bluuebunny/crossref_metadata_2025_split huggingface dataset:
Incidence of ANA Positivity After the Administration of Biologicals
Authors: Gopal Manikandan , Mathew John 
Published: November 2024

Background: Antinuclear antibodies (ANA) are often used in diagnosing autoimmune rheumatological diseases, but their behavior in response to biological therapies, particularly in Asian patients, is not well-documented. The impact of these therapies on ANA status is crucial for understanding disease management and treatment efficacy. This study aims to evaluate the prevalence and significance of ANA positivity following the administration of biological agents in patients with autoimmune rheumatological diseases. Methods: This pilot study was a prospective cohort study involving 100 patients with autoimmune rheumatological diseases who were initially ANA negative at baseline. Predominantly included patients were axial spondyloarthritis (AxSpA) (n=95) and ANCA vasculitis (n=5). Patients with AxSpA were diagnosed according to the 2009 ASAS criteria, and treated with biological agents (e.g., TNF inhibitors such as adalimumab, etanercept, or IL-17 inhibitors such as secukinumab) and followed at a tertiary care center in India from January 2021 to December 2023. ANA levels were measured before and after 3, 6, 9, or 12 months of biological therapy. Changes in ANA positivity, clinical outcomes, and correlations with disease activity were analyzed. Results: Out of the 100 patients enrolled, 67 (67%) completed the study. All participants were ANA negative at baseline. After biological therapy, ANA positivity developed in 4 patients (5.9%). Despite this increase in Ana positivity, there was no significant correlation between ANA positivity and clinical disease activity, as measured by the Ankylosing Spondylitis Disease Activity Score (ASDAS) and the visual analog scale (VAS) for pain, BVAS suggesting effective therapeutic response despite changes in ANA status. Additionally, 30 patients were lost to follow-up, highlighting challenges related to the financial sustainability of biological therapy in the resource limited countries. Conclusion: Biological therapies for AxSpA are associated with an increase in ANA positivity although this change does not appear to impact clinical disease activity. The rise in ANA titres may reflect disease-related changes or treatment effects rather than a direct correlation with therapeutic efficacy. Further longitudinal studies are needed to understand the clinical significance of these findings and to refine the interpretation of ANA results during biological therapy.
Global Burden of Inflammatory Bowel Disease Among Adolescents and Young Adults in 204 Countries and Territories, from 1990 to 2021: Results from the Global Burden of Disease Study 2021
Authors: Wang Song , Zhao Xiangyun , Wan Xinjian 
Published: November 2024

Background Inflammatory bowel disease (IBD) is a significant component of the global disease burden, particularly among adolescents and young adults. This study aimed to investigate the patterns and trends of IBD among adolescents from 1990 to 2021. Methods We retrieved data on the incidence and disability-adjusted life years (DALYs) of IBD in adolescents (aged 15-39 years) from the Global Burden of Disease (GBD) 2021 study, Temporal trends of incidence and DALYs were calculated using the average annual percent change (AAPC). The correlation between age-specific incidence rates and DALYs rates with the Sociodemographic Index (SDI) was performed using Spearman’s correlation analysis. Results From 1990 to 2021, age-specific incidence rates significantly increased while age-specific DALYs rates declined [AAPC: 0.22vs-0.16]. All SDI regions saw a decline in age-specific incidence rates while age-specific DALYs rates in adolescent showed an increasing trend in low and middle SDI regions but a-decrease in high, high-middle and low-middle SDI regions. Incidence rates rose from 1990 to 2021 in 21-GBD regions except for high-income North America. In contrast, DALYs rates increased in regions such as Western Sub-Saharan Africa, Central Latin America, Tropical Latin America, Australasia, Eastern Sub-Saharan Africa, Central Sub-Saharan Africa, Central Asia, and North Africa and the Middle East, while they declined in the remaining 21-GBD regions. Among the 204-countries, China exhibited the fastest-growing age-specific incidence rate among adolescents [AAPC: 2.60], followed by Libya (AAPC: 2.59) and Oman (AAPC: 1.56), while Italy showed the fastest decline [AAPC: -0.76] (Figure-1A). Mauritius experienced the fastest growth in age-specific DALYs rates among adolescents (AAPC: 1.93), followed by Libya (AAPC: 1.79) and Mexico (AAPC: 1.45), while Northern Mariana Islands saw the fastest decline (AAPC: -3.28) (Figure-1B). Moreover, the AAPC of age-specific incidence rates for adolescent IBD exhibited a slight decreasing trend with increasing SDI (Figure-1C), and the AAPC of DALYs rates significantly declined with increasing SDI (Figure-1D). Conclusion The regional disparities in the incidence and DALYs rates of adolescent IBD highlight the urgent need for innovative prevention and healthcare strategies to alleviate the global burden of IBD among adolescents. Early screening could be crucial in mitigating the impact of IBD on adolescents.
An Observational Cohort Study to Estimate the Durability of Humoral Immune Responses to Vaccination with ChAdOx1 nCoV-19 and the BBV152 in Adult Patients with Autoimmune Rheumatic Diseases (AIRD)
Authors: Kumar Ravi , Madhavan Ramya , Mathew John , Mathew Ashish , Goel Ruchika 
Published: November 2024

