bluuebunny/crossref_metadata_2025_split huggingface dataset:
Systemic Sclerosis Sine Scleroderma Presenting as Pulmonary Arterial Hypertension - A Case Report
Authors: Karar Ali 
Published: November 2024

A 60-year-old married male farmer with a significant medical history, including STElevation Myocardial Infarction (STEMI), coronary artery bypass grafting (CABG), and Raynaud’s disease, presented to the emergency department with sudden-onset syncope and progressive shortness of breath on exertion. The syncope episode involved an abrupt loss of consciousness, with spontaneous recovery and no associated symptoms like jerky movements or tongue biting. The shortness of breath had been gradually worsening over several weeks, becoming noticeable even during minimal exertion. On examination, the patient appeared chronically ill but had stable vital signs cardiovascular examination revealed bilateral pedal edema, while respiratory assessment and Neurological evaluation were normal. Blood tests indicated normal full blood count (FBC) and urea and electrolytes (U+E), but slight elevations in CRP 29 mg/L and ESR 20 mm/hr. Troponin levels remained stable, but brain natriuretic peptide (BNP) was markedly elevated at 2061 pg/mL. The electrocardiogram (ECG) showed right axis deviation and incomplete right bundle branch block (RBBB). A CT scan of the brain ruled out any acute pathology, such as stroke or hemorrhage. A previous echocardiogram (ECHO) had shown a left ventricular ejection fraction (LVEF) of 50-55%, indicating good left ventricular function. Given the findings, the patient was started on Furosemide 40 mg daily and advised to follow a fluid restriction regimen to prevent worsening of overload symptoms. A repeat echocardiogram revealed deterioration in right ventricular (RV) function with a significantly (RVSP) of 80 mmHg, suggesting severe pulmonary hypertension (PHTN). A D-dimer test was negative for pulmonary embolism (PE). Cardiology consultation raised concerns about possible chronic thromboembolic disease, leading to further testing, including a computed tomography pulmonary angiogram (CTPA) and an autoimmune screen, along with potential right heart catheterization (RHC). The CTPA was negative for PE, but the autoimmune screen returned positive results for antinuclear antibodies (ANA >1200) and anticentromere antibodies. Right heart catheterization confirmed pre-capillary pulmonary hypertension (PAH) with pulmonary artery pressure exceeding 44 mmHg and no nitrate response. A rheumatology review, in light of the patient’s history of Raynaud’s disease, positive ANA, and confirmed PAH, led to the diagnosis of Systemic Sclerosis Sine Scleroderma–a form of systemic sclerosis without skin involvement. The patient was initiated on Tadalafil, (PDE-5) for PAH management and prescribed home oxygen therapy, particularly during exertion. He was discharged with a plan for multidisciplinary followup involving rheumatology, cardiology, and pulmonology to manage his complex condition. Conclusion: Multidisciplinary is essential for optimizing outcomes in SSc and PAH.
Efficacy and Safety of Rituximab in Lupus Nephritis from a Tertiary Care Rheumatology Clinic in India
Authors: Gopal Manikandan , Mathew Ashish J , Mathew John 
Published: November 2024

Background: LUNAR trial failed in lupus nephritis. However case series across the globe have shown good outcome with rituximab in lupus nephritis. So we evaluated the efficacy and safety of rituximab in lupus nephritis in Indian patients Methods: This was single centre, retrospective study done in patients of lupus nephritis receiving rituximab between 2013 to 2022. All patients fulfilled the SLICC criteria. Rituximab was given if there was a flare with background immunosuppression or upfront for early steroid tapering. Patients were followed up at 3, 6 and 12 months. Complete kidney Response (CR) is defined as return of serum creatinine(Scr) to previous level with a decline in 24 hour urine protein to <500 mg at 12 months. (1) Partial kidney response (PR) was defined as an improvement of Scr, but not to the standardized normal, with at least 50% decline in the 24 hour urine protein achieved preferably by 6 months and no later than 12 months following treatment initiation. Results: Out of 125 patients who were included for analysis, CR and PR were achieved in 79(63%) and 18(14%) patients respectively at 12 months. Mean steroid dose in prednisolone equivalent was less than 10 mg at 6 months in all patients. Improvement in parameters like dsDNA, c3, c4 were observed. In follow up 14 patients (11%) developed Chronic kidney disease (CKD), 6 patients (4.8%) required dialysis and 8 patients (6.4%) expired. Subgroup analysis between patients who received rituximab upfront and those after treatment failure or relapse didn’t show statistical difference in results including CR,PR,CKD, dialysis requirement or death. Conclusions: These results indicate the possible efficacy and safety of rituximab based regimen with its potential to reduce the steroid requirement as well as achieving remission in treatment naïve and in patients who are refractory or relapse with standard agents in Indian Subcontinent.
Sleep Quality in Systemic Lupus Erythematosus
Authors: Aggarwal Anushka , Handa Rohini , Upadhyaya Sundeep , Gupta S J 
Published: November 2024

