratio (NLR) has been explored as a marker of disease in many autoimmune disorders including SLE. As such, our study objective is to investigate the correlation between neutrophil-to-lymphocyte ratio (NLR) and disease activity of SLE using the SLE disease activity score (SLE-DAS) in our local population. Methods: Consecutive adult patients who fulfilled the ACR or SLICC criteria for SLE were recruited between March 2023 and February 2024. SLE activity was assessed by SLE-DAS, physicians’ global assessment (PGA) and SLE disease activity index-2000 (SLEDAI-2K). The calculated NLR was correlated with disease activity indices and serological parameters (anti-dsDNA, C3/4) by Spearman’s rank correlation. Patients were stratified into remission, low and moderate/severe disease activity as defined by the SLE-DAS and comparison of the NLR was performed among these subgroups by one-way ANOVA. Results: A total of 420 SLE patients were studied (96.2% women, age 46.0± 11.0 years, SLE duration 16.4± 8.3 years). Moderate/severe, low disease activity and SLE-DAS remission was present in 70(16.7%), 66(15.7%), and 284(67.6%) patients, respectively. SLE-DAS correlated significantly with SLEDAI-2K (rho 0.9; p<0.001) and PGA (Rho 0.60; p<0.001). The mean NLR of all patients was 3.54± 4.0 and NLR correlated significantly with SLE-DAS (Rho 0.17; p<0.001). The NLR was significantly higher in active renal (urine P/Cr >0.5, active sediments or histological features) than inactive SLE (4.62 vs 3.27; p=0.02). The NLR in patients with moderate/severe SLE-DAS activity was significantly higher than those in remission (4.87 vs 3.27; p=0.007). ROC analysis showed that a NLR cut-off of 3.11 showed a sensitivity of 55.6% and specificity of 68.7% in detecting moderate/severe SLE-DAS (AUC 0.67[0.6-0.7]; p<0.001). Conclusion: The NLR is a satisfactory and convenient marker that correlates significantly with disease activity in SLE.
Clinical Profile and Treatment Response to Conventional DMARDs in a Tertiary Care Hospital of Pakistan
Authors: Yasmin Saira , Yasmin Saira , Salim Babur , Samreen Saba , Perveen Shahida , Gul Haris , Laila Umme 
Published: November 2024

Background Conventional disease modifying anti-rheumatic drugs (DMARDs) are the first line drugs in a conventional DMARDs in Pakistan. Methods and materials This prospective cohort study was conducted in rheumatology department of FFH Rawalpindi from October 2023 till March 2024. 224 cases of treatment naïve early RA, diagnosed on the basis of ACR2010 criteria, were included. Patients were started on Methotrexate or other conventional. Baseline daa were collected for demographic, clinical parameters including age, gender, smoking, BMI, RA duration and serology. Disease activity was assessed by DAS28 CRP at 3 months for assessing the response to treatment. Correlations and comparisons were performed by Pearson’s correlation coefficient and independent sample t-test. Statistically significance p value was 0.05. Results Females constituted19six (87.5%) and males 22(12.5%) patients. 130 patients were seropositive, 94 were seronegative. 13 patients were smokers(..%). Radiographic damage was present in 67 patients. Mean DAS28 of the study population was 3.05± 89. RA duration was 11.07± 10.70 years. BMI was 27.70± 22.48kg/m2. Table I and II show the results of correlation and comparison of the clinical features with respect to disease activity score. Conclusion Patients with higher BMI has poorer response to conventional DMARDs. Weight optimization may be important step in improving the response to DMARDs in patients with RA.
Initial Rheumatoid Arthritis Treatment: Does Female Age Matter?
Authors: Aldabie Ghaydaa , Khudadah Mohammad , Albasri Anwar , Al-Herz Adeeba , Alsaber Ahmad , Saleh K , Alawadhi Adel , Hasan Eman , Al-Kandari Waleed , Muhanna Aqeel , Ali Yaser , Alhadhood Naser , Tarakmeh Huda , Hayat Sawsan , Aldei Ali , Baroun Fatemah , kadi Amjad Al , Alhajeri Hebah , Abutiban Fatma , Alenzi Ahemd 
Published: November 2024

