Pub Date : 2024-07-14DOI: 10.1016/j.semarthrit.2024.152522
Ke Li Chow , Paula Elizabeth Keating , Kamal Solanki , Mark Sapsford , Karen Lindsay , John Liston O'Donnell
Aims
We describe the varied clinical presentations, barriers in diagnosis and outcomes of anti-HMGCR myopathy in a large national cohort.
Methods
Adults found positive for serum anti-HMGCR autoantibodies via line blot or enzyme-immunoassay followed by immunoprecipitation were included in the study.
Results
Of 75 patients identified, the records of 72 (96 %) described weakness as the presenting symptom. The records of 65 gave a reliable description of proximal weakness. In 22/65 (33.8 %) the weakness was described as predominantly or solely lower limb weakness. Forty-five of 75 (60 %) presented with a subacute onset (duration of symptoms >4 weeks -≤6 months), whilst 22/75 (29.3 %) presented with a more indolent chronic onset (duration of symptoms >6 months). Eighteen of 75 (24 %) suffered falls and 2/75 (2.7 %) had “general decline”. In three patients no weakness was described: two presented with myalgia and one with a skin rash characterized as Jessner lymphocytic skin rash. Median creatine kinase at presentation was 7337 U/L (range 1050–25,500). Muscle biopsy was performed in 38 (50.7 %). Associated malignancy was infrequent. Four patients recovered without immunosuppression. Five-year and 10-year survival was 92.7 % (95 % CI 80.6–97.4 %), and 82.5 % (95 % CI 61.2–92.8 %) respectively.
Conclusion
Recurrent falls, a long prodrome and dominant lower limb proximal weakness were common in this anti-HMGCR myopathy cohort. These features overlap with frailty syndrome and sporadic inclusion body myositis emphasizing the importance of considering anti-HMGCR myopathy in that clinical context. A minority of patients recover after statin withdrawal alone.
{"title":"Anti-HMGCR myopathy: Diversity of clinical presentations in a national cohort in New Zealand","authors":"Ke Li Chow , Paula Elizabeth Keating , Kamal Solanki , Mark Sapsford , Karen Lindsay , John Liston O'Donnell","doi":"10.1016/j.semarthrit.2024.152522","DOIUrl":"10.1016/j.semarthrit.2024.152522","url":null,"abstract":"<div><h3>Aims</h3><p>We describe the varied clinical presentations, barriers in diagnosis and outcomes of anti-HMGCR myopathy in a large national cohort.</p></div><div><h3>Methods</h3><p>Adults found positive for serum anti-HMGCR autoantibodies via line blot or enzyme-immunoassay followed by immunoprecipitation were included in the study.</p></div><div><h3>Results</h3><p>Of 75 patients identified, the records of 72 (96 %) described weakness as the presenting symptom. The records of 65 gave a reliable description of proximal weakness. In 22/65 (33.8 %) the weakness was described as predominantly or solely lower limb weakness. Forty-five of 75 (60 %) presented with a subacute onset (duration of symptoms >4 weeks -≤6 months), whilst 22/75 (29.3 %) presented with a more indolent chronic onset (duration of symptoms >6 months). Eighteen of 75 (24 %) suffered falls and 2/75 (2.7 %) had “general decline”. In three patients no weakness was described: two presented with myalgia and one with a skin rash characterized as Jessner lymphocytic skin rash. Median creatine kinase at presentation was 7337 U/L (range 1050–25,500). Muscle biopsy was performed in 38 (50.7 %). Associated malignancy was infrequent. Four patients recovered without immunosuppression. Five-year and 10-year survival was 92.7 % (95 % CI 80.6–97.4 %), and 82.5 % (95 % CI 61.2–92.8 %) respectively.</p></div><div><h3>Conclusion</h3><p>Recurrent falls, a long prodrome and dominant lower limb proximal weakness were common in this anti-HMGCR myopathy cohort. These features overlap with frailty syndrome and sporadic inclusion body myositis emphasizing the importance of considering anti-HMGCR myopathy in that clinical context. A minority of patients recover after statin withdrawal alone.</p></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":null,"pages":null},"PeriodicalIF":4.6,"publicationDate":"2024-07-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141692391","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-07-14DOI: 10.1016/j.semarthrit.2024.152518
Sheryl A. Kluberg , Andrew L. Simon , Sarah M. Alam , Alexander Peters , Casie Horgan , Dongdong Li , Erick Moyneur , Elizabeth Messenger-Jones , Richard Platt , Cheryl N. McMahill-Walraven , Djeneba Audrey Djibo , Kimberly Daniels , Aziza Jamal-Allial , Claire H. Pernar , Najat J. Ziyadeh , Qianli Ma , Mano Selvan , O'Mareen Spence , Driss Oraichi , Harry Seifert , Huifeng Yun
Objective
To assess whether recombinant zoster vaccine (RZV) is associated with an increased risk of new-onset gout among US adults aged ≥50 years.