Background The durability of immune responses after vaccination against SARS CoV-2 in patients with autoimmune rheumatic diseases (AIRD) is not well studied. Objective We aimed to determine the longevity of humoral immune response to homologous vaccination with 2 doses of ChAdOx1 nCoV-19 and BBV152 in adult patients with AIRD. Methods In this prospective observational study, patients aged 18 to 60 years, diagnosed with SLE, primary systemic vasculitis and inflammatory arthritis (IA) who were scheduled to or had received vaccination with either ChAdOx1 nCoV-19 or BBV152 were recruited between June 2021 to March 2023. IgG antibodies against SARS CoV-2 spike protein (anti-S) were estimated in serum or plasma of patients prior to vaccination if feasible (T0), 15 to 28 days (T1), 75-100 days (T2), 170-190 days (T3), 240-290 days (T4), 340-370 days (T5) after receiving Vx2 by Diasorin chemiluminescence assay. The association of antibody titers with baseline parameters is estimated using chi-square or Mann Whitney tests using SPSS 16. Results Overall, 118 patients (93 females; age: 37.1, IQR: 28- 45 years; disease duration: 60 ((IQR) 29-103) months) with AIRD (SLE:48(40.6%), IA:53(44.9%), systemic vasculitis:7(5.9%) and spondyloarthropathy: 9(7.6%)) were recruited. Baseline demographic data has been depicted in (Table 1). The median antibody titer did not differ significantly between the two vaccines at T1 while the titers at T2, T3 and T4 were significantly lower for those vaccinated with BBV 152 (Table 2). The steroid dose prior to T1 and T2, intake of DMARDs in peri-vaccination period, type of AIRD (IA vs CTD) was not associated with significant difference in antibody titers at T1 and T2. Conclusion The anti-S humoral immune response persisted beyond 1 year for most of our patients with AIRD. After 3 months of vaccination, these responses were higher for ChAdOx1 nCoV-19 as compared with BBV152 in our patients with AIRD.
Unveiling the Enigma: A Challenging Diagnostic Journey to Oculocutaneous Sarcoidosis
Authors: Kulasinghe Udari , Jagoda Jayathri 
Published: November 2024

Introduction Sarcoidosis is a complex, multisystem disorder characterized by the formation of non-caseating granulomas in various organs, most commonly the lungs and lymph nodes. Ocular involvement is frequent, occurring in up to 78% of patients, while skin manifestations are seen in approximately 25–35% of patients. Here we present a case of a 13-year-old boy with generalized body pain that later evolved to oculocutaneous sarcoidosis. Case Report A 13-year-old Sri Lankan boy presented in 2021 with severe generalized body pain, bilateral cervical lymph node swelling, occasional fever, and elevated inflammatory markers. Initial investigations for infections, malignancies, and autoimmune diseases were inconclusive. Lymph node biopsy suggested toxoplasmosis, but serological tests were normal. He later developed left eye visual blurring and periorbital swelling, with MRI showing left optic neuritis and orbital soft tissue oedema. Treatment with IV Methylprednisolone and oral prednisolone resolved his symptoms but was lost to follow-up. A year later, he presented with transient self-resolving subcutaneous nodules over trunk and limbs. due to its transient nature. ANA, RF, ACE level, serum Calcium, serum protein electrophoresis and IgG4 levels were negative. He was started on steroids, Methotrexate, and Infliximab assuming the possibility of autoinflammatory syndrome or sarcoidosis. In 2024 he again developed bilateral submandibular and cervical lymphadenopathy with acute right side visual blurring. MRI showed right optic neuritis and lacrimal gland swelling. (Figure 1) Lymph node Biopsy confirmed non-caseating granulomas. (Figure 2). Based on the findings, a diagnosis of oculocutaneous sarcoidosis was made. Treatment with steroids, methotrexate and Adalimumab was initiated, resulting in a remarkable response. Conclusion Sarcoidosis should be considered as a differential diagnosis in cases of optic neuritis with lymphadenopathy and cutaneous manifestations. Diagnosis of oculocutaneous sarcoidosis is challenging and often requires a multidisciplinary approach involving dermatologists, ophthalmologists, and rheumatologists.
Infection - The Comrade of Acute Pancreatitis in Systemic Lupus Erythematosus: A Case Series from Single Tertiary Centre in India
Authors: Haque Israrul , Ghosh Parasar , Sircar Geetabali , Ghosh Biswadip , Sinhamahapatra Pradyot 
Published: November 2024