Introduction Rheumatoid arthritis (RA) is a chronic autoimmune disease affecting females. In RA, the treat to target strategy necessitates an early and intensive approach to achieve disease control. (1, 2) However, the therapeutic options can adversely affect reproductive health and pregnancy. Hence, the age of female patients at presentation might be a factor in decision making; although, there is a scarcity of evidence to support this hypothesis. We analyzed prescribing trends in Kuwait to examine the effect of female age on initial treatment decision. Methods Retrospective analysis of patients enrolled in the Kuwait RA Registry from 2012 to 2024. Baseline characteristics and medication prescribed at the initial visit were analyzed based on age group. Results 1101 female patients were included and stratified into two age groups [Formula: see text]49 years and >49 years. 30.8% were [Formula: see text]49 years and had a higher proportion of rheumatoid factor (RF) n Anti-CCP positivity (82.4% vs 75.5%, p=0.087). There was no significant difference in the mean DAS-28 at baseline (3.2 vs 3.1, p=0.32). A numerically higher proportion of patients started methotrexate (MTX) in the >49 years group (59.4% vs 54%, p=0.090). No statistically significant difference was found between the groups in sulfasalazine (SSZ), hydroxychloroquine (HCQ), or leflunomide (Lef) use. Rituximab was the most prescribed biologic with no statistical difference between the groups in prescription rate (p=0.822); TNF inhibitors (TNFi) were prescribed more often in the >49 years group (16.5% vs 11%, p=0.022); adalimumab, highest TNFi, prescription was not statistically different between the groups; Etanercept was prescribed more often the >49 years group (7.7% vs 4.7% and 5% vs 1.2%) respectively. There was no statistical difference between the groups in monotherapy vs. combination therapy treatment strategy. Discussion & Conclusion This study showed that age at presentation does not significantly influence the initial choice of Disease-Modifying Anti-Rheumatic Drugs in female RA patients in Kuwait. Other factors, such as completion of family and patient preferences, likely play a more critical role in treatment decisions. Further research is needed to explore these influences and optimize management strategies for RA in women, particularly concerning reproductive health.
Anti-Ro Positivity in Systemic Lupus Erythematosus: Association with Clinical Phenotypes and Other Antibodies
Authors: Khan Anum , Perveen Shahida , Samreen Saba , Gul Haris , Salim Babur 
Published: November 2024

Background: Anti-Ro antibody positivity (anti-Ro+) can have possible association with the clinical features and serology in patients with systemic lupus erythematosus (SLE). Methods: This retrospective observational study enrolled 158 SLE patients at the Rheumatology department, Fauji Foundation Hospital, from 1st May to 31st October 2023. Demographic and clinical data were collected. Patients were stratified by anti-Ro (positive vs negative), concomitant anti-La (anti-Ro+/ anti-La+; anti-Ro- / anti-La-; anti-Ro+/anti-La- and anti-Ro-/anti-La+), and ANA status (positive vs negative). Mann-Whitney U and Chi-square tests were employed for comparisons, with statistical significance set at p[Formula: see text]0.05 Results: Study population consisted mainly of females (98.7%), with median age of 30± 20 years. Anti-Ro antibodies were positive in 74(46.8%) patients. This group had significantly higher discoid rash (16.2% vs 6%, p-value 0.04), sicca symptoms (41.1% vs 31%, p-value 0.005), anemia (71.6% vs 56%, p-value 0.04) compared to anti-Ro negative group. No significant differences were observed in nephritis (29.7% vs 34.5%, p=0.52) or neurological disease (9.5% vs 11.9%, p=0.69) between the two groups. Anti-Ro+ /anti-La+ group had higher myositis (20.6%), sicca symptoms (55.9%) and fever (70.6%) compared to other groups. ANA+/anti-Ro+ group (48.6%) had significant association with sicca symptoms (50.7%) and fever (64.2%). Conclusion: Anti-Ro positivity in SLE is associated with muco-cutaneous manifestations and anemia. Concomitant anti-La positivity is linked to increased xerophthalmia, myositis, and fever. While anti-Ro status is independent of ANA, the combination of both autoantibodies is associated with increased sicca symptoms and fever in SLE patients.
Automated Image Analysis of Ultrasound Image using Machine Learning Models: Detection of Double Contours
Authors: Goswami Rudra Prosad , Banerjee Arindam , Jain Aayush , Das Kanoy Kumar 
Published: November 2024

