{"title":"Clinical Characteristics, Predictors for Mortality and Comparison of the Birmingham Vasculitis Activity Score and the Five-Factor Score on Survival in ANCA-Associated Vasculitis in Hong Kong","authors":"Joshua Ka Ho Yeung, Joyce Kit Yu Young","doi":"10.1142/s2661341724500020","DOIUrl":"https://doi.org/10.1142/s2661341724500020","url":null,"abstract":"Objective: To describe the clinical profile and predictors of mortality of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) patients in Hong Kong. To compare the accuracy of the latest Five-Factor Score (FFS-2009) and the Birmingham Vasculitis Activity Score (BVAS) in prediction of survival with this local cohort. Methods: A retrospective observational study on newly diagnosed AAV patients, from January 1, 2011 to March 31, 2022, managed in the Kowloon West Cluster (KWC) hospitals in Hong Kong. Demographic and baseline characteristics, clinical profile, and treatment profile were reviewed. Factors associated with mortality were analyzed with the Cox proportional hazards model. The performances of FFS and BVAS in mortality prediction were analyzed by receiver operating characteristic (ROC) curves. Results: A total of 83 AAV patients were included in the study. The median age was 70.5 years at diagnosis. Microscopic polyangiitis (MPA; 69.9%) was the most common AAV subtype. The median FFS and BVAS were 2 and 20, respectively. The overall mortality was 45.6% across the study period. Multivariate Cox regression identified age at diagnosis (HR 1.043, [Formula: see text]), stabilized peak serum creatinine (HR 1.002, [Formula: see text]), hemoglobin level (HR 0.754, [Formula: see text]), cardiac involvement (HR 3.862, [Formula: see text]), and use of maintenance therapy (HR 0.261, [Formula: see text]) as independent predictors of overall survival. Both FFS and BVAS were significant predictors of overall survival. The areas under the curve (AUC) of ROC curves suggested FFS was a good prediction tool for early mortality in 1 year, with an AUC value of 0.874. Conclusion: Despite the advances in treatment, AAV still carried significant morbidities with high mortality. Clinical predictors and existing scoring systems showed good predictive power on mortality.","PeriodicalId":15538,"journal":{"name":"Journal of Clinical Rheumatology and Immunology","volume":" January","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141824280","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
L. Luk, Peter Kei Tat Hui, I. Tang, V. Wong, S. Pang, V. W. Lao, Hoch So
{"title":"Subclinical Coronary Artery Disease in Patients with Idiopathic Inflammatory Myopathy: An Evaluation by CT Coronary Angiography","authors":"L. Luk, Peter Kei Tat Hui, I. Tang, V. Wong, S. Pang, V. W. Lao, Hoch So","doi":"10.1142/s2661341724500019","DOIUrl":"https://doi.org/10.1142/s2661341724500019","url":null,"abstract":"Idiopathic inflammatory myopathy (IIM) poses elevated risk of cardiovascular event and mortality, similar to other autoimmune rheumatic diseases. We conducted a cross-sectional study to examine the prevalence and risk factor of subclinical coronary artery disease (CAD) in patients with IIM, using CT coronary angiogram (CTCA). The prevalence of obstructive CAD and CAD in IIM (13.3% and 66.7%, respectively) were significantly higher than age and sex-matched controls (0% and 30%, respectively, both [Formula: see text]). Diabetes mellitus and calcium calcification score [Formula: see text] units were found to be the independent predictors of obstructive CAD. Screening of high-risk patients with aggressive treatment of cardiovascular risk factors should be considered in managing IIM patients.","PeriodicalId":15538,"journal":{"name":"Journal of Clinical Rheumatology and Immunology","volume":"12 4","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-07-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141661566","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A Case of Polyarteritis Nodosa and Antiphospholipid Antibody Positivity Presenting with Intramuscular Haematoma","authors":"Cheryl Chun Man Ng","doi":"10.1142/s2661341723720021","DOIUrl":"https://doi.org/10.1142/s2661341723720021","url":null,"abstract":"Polyarteritis nodosa (PAN) is a medium-sized vessel vasculitis often presenting report as both stenotic and aneurysmal lesions. Association with antiphospholipid antibody (aPL) has also been described. Here, we reported a case of PAN with history of cutaneous vasculitis presenting as multiple territory ischemic stroke, mesenteric panniculitis, coronary artery stenosis on imaging; lupus anticoagulant (LA) was also identified, patient was treated with immunosuppressants and anticoagulation. Disease course was, however, complicated by development of thigh haematoma. Clinical manifestation of PAN and its association with aPL will be discussed.","PeriodicalId":15538,"journal":{"name":"Journal of Clinical Rheumatology and Immunology","volume":"44 21","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-01-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139387004","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Hong