Background: SLE is a chronic heterogenous systemic autoimmune disease associated with significant morbidity and poor health related quality of life. Chronic sleep disturbances have been found to have detrimental effects on health and functioning. In this study we aim to evaluate and compare the sleep quality in SLE patients vs. healthy controls using validated sleep questionnaires in the Indian population. Methods: We assessed sleep quality in enrolled SLE patients and age-sex matched controls using Pittsburgh Sleep Quality Index (PSQI) and Epworth Sleepiness score. Data on demographics, quality of life (QoL) and functional disability was collected using the WHO QoL Brief version and Health assessment Questionnaire (HAQ)-CRD version. Disease severity i.e. activity and damage scores were recorded during clinical examination. Results: 90 cases and 90 controls were enrolled in this study. In cases, the mean age was 37.11 ± 13.01 years with 90% females, average disease duration of 7.12 ± 6.65 years, SLEDAI-2k of 4.01± 3.72 (i.e. mild disease) and SLICC-DI of 0.40± 0.76 (Table 1). 72.2% cases had Musculo-skeletal involvement, 50% has mucocutaneous, 30% had nephritis and 25.6% had hematological abnormalities as primary complaints. Few cases had serositis (5.6%), gangrene (2.2%), anti-phospholipid syndrome (3.3%) and pulmonary involvement (3.3%). Cases demonstrated worse sleep quality than controls with a mean PSQI of 7.59± 3.33 vs 4.52± 2.90 respectively (p[Formula: see text]0.001). 82.2% (n=74) cases had PSQI score [Formula: see text]4. Cases also had poorer PSQI component scores i.e. quality, latency, disturbances, medication use and daytime dysfunction (p[Formula: see text]0.001). Epworth sleepiness score was significantly higher in the SLE group (4.68± 3.31 vs 2.19± 2.55) (Table 2). Poor sleep quality was associated with lower WHO QoL domain scores and higher HAQ scores (p[Formula: see text]0.001). Significant association was found between disease activity (SLEDAI-2K) and PSQI total score (p=0.027), but no such correlation was found with organ involvement or total damage. Conclusion: Sleep quality was significantly worse in SLE patients than controls. This was associated with overall poor quality of life and physical function. Given that sleep is multifactorial; symptoms such as fatigue, myalgia, joint pains and rashes significantly affect sleep but maybe missed on standard disease indices. Poor sleep translates into poor general health which the patient may experience despite controlled disease.
Ultrasound and Power Doppler Evaluation of the Distal Interphalangeal Joints in Patients with Psoriatic Arthritis
Authors: Vu Minh 
Published: November 2024