Introduction: Acute Pancreatitis in systemic lupus Erythematous is rare but has high mortality. Incidence varies from 1-5% and reported mortality is 3-37.04% with mortality being higher in juvenile SLE. Objective: Study of Clinical and serological profile of SLE patients presenting with acute pancreatitis. Method: Retrospective data of SLE patients visiting our institution was collected. Diagnosis was considered if patients had clinical + biochemical and radiological evidence of pancreatitis. Patients with diagnosis of gallstone, history of alcohol intake, serum Triglycerides>500 mg/dl, serum calcium>10.5 mg/dl and taking Azathioprine at the time of diagnosis were excluded. Results: 9 patients fulfilled inclusion and exclusion criteria. 7 had severe and 2 had moderately severe acute pancreatitis. Female: Male ratio was 8:1, Median age was 25(15-50) years, Median disease duration 12 months (3-48), 4(44.45%) presented within 1 year of disease onset. Mean SELENA-SLEDAI at the time of presentation was 26(19-41). 4(44.45%) were on corticosteroids at the time of presentation. Other active domains at the time of diagnosis were Nephritis in 7(77.78%), NPLE in 3(33.34%), Myositis in 4(44.45%), Autoimmune haemolytic anaemia in 2 (22.22%), Macrophage activation syndrome in 4 (44.45%) enteritis in 1 (11.11%) Myocarditis in 2 (22.22%) and Mucocutaneous in 9 (100%) patients. All patients had elevated Serum Amylase and Lipase level with Median serum Amylase being 545 Units/Litre (normal[Formula: see text]176) and median serum Lipase being 511 ([Formula: see text]160) Units/litre. 6(66.67%) had hypocalcemia and 5 (55.56%) had hypertriglyceridemia. Raised dsDNA was seen in 9(100%, median 277 IU/ml) and hypocomplementemia was seen in 7(77.78%, Median C3 value 49 mg/dL)). None of the patients had associated antiphospholipid syndrome but persistent antiphospholipid antibodies were present in 5(55.56%)-4 had lupus anticoagulant and 1 had Anticardiolipin antibody. 6(66.67%) patients had microbiologically confirmed infection the time of diagnosis of acute pancreatitis (4 bacterial and 1 fungal) among which 3 had lower respiratory tract infection, 1 had urinary tract infection, 1 had pseudomembranous colitis and 1 had peri-pancreatic abscess. 7 patients received only corticosteroids, and 2 patients received IVIG with corticosteroids. 3 out of 9 patients (33.34%) expired due to multi-organ failure. Out of 6 surviving patients all received IV cyclophosphamide (NIH protocol) and are doing fine. Conclusion: Acute pancreatitis in SLE has high mortality probably due to associated high clinical and serological disease activity and high incidence of concomitant infection. Extensive investigation for underlying infection (bacterial, viral and fungal) should be done in SLE patients presenting with acute pancreatitis for comprehensive management.
Clinical Profile and Outcome of Pulmonary Hypertension With and Without Interstitial Lung Disease in Scleroderma
Authors: Jacob Manna , Mathew Ashish , Goel Ruchika , Mathew John 
Published: November 2024