Background: Musculoskeletal ultrasound (MSUS) is commonly used to detect double contour sign (DCS) to diagnose gout. MSUS is subjective and needs user training and experience for optimal visualisation. Machine learning (ML) models can be used for automatic detection of DCS. We sought to identify the optimal algorithm for detection of DCS on ultrasound images of the fist metatarsophalangeal joint (MTPJ1) using candidate ML algorithms. Methods: MSUS images of MTPJ1 of male patients acquired between January, 2020 and January, 2023 were included in this study. Diagnosis of gout was based on imaging, joint aspiration, serum uric acid level, and clinical evaluation. Images were acquired with a high frequency linear transducer (18 MHz) with a dedicated MSUS machine (Toshiba Xario 200). Patients diagnosed with calcium pyrophosphate crystal disease or gross deformities of MTPJ1 were excluded. Selected images were pre-processed using Keras-OCR. The following candidate ML algorithms were trained: Support Vector machine, tree-based algorithms like Random Forest and Decision Tree and boosting-based algorithms like Gradient Boosting and XGBoost and these were compared with a deep neural network (2D-CNN). Classification outcomes (DCS positive or negative) were compared before and after image augmentation. Classification performance was measured using sensitivity, specificity, and area under the receiver operating curve (AUROC) along with accuracy, precision and F1-score. Results: We used 410 high resolution ultrasound images of MTPJ1 of male patients, 311 patients with gout and positive DCS and 99 patients without gout and no DCS. On average, the final model using XGBoost for detection of DCS showed sensitivity of 94% and specificity of 93.7% and AUROC of 85%. Using XGBoost accuracy and precision were 84.74% and 87% respectively and recall and F1-scores were 94% and 90% respectively. Comparison of different models is given in Table 1. Classification performance improved after augmentation in almost all the models. Conclusions: The classification model based on a boosting based ML model (XGBoost) was trained with a relatively small dataset; however, it showed good accuracy. This proposed system was useful in correct classification of DCS in MSUS images with potential to aid imaging diagnostics of gout in a wider clinical setting.
Comparison of Disease Activity Indices in Patients of Rheumatoid Arthritis
Authors: Firdus Maimoona 
Published: November 2024

Objective: To find out correlation between disease activity scores of Rheumatoid arthritis (RA), shedding light on significance of each disease activity score. Patients and methods: It is a cross sectional comparative study. The study was carried out at Rheumatology department, Fauji Foundation Hospital Rawalpindi, Pakistan over a period of six months. 240 patients (33 males, 203 females) aged [Formula: see text]18 years diagnosed as RA according to ACR 2010 criteria, were selected. We recorded demographics, details of RA, laboratory value (baseline chemistry, acute phase reactants, and RA serology), comorbidities, DAS 28 ESR, DAS 28 CRP, CDAI, SDAI and RAPID 3 of all the patients. Analysis was performed using Statistical Package for Social Sciences (SPSS) version 23. Pairwise correlation between the disease activity indices was assessed by Spearman’s log-rank analysis. Results: Median age of the study population was 53 years (range 20-87 years). Duration of RA in study population was 8 years (IQR= 4-15). Twenty-two patients (9.6%) had early RA, whereas 208 patients (90.4%) had established RA. ESR was raised in 163 (70.9%) of the RA patients. Methotrexate was the most common disease modifying drug, which was being used by 180(77.9%) patients. All the disease activity scores showed a significant correlation on bivariate spearman’s analysis. Although the strength of the correlation varied. Disease activity by DAS-28 ESR was most strongly correlated with CDAI. Disease activity assessment via RAPID-3 had the highest correlation with CDAI. There was a fair correlation of RAPID-3 with DAS-28 and a weak correlation with SDAI. Disease activity, as assessed by CDAI and SDAI had a moderate correlation with each other. Conclusion: While all disease activity indices demonstrate agreement in clinical use, CDAI and RAPID-3 offer suitable alternatives to DAS28 in resource-limited settings.
ANCA-Associated Vasculitis in Hong Kong: Clinical Presentation, Treatment and Outcomes from a Multi-centre Retrospective Study
Authors: Ho Cheuk Man 
Published: November 2024