Methods
We conducted a real-world, retrospective safety study with a self-controlled risk interval (SCRI) design using administrative claims data. We included health plan members aged ≥50 years with RZV exposure, followed by incident gout within 60 days. Days 1−30 following RZV exposure were considered the risk window (RW), and days 31−60 were considered the control window (CW). We estimated the risk ratio (RR) of gout in the RW versus CW, using a conditional Poisson model. The primary analysis estimated the risk of incident gout following any RZV dose. Sensitivity analyses evaluated dose 1- and dose 2-specific risks, risk among patients compliant with recommended dose spacing of 60−183 days, adjustment for seasonality, and restriction to the pre-COVID-19 era (before December 1, 2019).
Results
A total of 461,323 individuals received ≥1 RZV dose; we included 302 individuals (mean age 72.5 years; 66 % male) with evidence of new-onset gout within 60 days in SCRI analyses. A total of 153 (50.7 %) individuals had gout events in the RW and 149 (49.3 %) in the CW (RR 1.03; 95 % confidence interval 0.81, 1.29). All sensitivity analyses had consistent results, with no association of RZV with incident gout.
Conclusion
In a population of US adults aged ≥50 years, there was no statistically significant increase in the risk of gout during the 30 days immediately after RZV exposure, compared with a subsequent 30-day CW.
{"title":"Risk of incident gout following exposure to recombinant zoster vaccine in US adults aged ≥50 years","authors":"Sheryl A. Kluberg , Andrew L. Simon , Sarah M. Alam , Alexander Peters , Casie Horgan , Dongdong Li , Erick Moyneur , Elizabeth Messenger-Jones , Richard Platt , Cheryl N. McMahill-Walraven , Djeneba Audrey Djibo , Kimberly Daniels , Aziza Jamal-Allial , Claire H. Pernar , Najat J. Ziyadeh , Qianli Ma , Mano Selvan , O'Mareen Spence , Driss Oraichi , Harry Seifert , Huifeng Yun","doi":"10.1016/j.semarthrit.2024.152518","DOIUrl":"10.1016/j.semarthrit.2024.152518","url":null,"abstract":"<div><h3>Objective</h3><p>To assess whether recombinant zoster vaccine (RZV) is associated with an increased risk of new-onset gout among US adults aged ≥50 years.</p></div><div><h3>Methods</h3><p>We conducted a real-world, retrospective safety study with a self-controlled risk interval (SCRI) design using administrative claims data. We included health plan members aged ≥50 years with RZV exposure, followed by incident gout within 60 days. Days 1−30 following RZV exposure were considered the risk window (RW), and days 31−60 were considered the control window (CW). We estimated the risk ratio (RR) of gout in the RW versus CW, using a conditional Poisson model. The primary analysis estimated the risk of incident gout following any RZV dose. Sensitivity analyses evaluated dose 1- and dose 2-specific risks, risk among patients compliant with recommended dose spacing of 60−183 days, adjustment for seasonality, and restriction to the pre-COVID-19 era (before December 1, 2019).</p></div><div><h3>Results</h3><p>A total of 461,323 individuals received ≥1 RZV dose; we included 302 individuals (mean age 72.5 years; 66 % male) with evidence of new-onset gout within 60 days in SCRI analyses. A total of 153 (50.7 %) individuals had gout events in the RW and 149 (49.3 %) in the CW (RR 1.03; 95 % confidence interval 0.81, 1.29). All sensitivity analyses had consistent results, with no association of RZV with incident gout.</p></div><div><h3>Conclusion</h3><p>In a population of US adults aged ≥50 years, there was no statistically significant increase in the risk of gout during the 30 days immediately after RZV exposure, compared with a subsequent 30-day CW.</p></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":null,"pages":null},"PeriodicalIF":4.6,"publicationDate":"2024-07-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S0049017224001586/pdfft?md5=032644a2a38a35b39debcbe16c27c772&pid=1-s2.0-S0049017224001586-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141701155","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-07-11DOI: 10.1016/j.semarthrit.2024.152523
Mark Siderius , Freke Wink , Stan Kieskamp , Fiona Maas , Reinhard Bos , Frans G.M. Kroese , Anneke Spoorenberg , Suzanne Arends
Objective
In our prospective cohort with standardized bi-annual measurements of bone mineral density (BMD) and spinal radiographs, we evaluated the long-term course of BMD and the development of radiographic vertebral fractures (VFs) during 8 years of TNFi treatment in patients with radiographic axial spondyloarthritis (r-axSpA).