Background: Scleroderma associated pulmonary arterial hypertension and interstitial lung disease (ILD) with pulmonary hypertension are considered to be separate pathological processes with different approach to management. In this study, we looked at the demographics, clinical features, and outcome of patients with both type of pulmonary hypertension in systemic sclerosis Methods: Patients diagnosed with scleroderma and with pulmonary hypertension as per transthoracic ECHO in the time period between 2010 and 2023 were retrospectively identified enrolled in the study. They were divided into two groups-isolated PAH and ILD-PH. ECHO parameters, clinical data were recorded Results: There were 800 patients with a diagnosis of systemic sclerosis during this period. In total, 49 incident scleroderma PH cases were identified (6.13%); 25 with diffuse cutaneous SSc (51.02%), 19 with limited cutaneous SSc (38.78%), and 5 with sine scleroderma (10.2%). Among the study group, 39 (79.59%) patients had ILD with PH and 10 (20.4%) had isolated PH (Table 1). Though the baseline ECHO parameters were similar in both the groups, features of severe PH - Right atrial and ventricular dilatation with Right ventricular dysfunction was more in Isolated PH than ILD-PH (50% vs 23%). Other clinical features were similar in both the groups (Table 2). Isolated PH was treated with vasodilatory therapy and all patients with ILD -PAH were treated with immunosuppression along with vasodilatory therapy. Follow up of ECHO parameters of the two groups at the end of 2 years showed better improvement in the ILD-PH group. Unsatisfactory long-term outcome as defined by clinical NYHA progression, hospitalization and need for long term oxygen therapy was similar in both isolated PH and PH-ILD (40% vs 41%). 1 year survival was 95.9% (2/49 lost to follow up). Conclusions: The prevalence of Pulmonary Hypertension in our scleroderma cohort of a tertiary care center was 6.13%. ILD-PH was 4 times more prevalent than isolated PH.46.94% of all our PH patients had it at presentation. The one-year survival of our cohort was 95.9% almost equivalent to other international cohorts
Pachydermoperiostosis (Touraine-Solente–Gole Syndrome): A Case Report of Primary Hypertrophic Osteoarthropathy
Authors: Islam Asif , Shahid Kinza , Khurshid Fatima , Khalood Memoona 
Published: November 2024

Background: Pachydermoperiostosis (PDP), or primary hypertrophic osteoarthropathy (PHO), also known as the Touraine–Solente–Gole syndrome, is a genetic disorder that is uncommon and is identified by finger clubbing, skin thickening, and periosteal growth. Case Report: This case study details the case of a 21-year-old man with PDP to raise awareness, improve diagnosis, and enhance management strategies for the condition. The individual showed common signs like digital clubbing, pachydermia, and periostosis, as well as related symptoms like hyperhidrosis. Radiological imaging supported the diagnosis by revealing periosteal reactions and cortical thickening in multiple bones. Other conditions with comparable clinical characteristics were considered in the differential diagnosis, however, the diagnosis of PHO was confirmed by the specific radiological results and normal hormonal levels. The treatment primarily targets alleviating symptoms with drugs like NSAIDs and corticosteroids, along with newer options such as bisphosphonates. Conclusion: Timely detection and correct treatment are essential to enhance the well-being of people with PHO. This case study emphasizes the significance of tracking symptoms and offering thorough care to those with PDP/PHO.
Treatment Escalation of JIA in Local Setting: Timing, Role of Disease Activity Index
Authors: Ho Assunta Chi Hang , Kwong Hiu Ching , Chan Oi Man 
Published: November 2024