Background ANCA-associated vasculitis (AAV) is a rare disease with significant mortality and morbidity, exhibiting different spectrums among ethnic groups. Chinese patients are more likely to be diagnosed with microscopic polyangiitis (MPA), be MPO-positive, and have renal involvement. However, reliable epidemiological data on AAV in China is scarce. This study aims to provide detailed information on the epidemiology, clinical features, treatment, and outcomes of AAV patients in Hong Kong. Methods Patients diagnosed with Granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), or eosinophilic granulomatosis with polyangiitis (EGPA) and followed up in 8 public hospitals from 2013-2023 in Hong Kong were identified using the Hospital Authority Clinical Data Retrieval System (CDARS) with ICD-10 diagnostic codes. Diagnoses were confirmed by designated rheumatologist specialists at each center. Data on demographics, clinical presentations, laboratory results, biopsy findings, treatments, and outcomes were recorded. Overall survival, relapse-free survival, and dialysis-free survival, along with their associated risk factors, were analyzed using the Kaplan-Meier method and Cox Regression. Data of GPA and MPA patients are presented here. Results A total of 35 GPA and 127 MPA patients were identified, with 91 (56.2%) being female. The mean age of onset was 67.73 years. Forty-six patients (28.4%) were PR3 positive, and 134 patients (82.7%) were MPO positive. Most patients had organ-threatening or life-threatening disease, with 127 patients (78.4%) having an FFS [Formula: see text]2. The mean BVAS score was 15.7. Pulmonary haemorrhage occurred in 35 patients (21.6%), and advanced renal insufficiency (Cr > 4g/L or dialysis) occurred in 49 patients at presentation (30.2%). Thirty-four patients received rituximab, and 83 received cyclophosphamide for induction. PLEX was performed in 30 patients. Over a median follow-up period of 39 months, mortality occurred in 64 patients (39.5%). The 1-year mortality rate was 22.2%, and the 5-year mortality rate was 33.3%. Infections accounted for most of the mortality (46.7%), followed by active vasculitis (15%), renal failure (11.7%), cardiovascular/cerebrovascular events (10%) and malignancy (1.9%). Factors associated with mortality included MPO positivity (OR 4.25, p=0.01), PR3 positivity (OR 3.87, p=0.01), FFS [Formula: see text]2 (OR 2.54, p=0.04) and pulmonary haemorrhage (OR 2.13, p=0.01) in multivariate analysis. Relapse occurred in 30 patients (18.5%), and 30 patients (18.5%) required dialysis. Conclusion AAV patients in Hong Kong, predominantly MPA and MPO positive, often present with severe organ or life-threatening manifestations. There is a high mortality risk among these patients, with MPO or PR3 positivity, FFS[Formula: see text]=2 and pulmonary haemorrhage being significant predictors of mortality.
The Knowledge Level of Patients About Rheumatology and Rheumatic Diseases in Developing Country: A Tertiary Care Hospital Study
Authors: Islam Asif , Imran Yasir , Fatima Tooba , Siddique Samara 
Published: November 2024

Background: In developing nations, rheumatic diseases bear significant health issues, affecting millions of people and placing a substantial burden on healthcare systems. Education of the patient is crucial for the management and prevention of rheumatic diseases. Objectives: To determine the level of patient knowledge regarding rheumatology and rheumatic diseases in a developing nation. Methods: Patients attending rheumatology clinics of Mayo Hospital, Lahore, in 2021-23, participated in this cross-sectional study. A structured questionnaire was used to collect data on demographic characteristics and rheumatology and rheumatic disease knowledge of patients. Questions pertaining to the definition, symptoms, causes, and treatment of rheumatic diseases were included in the questionnaire. Results: The study enrolled a total of 1306 patients. Demographic characteristics of rheumatic disease patients comprised 377 males (28.9%) and 929 females (71.1%), whereby the proportion of female patients was significantly high (p[Formula: see text]0.05). The mean age of the participants was 43.22+10.98 years. Patients’ locations were categorized as either rural or urban with statistically significant number (p[Formula: see text]0.05) of 757 (57.96%) and 549 (42.03%), respectively. Majority of patients were educated (p[Formula: see text]0.05), with 706 females (74.5%) and 248 males (65.7%). Patients’ overall knowledge of rheumatology and rheumatic diseases was inadequate, with majority of the patients (56.4%) had scores between 0 and 2, indicating a lack of understanding of these conditions. Rheumatic disease causes, symptoms, and treatment options were poorly understood by patients. Conclusion: In developing nations, patient education regarding rheumatology and rheumatic diseases appears to be deficient. Providers of healthcare should address this deficiency by providing patients with adequate education and resources. Improved patient knowledge will ultimately improve patient outcomes and lessen the burden of rheumatic diseases on the healthcare systems of developing nations.
Comparing the Efficacy of Conventional Immunosuppression and Rituximab in Anti-SRP Myositis: Insights from a Tertiary Care Centre Experience
Authors: Manwatkar Abhilasha 
Published: November 2024