Methods
Consecutive axSpA patients from the GLAS cohort receiving TNFi for ≥8 years were included. Patients who received anti-osteoporotic treatment were excluded. Lumbar spine (LS) BMD was assessed at baseline, 1 year and bi-annually using DEXA. Radiographic VFs were evaluated using the Genant classification.
Results
126 axSpA patients were included; 75 % male, mean age 42 ± 11 years, ASDAS 3.8 ± 0.8, median LS BMD Z-score -0.5 (IQR -1.4–0.7) and 20 % had radiographic VFs at baseline. Disease activity improved rapidly and sustained. LS BMD Z-score improved significantly up to 4 years compared to the previous time point and sustained thereafter. Median percentage of improvement compared to baseline was 8.9 % (2.8–15.8) and 7.2 % (2.2–14.7) after 4 and 8 years, respectively. Of 90 patients with baseline and 8-year radiographs, 14 (16 %) developed new VFs and 5 (6 %) showed an increase in severity of existing VFs. Of all 44 VFs present at 8 years, 30 % were grade 2 (n = 12) or grade 3 (n = 1).
Conclusion
In r-axSpA patients treated with TNFi for 8 years, LS BMD Z-score increased significantly, especially during the first 4 year of treatment. Radiographic VFs continued to develop or progressed, irrespective of improvement in BMD. Therefore, clinical attention for trabecular bone loss is important in daily clinical practice.
{"title":"Improvement of bone mineral density and new vertebral fractures during 8 years of TNF-α inhibition in patients with axial spondyloarthritis","authors":"Mark Siderius , Freke Wink , Stan Kieskamp , Fiona Maas , Reinhard Bos , Frans G.M. Kroese , Anneke Spoorenberg , Suzanne Arends","doi":"10.1016/j.semarthrit.2024.152523","DOIUrl":"10.1016/j.semarthrit.2024.152523","url":null,"abstract":"<div><h3>Objective</h3><p>In our prospective cohort with standardized bi-annual measurements of bone mineral density (BMD) and spinal radiographs, we evaluated the long-term course of BMD and the development of radiographic vertebral fractures (VFs) during 8 years of TNFi treatment in patients with radiographic axial spondyloarthritis (r-axSpA).</p></div><div><h3>Methods</h3><p>Consecutive axSpA patients from the GLAS cohort receiving TNFi for ≥8 years were included. Patients who received anti-osteoporotic treatment were excluded. Lumbar spine (LS) BMD was assessed at baseline, 1 year and bi-annually using DEXA. Radiographic VFs were evaluated using the Genant classification.</p></div><div><h3>Results</h3><p>126 axSpA patients were included; 75 % male, mean age 42 ± 11 years, ASDAS 3.8 ± 0.8, median LS BMD Z-score -0.5 (IQR -1.4–0.7) and 20 % had radiographic VFs at baseline. Disease activity improved rapidly and sustained. LS BMD Z-score improved significantly up to 4 years compared to the previous time point and sustained thereafter. Median percentage of improvement compared to baseline was 8.9 % (2.8–15.8) and 7.2 % (2.2–14.7) after 4 and 8 years, respectively. Of 90 patients with baseline and 8-year radiographs, 14 (16 %) developed new VFs and 5 (6 %) showed an increase in severity of existing VFs. Of all 44 VFs present at 8 years, 30 % were grade 2 (<em>n</em> = 12) or grade 3 (<em>n</em> = 1).</p></div><div><h3>Conclusion</h3><p>In r-axSpA patients treated with TNFi for 8 years, LS BMD Z-score increased significantly, especially during the first 4 year of treatment. Radiographic VFs continued to develop or progressed, irrespective of improvement in BMD. Therefore, clinical attention for trabecular bone loss is important in daily clinical practice.</p></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":null,"pages":null},"PeriodicalIF":4.6,"publicationDate":"2024-07-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S004901722400163X/pdfft?md5=81d25b871991a960a392b060f9f955f3&pid=1-s2.0-S004901722400163X-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141630206","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-07-07DOI: 10.1016/j.semarthrit.2024.152513
Mustafa Ekici, Sibel Kadayıfçılar, Figen Bezci, Ömer Karadağ
{"title":"Commentary on IgG4-related uveitis. A French cohort and literature review.","authors":"Mustafa Ekici, Sibel Kadayıfçılar, Figen Bezci, Ömer Karadağ","doi":"10.1016/j.semarthrit.2024.152513","DOIUrl":"https://doi.org/10.1016/j.semarthrit.2024.152513","url":null,"abstract":"","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":null,"pages":null},"PeriodicalIF":4.6,"publicationDate":"2024-07-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141634485","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-07-04DOI: 10.1016/j.semarthrit.2024.152480
François Zimmermann , François Robin , Elisabeth Diot , Aurore Bleuzen , Sandrine Jousse-Joulin , Claire de Moreuil , Nicolas Belhomme , Claire Cazalets , Ronan Garlantézec , Agnès Gazzola , Francisco Llamas-Gutierrez , Romain Muraz , Antoinette Perlat , Guillaume Coiffier , Alain Lescoat