Introduction The paradigm of managing JIA has been changing. There is ongoing discussion whether using bDMARDs early in the treatment course is superior to sequential use after cDMARDs1. On the other hand, with a treat to target approach, it is shown both approaches achieve comparable outcome2. We aim to investigate the real life experience of a single-centre JIA cohort regarding treatment escalation, including the timing and the role of disease activity index. Method A chart review on all the JIA attending the Paediatric Rheumatology Clinic at the Prince of Wales Hospital between JUL 2023 to AUG 2024 was performed. SJIA was excluded as it follows different treatment algorithm. Outcome of interests are time to cDMARDs or bDMARDs from first clinic visit, time to escalation to cDMARDs and bDMARDs, disease activity indexes (cJADAS, BASDAI and BASFI for ERA) at the time of treatment escalation. Results are presented in descriptive analysis. Result Total 17 cases with 30 treatment escalation events were recorded. The median age at last FU was 15 years old (IQR 9.5-18.5), with a median duration of FU of 51 months (IQR 25-79.5). For the whole group the median time from first clinic visit to cDMARDs was 1 month (IQR 0-26), to bDMARDs was 12 months (IQR 5-33). 3 cases required further switch. The median time to 1st treatment escalation was 1 month (IQR 0-26). The median time between 1st and 2nd treatment escalation was 4.5 months (IQR 3-17). These usually involved change from cDMARDs to bDMARDs. cJADAS scores were recorded at the time of switch in 23 events. The median score at each treatment escalation was 14 (IQR 8.9-18). It signified high or moderate disease activity in oligoarticular or polyarticular course JIA, respectively. For the eight ERA, the median time to first cDMARDs was 6 months (IQR 0-28). It was 17 months (IQR 3-29) to next treatment, mostly bDMARDs. Regarding disease activity index at the time of switch, the median cJADAS of 13 events was 11.2 (IQR 6.5-14), ie moderate disease activity, while the median BASDAI of 14 events was 2.55 (IQR2.07-4.4), ie not active disease. Conclusion Overall the timing of cDMARDs use, treatment switch and performance of cJADAS were inline with other centres. In this small cohort BASDAI is not optimal in terms of indicating treatment switch in ERA. 1 Kimura Y et al. Arthritis Rheumatol. 2021 Oct;73(10):1898-1909. 2 Hissink Muller P et a1. Ann. Rheum. Dis. 2019;78:51–59
Final Outcome Analysis of Hospitalized Patients with Suspected NPSLE: A Retrospective Single-Center Study
Authors: Fang Wei Ting 
Published: November 2024

Background: Neuropsychiatric systemic lupus erythematosus (NPSLE) is a serious complication in SLE, and distinguishing these manifestations from other neuropsychiatric disorders is challenging. Method: This was a retrospective single-center study. NP manifestations were attributed to SLE according to clinical experts’ opinions. Demographic and clinical data were obtained in patients with and without NP manifestations attributed to SLE. And antibodies profiles were included for hierarchical cluster analysis, analyze the correlation between different antibody clusters and clinical manifestations of SLE. Results: The final analysis group included 176 patients, of which 151 (85.8%) had NP manifestations attributed by SLE. 1, The positive rates of anti-nuclear antibodies (ANA) and anti-SSB of NPSLE patients and the levels of immunoglobulin G (IgG) were significantly higher in comparison with those NP symptoms not attributed to SLE (non-NPSLE) patients [145 (96.0%) vs 18 (72.0%), P [Formula: see text] 0.001; 23 (15.2%) vs 0 (0.0%), P=0.048; median 23.5 vs 9.6 points, IQR 10.00, 48.25 vs 7.58,15.83 points, P [Formula: see text] 0.01; respectively]. The levels of C4 and the proportion of patients with concomitant hemolytic anemia were lower than non-NPSLE [median 0.09 vs 0.15 points, IQR 0.05, 0.14 vs 0.07, 0.17 points, P [Formula: see text] 0.01; 9 (6.0%) vs 7 (28.0%), P [Formula: see text] 0.005; respectively]; 2, NP manifestations in the central nervous system were the most frequent (86.9%). In patients with NPSLE, cerebrovascular disease, seizures, headche occurred most often; Among non-NPSLE, common causes included infection, non-specific cerebrovascular disease, hematological disease; 3, Four clusters among 151 NPSLE patients were identified. Cluster 1 comprised patients with triple aPLs (antiphospholipid antibodies) positivity and demonstrates a high incidence of cerebrovascular disease, seizure, Cluster 2 was characterized by high positive rates of anti-SSA and anti-SSB, with high risk of system damage such as mucocutaneous, renal. Cluster 3 was characterized by a high positive rate of anti Sm, mainly present with aseptic meningitis and polyneuropathy, often accompanied by renal and hematological involvement. Cluster 4 was characterized by high positive rates of anti Ro52, anti RNP (ribosome P protein), includes seizure and demyelinating syndrome. Conclusion: Four characterized antibody clusters are identified in NPSLE patients in this study. Different clusters are associated with certain clinical features and complications of NPSLE. However, the correlations found in this study need to be investigated further in larger populations.