Kong Guangdong Rheumatology Meeting","authors":"Ho So","doi":"10.1142/s2661341723740048","DOIUrl":"https://doi.org/10.1142/s2661341723740048","url":null,"abstract":"The availability of biological or targeted synthetic disease-modifying anti-rheumatic drugs (b/tsDMARDs) has revolutionized the treatment of rheumatoid arthritis (RA). Given the changing landscape of RA management with the ever-expanding armamentarium of advanced therapeutic agents, guidelines are important to provide clinicians with recommendations for decisions frequently faced in clinical practice. To reflect the latest developments in RA research, the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR) updated their RA management guidelines in 2021 and 2022, respectively. While the general principles of management are largely similar, there are a few divergent recommendations. Two circumstances of particular attention include the polarized view on systemic glucocorticoid bridging and the position of Janus kinase (JAK) inhibitors in the RA treatment cascade. On the other hand, the EULAR recommendations might appear more directly applicable, whereas the ACR counterpart provides treatment guidance in special at-risk populations. In this presentation, the two latest recommendations from ACR and EULAR will be compared, highlighting the salient differences. The supporting literature will also be discussed, including the latest studies attempting to address the controversies in the management of patients with RA.","PeriodicalId":15538,"journal":{"name":"Journal of Clinical Rheumatology and Immunology","volume":"41 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139293578","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Abstract 7 — Deep Learning Differentiation of Inflammatory Lesions in Sacroiliac Joint MRI Based on Spondyloarthritis Research Consortium of Canada (SPARCC) System","authors":"Ho Yin Chung, S. C. Chan, Yingying Lin, Peng Cao","doi":"10.1142/s2661341723740231","DOIUrl":"https://doi.org/10.1142/s2661341723740231","url":null,"abstract":"Objective To develop a deep learning algorithm for grading sacroiliitis based on SPARCC in magnetic resonance imaging (MRI). Method A total of 996 images with inflammatory lesions from 210 participants with MRI sacroiliitis were used for training and validation. The testing cohort consisted of 18 participants with and 19 without MRI sacroiliitis. One hundred and fifty four images from the testing cohort had inflammatory lesions identified by a pre-trained algorithm from our previous study[1]. The ground truth was defined by manually outlined regions of interests (ROIs) consisting of bone marrow edema (BME) at the sacroiliac joint. The performance of the deep learning pipeline in predicting the SPARCC score was compared to manual interpretation by two experienced readers. Result The intra-observer reliability and the Pearson coefficient between the SPARCC scores from two experienced readers and the deep learning pipeline were 0.83 and 0.86, respectively. The sensitivities in identifying all inflammatory lesions, deep lesions, and intense lesions were 0.83, 0.79 and 0.81, respectively. The Dice coefficients of the sacrum and ilium segmentation were 0.82 and 0.80, respectively. The accuracies of identifying the SI joint and reference vessel were 0.90 and 0.88, respectively. Conclusion The performance of AI algorithms in SPARCC scoring was compatible with manual scoring by experienced readers. This proposed deep learning pipeline could be the first demonstration of a complete and SPARCC-informed deep-learning approach in scoring STIR images in SpA.","PeriodicalId":15538,"journal":{"name":"Journal of Clinical Rheumatology and Immunology","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139295954","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Symposium 4","authors":"Shirley Chan","doi":"10.1142/s2661341723740139","DOIUrl":"https://doi.org/10.1142/s2661341723740139","url":null,"abstract":"Extra-articular manifestations are common in Rheumatoid-arthritis (RA), involving the skin, eyes, heart, and lung, causing significant comorbidities. In particular, clinically significant rheumatoid arthritis-associated interstitial lung disease (RA-ILD) has a prevalence of about 10%-19% among RA patients. However, the exact prevalence of ILD in RA patients is not well known and there is also a lack of local data in Hong Kong. Early screening and identification of ILD in RA patients is important for improving patient outcomes. Currently, there is no established algorithm for screening in asymptomatic patients with RA. High resolution computed tomography (HRCT) is a standard assessment in ILD diagnosis but is associated with high cost. Pulmonary function test and lung ultrasound might also be useful in identifying ILD. Besides, multiple clinical risk factors and novel biomarkers have been explored for early identification of RA-ILD. To evaluate the usefulness of these predictors and to evaluate the burden of interstitial lung disease (ILD) among