Introduction & objectives
This study aimed to characterize the whole phenotype of Systemic sclerosis (SSc) patients with sicca symptoms, using major salivary glands Ultrasound (SGUS) parameters, minor salivary glands biopsies (mSGB) and clinical findings, and to compare these characteristics with those from patients with Sjogren's Disease (SjD), and patients with sicca manifestations from other causes.
Methods
Sixty SSc patients fulfilling the 2013 ACR/EULAR classification criteria and with subjective self-declared sicca symptoms were consecutively recruited and had SGUS and mSGB. Fifteen SSc patients without subjective sicca symptoms and 65 patients with sicca symptoms from other causes (including 37 SjD with no SSc).
Results
SSc patients with subjective sicca symptoms had frequent objective clinical (up to 83 %), histological (44 % of Focus score≥1/ mm2) and US anomalies (63 % of OMERACT ≥2). 53 % patients without subjective clinical complaint also had abnormal objective tests, suggesting the existence of a sub clinical involvement of salivary glands in SSc. SjD-SSc patients had more severe glandular involvement as compared to patients with isolated SjD and isolated Sicca-SSc patients (70%, 48,6 % and 38% of patients with OMERACT ≥2 respectively) suggesting additive impact of both diseases on glandular physiology and structure.
Conclusion
SjD-SSc overlap have more severe sicca features as compared to isolated sicca-SSc and isolated SjD, suggesting a specific impact of SSc on salivary gland physiology. Further translational studies are needed to identify the underlying pathways that could serve as therapeutic targets.
{"title":"Whole phenotype of patients with systemic sclerosis and sicca manifestations: Comparison with sicca manifestations from other causes","authors":"François Zimmermann , François Robin , Elisabeth Diot , Aurore Bleuzen , Sandrine Jousse-Joulin , Claire de Moreuil , Nicolas Belhomme , Claire Cazalets , Ronan Garlantézec , Agnès Gazzola , Francisco Llamas-Gutierrez , Romain Muraz , Antoinette Perlat , Guillaume Coiffier , Alain Lescoat","doi":"10.1016/j.semarthrit.2024.152480","DOIUrl":"10.1016/j.semarthrit.2024.152480","url":null,"abstract":"<div><h3>Introduction & objectives</h3><p>This study aimed to characterize the whole phenotype of Systemic sclerosis (SSc) patients with sicca symptoms, using major salivary glands Ultrasound (SGUS) parameters, minor salivary glands biopsies (mSGB) and clinical findings, and to compare these characteristics with those from patients with Sjogren's Disease (SjD), and patients with sicca manifestations from other causes.</p></div><div><h3>Methods</h3><p>Sixty SSc patients fulfilling the 2013 ACR/EULAR classification criteria and with subjective self-declared sicca symptoms were consecutively recruited and had SGUS and mSGB. Fifteen SSc patients without subjective sicca symptoms and 65 patients with sicca symptoms from other causes (including 37 SjD with no SSc).</p></div><div><h3>Results</h3><p>SSc patients with subjective sicca symptoms had frequent objective clinical (up to 83 %), histological (44 % of Focus score≥1/ mm2) and US anomalies (63 % of OMERACT ≥2). 53 % patients without subjective clinical complaint also had abnormal objective tests, suggesting the existence of a sub clinical involvement of salivary glands in SSc. SjD-SSc patients had more severe glandular involvement as compared to patients with isolated SjD and isolated Sicca-SSc patients (70%, 48,6 % and 38% of patients with OMERACT ≥2 respectively) suggesting additive impact of both diseases on glandular physiology and structure.</p></div><div><h3>Conclusion</h3><p>SjD-SSc overlap have more severe sicca features as compared to isolated sicca-SSc and isolated SjD, suggesting a specific impact of SSc on salivary gland physiology. Further translational studies are needed to identify the underlying pathways that could serve as therapeutic targets.</p></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":null,"pages":null},"PeriodicalIF":4.6,"publicationDate":"2024-07-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141564258","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-07-04DOI: 10.1016/j.semarthrit.2024.152508
Marc K. Nielsen , Andreas W. Nielsen , Agnete O. Donskov , Ib T. Hansen , Berit D. Nielsen , Christoffer Mørk , Ellen M. Hauge , Kresten K. Keller
Introduction
Following the approval of tocilizumab (TCZ) for giant cell arteritis (GCA), recent studies have shown a high relapse frequency after abrupt discontinuation of TCZ. However, a thorough exploration of TCZ tapering compared to abrupt discontinuation has never been undertaken. Likewise, adverse events have only been scarcely investigated in routine care. This study aimed to compare the incidence of relapses in GCA patients undergoing TCZ tapering compared to abrupt discontinuation.