Objective: 1. To evaluate the frequency of the various lesions in distal interphalangeal joints of patients with psoriatic arthritis by clinical and ultrasound assessment. 2. To determine the correlation between grayscale and Power Doppler ultrasonography score with disease activity score. Methods: This cross-sectional study was performed on 41 patients, who were diagnosed with psoriatic arthritis by CASPAR 2006 criteria. Results: Ultrasound detected more joint lesions than clinical examination. The proportion of patients with clinical distal interphalangeal arthritis was 12,2%. 100% of patients had at least one distal interphalangeal arthritis by ultrasound, and 27,6% of patients had periarticular soft tissue lesions. The frequency of various ultrasound abnormalities was as follows: Synovial hypertrophy was seen in 55,9%, Power Doppler abnormality suggesting hypervascularity in 52,5%. The periarticular lesion frequency: extensor tendonitis in 6,1%; loss of normal fibrillary architecture of extensor tendon erosions was seen in 14,4%; flexor tenositis in 7,1%; and other lesions were seen in 32,8% of patients. Significant correlation was found between DAPSA score and grayscale joint count (GSJC) (Spearman’s R=0,55; p[Formula: see text]0,001), grayscale joint score (GSJS) (R=0,62; P<0,001), Power Doppler joint count (PDJC) (R=0.68; p[Formula: see text]0,001), and Power Doppler joint score (PDJS) (R=78; p[Formula: see text]0,001). Conclusion: Ultrasound can detect more joint lesions than clinical assessment. The severity of lesions on ultrasound correlates with the disease activity score.
Impact on Work Productivity Among Patients with Axial Spondyloarthritis in a Rheumatology Specialized Care Centre in Sri Lanka
Authors: Kulasinghe Udari , Abeysinghe Duminda 
Published: November 2024

Background: Axial Spondyloarthritis (AxSpA) is a chronic inflammatory condition which impacts various aspects of patients’ lives, including work productivity. This study aims to identify the impact of AxSpA on key work outcomes including sick leave rate, disease related unemployment rate, work productivity loss and its association with disease activity. Methods: 62 Adult patients aged ¿20 years who met the Assessment in Spondyloarthritis International Society (ASAS) 2009 criteria for AxSpA were enrolled. Work productivity was assessed by the Work Productivity and Activity Impairment scale (WPAI:SpA). The validated Sinhala version of WPAI questionnaire was used to obtain data and calculate absenteeism, presenteeism and work productivity loss (WPL). Higher scores for presenteeism, absenteeism, or WPL indicate greater work impairment due to illness. Disease activity was assessed by Ankylosing Spondylitis Disease Activity Score (ASDAS). Multivariate linear regression was utilized to assess the association between work productivity and disease activity. Results: Among 62 patients who were on paid employment, permanent loss of employment rate due to disease was 20.9% and disease related sick leave rate for [Formula: see text] 2 months was 58.06%. Out of them 48% engaged in heavy labor jobs and 65% had high or very high disease activity. (Figure 1) Among employed patients, mean Absenteeism, Presenteeism and WPL were 22%, 51.04% and 56% respectively. A decrease in work productivity was related to an increase in disease activity. Disease activity was strongly correlated with absenteeism (p [Formula: see text] 0.01), presenteeism (p [Formula: see text] 0.01) and work productivity loss (p [Formula: see text] 0.001). (Figure 2) Conclusion: The impact of AxSpA on work outcomes is substantial and necessitates effective intervention, like controlling disease activity. The significantly elevated levels of presenteeism, absenteeism, and WPL when compared to global data may be linked to suboptimal disease management due to limited availability of biological treatments as well as labor-intensive jobs among the population.
Primary Apla Syndrome Presenting as Stroke in Young Male: A Case Report
Authors: Islam Asif , Haider Umer , Khurshid Fatima , Shahid Kinza , Khalood Memoona 
Published: November 2024

Background: Antiphospholipid syndrome (APLA) is an autoimmune disease with autoantibodies and hypercoagulability. APLA commonly presents vascular thrombosis and obstetrical complications. Stroke in young adults is rare, and unusual causes must be considered. Case Report: Here we present a case of 26 year-old male patient with sudden onset of hemiparesis marked on right side without evidence of atherothrombosis or other vascular pathology. However extensive workup revealed primary Antiphospholipid Antibody Syndrome (APLA) as the underlying cause. MRI brain showing acute infarcts involving both cerebellar hemispheres more on left side, bilateral periventricular and deep white matter infarcts involving bilateral frontal and parietal lobes, Periventricular T2WI hyperintensities likely suggestive of Fazekas II change. Prompt treatment with anticoagulation led to significant neurological recovery. Conclusion: This case highlights the importance of considering APLA in the differential diagnosis of stroke in young adults, and the need for early recognition and treatment to improve outcomes.
Anti-Synthetase Syndrome: A Rare and Challenging Diagnosis for Interstitial Lung Disease (ILD): A Case Report
Authors: Islam Asif , Shahid Kinza , Khurshid Fatima , Haider Umer , Khalood Memoona 
Published: November 2024