Background Consensus on anti-SRP myositis shows that early rituximab (RTX) is favoured over conventional immunosuppression (cIS), but the data is derived from case series without comparative studies. This study aims to a) explore poor prognostic features, and b) assess and compare the efficacy and safety of RTX with cIS in individuals with anti-SRP myositis. Methods This is an observational study in a tertiary care centre in India. Data was obtained from a prospectively maintained database comprising consecutive adult (> 16 years) patients diagnosed myositis. A total of 1033 myositis patients were screened from January 2019 to January 2024. Fourty IIM patients with confirmed anti-SRP positivity fulfilling inclusion criteria with more than 3 months of follow-up were selected for the study and response analysis. Details were obtained at baseline, 3 months, and 6 months, as well as subsequent follow-up. Treatment response was categorised as improved, worsening, or stable disease. The characteristics of patients between “RTX” and “cIS” groups were compared using t-test and Chi-square test as appropriate. Survival analysis curves were plotted for improvement and death between the two groups. Results The mean age of onset of anti-SRP myositis was 44.2 (SD13.14) years; 71.4% of patients were female. Baseline characteristics are described in Table 1. For response analysis, 35 patients into two groups: RTX and cIS. Fifteen (42.85%) patients were given RTX. While 20 received cIS (57.14%, 12/20 (60%) received mycophenolate mofetil, 8/20 (40%) received methotrexate, and 5/20 (25%) received cyclophosphamide). The median follow-up was 12 (IQR 6-24) months. There was no significant difference in improvement between RTX and cIS groups (11/15 (73.3%) RTX group and 9/20(45%) cIS group (p-value -0.465)). Time to improvement was also not significantly different (Figure 1). However, the Mean change in MMT8 from baseline to 6 months was more in the RTX group (p-value 0.001). The average monthly steroid dose significantly decreased six months after RTX with a p-value of 0.001(1600.13mg (SD 271.53) in RTX versus 826.6 mg (SD 370.35) in cIS). Mortality rates stood at 1/15 (6.67%) for RTX and 4/20 (20%) for cIS. Unravelling predictors of mortality revealed significant associations with cardiac involvement (p-value - 0.02) and severe non-ambulant muscle weakness (p-value - 0.03). Conclusions Both cIS and RTX are effective and safe in anti-SRP myositis for muscle affection and time for response. Cardiac involvement and severe muscle weakness are associated with mortality. RTX use decreases steroid requirement in anti-SRP myositis.
Juvenile Dermatomyositis Outcome Prediction Using Magnetic Resonance Imaging STIR Sequences
Authors: George Sabin , Balan Suma , Thottiyil Jasica Joy , Abhinav Kumar 
Published: November 2024

Background – Juvenile Dermatomyositis can have a unpredictable course after diagnosis, with no tools to prognosticate near-term outcomes. We explored a Short Tau Inversion Recovery (STIR) sequence Magnetic Resonance Imaging (MRI) protocol as a non-invasive baseline investigation to assess the present outcome in a cohort of JDM patients. Objectives – To determine the difference in baseline STIR-MRI parameters between children with JDM, who are currently in remission with medication and children in sustained remission off medications. Methods – We retrospectively reviewed the baseline MRI findings of 28 consecutive patients, evaluated and followed up for at least one year, by a senior paediatric rheumatologist. A radiologist reviewed the images of each child’s MRI thigh STIR Sequences, scored on four domains - STIR hyper-intensities, fatty infiltration of muscle, presence of skin and subcutaneous tissue involvement and muscle atrophy. We compared the median (inter quartile range) scores between children in remission with and without medication. Results – Median baseline age was 7 (7.5) years. At baseline, they reported a median CMAS of 17 (21), with STIR signal intensity of 2 (2), fatty infiltration grade of 0 (1). Twelve (42.9%) and four children (14.3%) showed evidence of skin and subcutaneous involvement and muscle atrophy, respectively. After being followed up for a median of 6 (6) years, eight children (28.6%) were in remission off medication. There was evidence that fatty infiltration differed between children in remission with medication vs off medication (p = 0.043). MRI Fatty infiltration grades predicted delayed treatment initiation ([Formula: see text] 6 months since symptom onset) in children, fairly accurately (AUC 0.731 ± 0.11, p = 0.036). Conclusions – Our study indicates that fatty infiltration in baseline STIR-MRI protocol may suggest a chronic disease course. Fatty infiltration, also suggests a history of inadequate/delayed diagnosis and treatment since symptom onset. Thus, early MRI may prognosticate the disease course in children with JDM. Further research is needed to exclude potential effect modifiers and confounders in the analysis.