Methods
We performed a single-center retrospective cohort study from 2012 to 2022. Data from GCA patients treated with TCZ was obtained from the Electronic Patients Record. Relapse-free survival is reported in Kaplan-Meier plots and tapering versus abrupt discontinuation were compared using a Wilcoxon-Brewlos-Gehan test.
Results
We included 155 patients receiving TCZ treatment for GCA, of which 104 discontinued TCZ. Among the 104 patients discontinuing TCZ, 42 (40 %) experienced a relapse within the first year. A total of 57 patients underwent taper with 6/38 (16 %) and 2/19 (11 %) relapsing while receiving TCZ every second or third week, respectively. In comparison, 59 patients underwent abrupt discontinuation with 27 (46 %) relapsing during follow-up. The patients undergoing abrupt TCZ discontinuation demonstrated a significantly shorter time to relapse compared to all tapered patients (p = 0.02) as well as patients tapered from weekly TCZ treatment to every second week (p < 0.01). Furthermore, 15 % of patients discontinued TCZ due to adverse events.
Conclusion
This is the first study indicating that TCZ taper induced longer relapse-free survival than abrupt discontinuation, implying that taper may be favored over discontinuation in patients with GCA.
{"title":"Taper versus discontinuation of tocilizumab in patients with giant cell arteritis: Real-world experience from a tertiary center","authors":"Marc K. Nielsen , Andreas W. Nielsen , Agnete O. Donskov , Ib T. Hansen , Berit D. Nielsen , Christoffer Mørk , Ellen M. Hauge , Kresten K. Keller","doi":"10.1016/j.semarthrit.2024.152508","DOIUrl":"10.1016/j.semarthrit.2024.152508","url":null,"abstract":"<div><h3>Introduction</h3><p>Following the approval of tocilizumab (TCZ) for giant cell arteritis (GCA), recent studies have shown a high relapse frequency after abrupt discontinuation of TCZ. However, a thorough exploration of TCZ tapering compared to abrupt discontinuation has never been undertaken. Likewise, adverse events have only been scarcely investigated in routine care. This study aimed to compare the incidence of relapses in GCA patients undergoing TCZ tapering compared to abrupt discontinuation.</p></div><div><h3>Methods</h3><p>We performed a single-center retrospective cohort study from 2012 to 2022. Data from GCA patients treated with TCZ was obtained from the Electronic Patients Record. Relapse-free survival is reported in Kaplan-Meier plots and tapering versus abrupt discontinuation were compared using a Wilcoxon-Brewlos-Gehan test.</p></div><div><h3>Results</h3><p>We included 155 patients receiving TCZ treatment for GCA, of which 104 discontinued TCZ. Among the 104 patients discontinuing TCZ, 42 (40 %) experienced a relapse within the first year. A total of 57 patients underwent taper with 6/38 (16 %) and 2/19 (11 %) relapsing while receiving TCZ every second or third week, respectively. In comparison, 59 patients underwent abrupt discontinuation with 27 (46 %) relapsing during follow-up. The patients undergoing abrupt TCZ discontinuation demonstrated a significantly shorter time to relapse compared to all tapered patients (<em>p</em> = 0.02) as well as patients tapered from weekly TCZ treatment to every second week (<em>p</em> < 0.01). Furthermore, 15 % of patients discontinued TCZ due to adverse events.</p></div><div><h3>Conclusion</h3><p>This is the first study indicating that TCZ taper induced longer relapse-free survival than abrupt discontinuation, implying that taper may be favored over discontinuation in patients with GCA.</p></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":null,"pages":null},"PeriodicalIF":4.6,"publicationDate":"2024-07-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S0049017224001483/pdfft?md5=84ee41d18aa0d41e781f6c2d660917a5&pid=1-s2.0-S0049017224001483-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141564257","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-07-03DOI: 10.1016/j.semarthrit.2024.152511