Background: ASA syndrome is a rare and complex immune-mediated entity with heterogeneously diverse presentations diagnosed by the presence of antibodies directed against aminoacyl tRNA synthetase (most common anti-JO-1). The clinical manifestations range from myositis, interstitial lung disease (NSIP PATTERN), non-erosive arthritis, unexplained recurrent fever, Raynaud’s phenomenon, mechanics hands involving mainly skin, respiratory and musculoskeletal systems. Case Report: We report a case of 37-year-old female who had multiple admissions for polyarthritis which was symmetrical, progressive, involving large and small joints associated with morning stiffness, relieved by medication temporarily, with subsequent development of respiratory symptoms like cough, fever off and on, shortness of breath, exertional dyspnea, along with cracks on fingers of hands (mechanics hands). Symptoms worsened gradually with time, affecting her daily routine activities like combing hair, changing clothes, exertional dyspnea on taking few steps, debilitating her quality of life. Extensive and detailed workup was done, Anti-Jo-1 antibodies and Anti-SSA/Ro were positive, CPK level raised, serum aldolase raised, HRCT chest showed interstitial lung disease most likely NSIP, ANA negative, ACE levels normal, Bronchial biopsy showed benign respiratory mucosa, no evidence of malignancy was found. Patient was diagnosed with anti-synthetase antibody syndrome and started on pulse therapy with methylprednisolone. Conclusion: This article explores the clinical manifestations, diagnostic criteria, treatment, progression and prognosis of the disease course.
Efficacy of Mycophenolate in Interstitial Lung Disease Associated with Rheumatoid Arthritis
Authors: Rajendran Sindhuja , Mathew Ashish Jacob , Mathew John , Goel Ruchika 
Published: November 2024

Background: Interstitial lung disease (ILD) is a significant cause of mortality in patients with Rheumatoid Arthritis (RA). Existing studies on the efficacy of Mycophenolate (MPA) for RA-associated ILD are limited, with the largest study including only 26 patients. This study aims to evaluate the efficacy of MPA in stabilizing or improving lung function in a larger cohort of RA-ILD patients over an extended follow-up period. Methods: Patients diagnosed with RA-ILD and treated with MPA for [Formula: see text] 1year, attending our clinics between 2017 and 2023 were included. Data regarding demography, spirometry, HRCT and treatment were noted retrospectively at the diagnosis of ILD (baseline) and 6 monthly visits thereafter till last follow up. Data were collected on patient demographics, spirometry, high-resolution CT (HRCT) scans, and treatment regimens. The primary outcome was the change in predicted forced vital capacity (FVC) over the follow-up period, with a [Formula: see text] 10% decline considered as progression. Clinical improvements in cough, dyspnea, and HRCT findings were recorded as binary outcomes. Results: Of the 73 patients (58 females), 70% showed stability or improvement in FVC, while 18% experienced a decline. Clinically, 86% of patients improved or remained stable, while 12% worsened. HRCT results showed stable or improved disease in 34% of patients, 30% had worsening of fibrosis, while 36% did not undergo follow-up HRCT due to clinical stability. Physiologically - 52(70%) met the primary outcome of stability/ improvement in % FVC while 18% had worsening in % FVC. Use of rituximab was not associated with better outcomes. None of the baseline clinical parameters predicted better outcomes in univariate. The most common adverse events were dyspepsia and serious infections, occurring in 12% of patients Conclusion: MPA is an effective and relatively safe treatment option for RA-ILD, with 70% of patients achieving stabilization or improvement in lung function.
Cardiovascular and Cerebrovascular Outcomes in Anti-neutrophil Cytoplasmic Antibody-Associated Vasculitis
Authors: Xiao Shiyu , Xie Wenhui 
Published: November 2024