patients with RA, a local study (RAISE, Rheumatoid Arthritis-associated ILD: Screening and Evaluation in high-risk patients) was designed in Hong Kong to assess the prevalence of RA-ILD among RA patients with high risk, and to identify potential clinical and biochemical markers associated with the condition. In this session, Dr. Shirley Chan will share the protocol of the RAISE study and the screening approach. The interim results will also be analyzed and presented.","PeriodicalId":15538,"journal":{"name":"Journal of Clinical Rheumatology and Immunology","volume":"5 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139300157","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Symposium 4","authors":"Vanessa Smith","doi":"10.1142/s2661341723740127","DOIUrl":"https://doi.org/10.1142/s2661341723740127","url":null,"abstract":"Interstitial lung disease (ILD) is a fibrotic disease of the lung parenchyma. It can occur in different connective tissue diseases, including rheumatoid arthritis (RA). Smoking, male gender and longstanding RA are possible risk factors for developing ILD 1 . Being a common extra-articular manifestation of RA, it can contribute to decreased quality of life, chronic disability, high utilization of healthcare resources, and may also lead to substantial morbidity and mortality for affected patients1. Hence, early identification and management is of paramount importance to improve patient outcomes. Clinical presentation, chest X-ray, pulmonary function testing and high-resolution computed tomography are common tools for investigation and they also allows assessment of subtype and disease extent 2 . The histopathologic and radiographic features of RA-ILD are heterogeneous. The most frequent patterns of RA-ILD are usual interstitial pneumonia (UIP) and nonspecific interstitial pneumonia (NSIP). Distinguishing the patterns is useful in predicting prognosis and would also affect subsequent management approach 2 . Traditionally treatment initiated for RA-ILD was generally empirical. Corticosteroids were often used as first-line agents and immunosuppressants maybe added2. However, these treatments are not specific and mainly target inflammation instead of fibrosis. With the advance in medicine, antifibrotic is now indicated for treating fibrosing ILD with progressive phenotype. FVC decline can be slowed in patients with connective tissue disease associated progressive ILD 3 . In this lecture, Prof. Vanessa Smith will share the current knowledge and evidence in RA-ILD. The approach on identifying and screening ILD in RA patients would be presented. The management strategy and options would also be reviewed.","PeriodicalId":15538,"journal":{"name":"Journal of Clinical Rheumatology and Immunology","volume":"27 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139300965","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"MRI Workshop for Axial Spondyloarthritis 2023","authors":"Victor Lee","doi":"10.1142/s2661341723740061","DOIUrl":"https://doi.org/10.1142/s2661341723740061","url":null,"abstract":"MRI sacroiliac joint is a sensitive imaging modality for evaluation of inflammation and the accompanying structural changes. Assessment of SpondyloArthritis international Society (ASAS) classification criteria incorporates active inflammation of the sacroiliac joint on MRI as one of the criteria of imaging arm of axial spondylarthritis. The current lecture would cover basic imaging anatomy of sacroiliac joints, typical imaging features of sacroiliac inflammation in axial spondyloarthritis and ‘red-flags’ suggesting alternative diagnosis. The supplementary role of structural changes on MRI in aiding diagnosis of axial spondyloarthritis will also be highlighted.","PeriodicalId":15538,"journal":{"name":"Journal of Clinical Rheumatology and Immunology","volume":"182 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139301145","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Xianfeng Yan, Isaac T Cheng, Jacqueline So, Ho So, Ryan Ka Lok Lee, James Francis Griffith, L. Tam
{"title":"Abstract 20 — Performance of Various Definitions for Active MRI: Lesions in Sacroiliac Joint and Spine in Discriminating Patients with Axial Psoriatic Arthritis","authors":"Xianfeng Yan, Isaac T Cheng, Jacqueline So, Ho So, Ryan Ka Lok Lee, James Francis Griffith, L. Tam","doi":"10.1142/s266134172374036x","DOIUrl":"https://doi.org/10.1142/s266134172374036x","url":null,"abstract":"Background Unlike axial spondyloarthritis, no classification criteria exist for axial psoriatic arthritis (axPsA). In 2021, the Assessment of SpondyloArthritis International Society (ASAS) revised the magnetic resonance imaging (MRI) criteria for active sacroiliitis (2021 criteria: bone marrow edema [BME] present in [Formula: see text]4 sacroiliac joint [SIJ] quadrants or [Formula: see text]3 consecutive SIJ slices[1]). This study aimed to compare the utility of this new cut-off in discriminating PsA patients with/without axPsA versus the 2009 ASAS criteria[2] for active-MRI-SIJ (BME [Formula: see text] 2 consecutive slices or [Formula: see