Background: Cardiovascular and cerebrovascular outcomes in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) remain elusive, especially for some specific cardiovascular and cerebrovascular disease (CCVD). We aimed to quantify the magnitude of the risk of total and type-specific CCVD in AAV population. Method: Electronic database searches of PubMed, EMBASE and Cochrane Library plus a hand search of conference proceedings were performed. Observational studies reporting data on CCVD in AAV patients were eligible. Pooled risk ratios (RR) with 95% confidence intervals were calculated. The primary outcome was the risk of CCVD, CAD and CVA in AAV population. The secondary outcomes were the risk of cause-specific CCVD, including myocardial infraction, angina pectoris, heart failure, stroke, ischemic stroke. Results: Fourteen studies met the inclusion criteria, comprising 20,096 AAV patients (over 46,495 person-years) with 5,757 CCVD events. Compared with non-vasculitis population, AAV patients showed an 83% increased risk of incident CCVD (1.83 [1.37-2.45]; n=10), 48% for coronary artery disease (1.48 [1.26-1.75]; n=9), and 56% for cerebrovascular accident (1.56 [1.22-1.99]; n=9). For type-specific CCVD, the risks of myocardial infarction, stroke, heart failure were increased by 67% (1.67 [1.29-2.15]; n=6), 97% (1.97 [1.19-3.25]; n=8) and 72% (1.72 [1.28-2.32]; n=4), whereas there was only a trend toward a higher risk of angina pectoris (1.46 [0.90-2.39]; n=2), and ischemic stroke (1.88 [0.86-4.12]; n=4). Subgroup analyses by AAV type found significantly increased CCVD risk in both granulomatosis with polyangiitis (1.87 [1.29-2.73]; n=7) and microscopic polyangiitis (2.93 [1.58-5.43]; n=3). In three studies reporting impact of follow-up period after AAV diagnosis, the CCVD risk was significantly higher in the first two years after diagnosis than the subsequent follow-up (2.23 [2.00-2.48] vs. 1.48 [1.40-1.56]; p < 0.01). Significant heterogeneity existed in the main analyses. Conclusions: This meta-analysis demonstrates that AAV is associated with increased risks of overall and type-specific CCVD, especially within two years after AAV diagnosis.
Association Between Triglyceride-glucose Index and Carotid Atherosclerosis in Patients with Systemic Lupus Erythematosus: A Cohort-based Study
Authors: Xie Wenhui , Zhang Zhuoli 
Published: November 2024

Background: Individuals with systemic lupus erythematosus (SLE) patients confer substantial increased risks of cardiovascular complications that have become the primary cause of premature mortality worldwide. We aimed to investigate the association between triglyceride-glucose (TyG) index and carotid atherosclerosis in patients with systemic lupus erythematosus (SLE). Methods: This cross-sectional study in a tertiary hospital included 333 consecutive SLE patients receiving carotid ultrasonography with integrated TyG index, calculated as ln [fasting triglycerides (mg/dL) × fasting glucose (mg/dL)/2]. The subjects were categorized into three groups according to the TyG index tertiles. Logistic regression models were used to assess the association of TyG index with carotid atherosclerosis and carotid artery plaque. Fully adjusted model included age, gender, body mass index, comorbidities, low-density lipoprotein cholesterol and SLE-related variables. Results: There were 10.5% (35/333) of participants identified as having carotid atherosclerosis. Patients with carotid atherosclerosis had significantly higher TyG index as compared with those without (8.77 ± 0.45 vs. 8.45 ± 0.49, p [Formula: see text] 0.001). The frequency of carotid atherosclerosis was increased with increases in TyG index tertiles (3.6% for tertile 1, 10.0% for tertile 2, 17.7% for tertile 3; p = 0.003). Multivariate logistic analyses showed that each 1-unit increase in TyG index was significantly associated with prevalent carotid atherosclerosis (unadjusted OR 4.07 (1.87-8.87); fully-adjusted OR 4.88 (1.74-13.69)). The unadjusted and fully-adjusted OR for occurrence of carotid atherosclerosis were 5.70 (1.88-17.28) and 5.88 (1.66-20.74) in patients with tertile 3 compared to patients with tertile 1 of TyG index (P for trend 0.006 and 0.019, respectively). Similar findings were noticeable for the outcome of carotid artery plaque. Conclusions: The present study suggested that an elevated TyG index was associated with a higher risk of carotid atherosclerosis, independent of traditional cardiovascular risk factors and SLE-related factors. TyG index may be a promising and accessible atherosclerotic indictor for SLE population.