text]1 location in a single slice). Methods Consecutive patients who fulfilled the classification criteria for PsA were recruited into this cross-sectional study, regardless of back pain. Sixty-seven patients underwent radiography (including pelvis, cervical/thoracic/lumbar-spine) and MRI-SIJ. Additionally, 47 underwent whole-spine MRI. AxPsA diagnosis was based on clinical information and imaging findings, as determined by an expert rheumatologist and a radiologist, and used as the reference standard. Two independent readers evaluated the MRI images based on two criteria for active sacroiliitis (BME cut-off: [Formula: see text] 4[1] vs [Formula: see text] 2[2]) and spondylitis (BME cut-off: [Formula: see text] 5[3] vs [Formula: see text] 3[4]). The agreement between the two MRI BME cut-offs for active sacroiliitis/spondylitis and the reference standard was evaluated. Results Sixty-seven patients (mean age: 47±12 years, 44 (65.7%) male, psoriasis and PsA disease duration: 13.5±10.3 and 3.8±6.1 years respectively) were recruited (Table 1). Twenty-three (34.3%) were diagnosed with axPsA, including 13 (56.5%) with radiographic sacroiliitis and 10 (43.5%) with non-radiographic axPsA. 12/67 (17.9%) had active MRI-sacroiliitis based on the 2021 ASAS criteria, while 4/47 (8.5%) had spondylitis based on the 2016 proposed definition[3]. Compared with the reference standard, the agreement increased after applying a more stringent threshold to define active sacroiliitis (BME cut-off: [Formula: see text] 4 vs [Formula: see text] 2; Kappa: 0.514 vs 0.392, respectively; Fig. 1A-B). The agreement with the reference standard further increased by applying a more stringent criteria for active spondylitis in addition to active sacroiliitis (BME cut-off for MRI-SIJ and spine: [Formula: see text] 4 and [Formula: see text] 5 vs [Formula: see text] 2 and [Formula: see text] 3; Kappa: 0.717 vs 0.342, respectively; Fig. 1C-D), resulting in higher specificity (active-sacroiliitis: 97.7% vs 81.8%; active-sacroiliitis and/or spondylitis: 100% vs 72.7%) and higher positive predictive value (91.7% vs 61.9%; 100% vs 50.0%, respectively). Conclusion At least four BME lesions on MRI-SIJ and five inflammatory lesions on MRI-spine allow acceptable discrimination of axPsA and no axPsA while assuring [Formula: see text]95% specificity.","PeriodicalId":15538,"journal":{"name":"Journal of Clinical Rheumatology and Immunology","volume":"22 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139291298","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Abstract 15 — Lupus Low Disease Activity State: An Underutilised Clinical Target that is Attainable and Protective against Lupus Nephritis Relapse","authors":"C. Cheung, Chak Sing Lau, S. C. Chan","doi":"10.1142/s2661341723740310","DOIUrl":"https://doi.org/10.1142/s2661341723740310","url":null,"abstract":"Background Lupus nephritis (LN) is a significant comorbidity affecting approximately 50-60% of patients with systemic lupus erythematosus (SLE). Complete and partial renal response (CRR/PRR) have been recommended as treatment targets in LN. Lupus low disease activity state (LLDAS) is also associated with favourable clinical outcomes. This study aims to investigate the LLDAS attainment rate and outcomes in patients with LN. Methods Patients with biopsy-proven LN during 2010-2020 in Queen Mary Hospital were included. Baseline demographics, blood parameters and urinalysis results were documented. Renal response and LLDAS attainment were assessed at 12 months after LN diagnosis. CRR was defined as proteinuria [Formula: see text]0.5g/day with a normal estimated glomerular filtration rate (eGFR); PRR was defined as a reduction in proteinuria by [Formula: see text]50% with near normal eGFR. A relapse was defined as a biopsy-proven active LN on histology after an initial treatment response of proteinuria reduction of [Formula: see text]50% or to sub-nephrotic range. Time-to-relapse survival analysis was performed to compare the significance of CRR/PRR and LLDAS attainment. Results 143 LN patients were included, with a median follow-up duration of 10.4 years. At 12 months, 57 (40%), 14 (10%) and 69 (48%) patients achieved CRR, PRR and LLDAS, respectively. Although 39 (27%) patients attained both CRR/PRR and LLDAS, a significant number of 30 (21%) patients reached LLDAS without meeting CRR/PRR (Figure 1). Among 136 patients who achieved the pre-defined treatment response, 30 (22%) patients developed LN relapse after a median of 2.98 years. Patients reaching either CRR/PRR or LLDAS had a significantly lower risk of relapse (CRR/PRR: HR = 0.34, p = 0.02; LLDAS: HR = 0.28, p = 0.003). The attainment of both CRR/PRR and LLDAS was associated with the lowest risk of relapse (Figure 2). Conclusion We advocate LLDAS as a target for LN patients as attaining LLDAS reduces future LN relapse risks.","PeriodicalId":15538,"journal":{"name":"Journal of Clinical Rheumatology and Immunology","volume":"85 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139294187","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}