Tomoyuki Kawada
{"title":"Mortality risk in patients with anti-MDA5 antibody-positive dermatomyositis: A risk assessment.","authors":"Tomoyuki Kawada","doi":"10.1016/j.semarthrit.2024.152511","DOIUrl":"https://doi.org/10.1016/j.semarthrit.2024.152511","url":null,"abstract":"","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":null,"pages":null},"PeriodicalIF":4.6,"publicationDate":"2024-07-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141545187","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-07-03DOI: 10.1016/j.semarthrit.2024.152487
S.E.(Sabine) Kloprogge , R.M.M.(Roshni) Lachmipersad , N.(Nienke) Katier , A.K.E.(Adinda) Mailuhu , J.(Jeanette) van Vooren , J.M.(John) van Ochten , P.J.E.(Patrick) Bindels , S.M.A.(Sita) Bierma-Zeinstra , M.(Marienke) van Middelkoop
Background
Ankle symptoms are a common reason to consult the general practitioner and often persist for years. In a population referred for ankle radiography, the prevalence of radiographic osteoarthritis (OA) is substantial, but its additional predictive value for persistent symptoms is unknown. Therefore, we examined the prognosis of symptoms 2–3 years after referral for ankle radiography, assessed clinical prognostic factors, and the additional predictive value of radiographic OA for persistent ankle complaints.
Methods
We included 893 adults referred for ankle radiography and studied the following candidate prognostic factors at baseline: age, sex, body mass index (BMI), referral for chronic complaints (>3 months), pain during activity (NRS-11) and presence of stiffness and functional loss as predominant symptom. X-rays were scored for radiographic OA. After 2–3 years participants were invited for a follow-up questionnaire including persistence of ankle complaints. To assess prognostic factors for persistent complaints, uni- and multivariable logistic regression were used.
Results
Of the 194 responders at follow-up, ankle complaints persisted in 71(36.6 %). BMI (OR 1.08; 95 % CI 1.01–1.15), stiffness as predominant symptom (OR 1.69; 95 % CI 0.89–3.21), and chronic complaints (OR 2.84; 95 % CI 1.45–5.57) were in the initial model for persistent complaints (AUC=0.69). After adding radiographic OA (OR 2.36; 95 % CI 1.01–5.50), the AUC of the final model became 0.70.
Conclusion
Ankle complaints persist in a considerable proportion of patients 2–3 years after referral for ankle radiography. BMI, chronic complaints and radiographic OA are prognostic factors for persistent complaints, but the additional predictive value of radiographic OA on top of clinical factors is negligible.
背景踝关节症状是向全科医生求诊的常见原因，而且往往持续多年。在转诊接受踝关节放射摄影检查的人群中，放射性骨关节炎（OA）的发病率很高，但其对持续症状的额外预测价值尚不清楚。因此，我们研究了转诊接受踝关节放射摄影检查 2-3 年后的症状预后，评估了临床预后因素以及放射学 OA 对持续性踝关节不适的额外预测价值。方法：我们纳入了 893 名转诊接受踝关节放射摄影检查的成年人，并研究了基线时的以下候选预后因素：年龄、性别、体重指数（BMI）、因慢性不适转诊（3 个月）、活动时疼痛（NRS-11）以及主要症状为僵硬和功能丧失。对 X 射线检查结果进行评分，以确定是否存在放射性 OA。2-3年后，受试者将被邀请接受包括踝关节持续不适症状在内的后续问卷调查。结果 在随访的 194 名应答者中，有 71 人（36.6%）的踝关节症状持续存在。体重指数（OR 1.08; 95 % CI 1.01-1.15）、僵硬为主要症状（OR 1.69; 95 % CI 0.89-3.21）和慢性症状（OR 2.84; 95 % CI 1.45-5.57）是持续症状的初始模型（AUC=0.69）。在加入放射性 OA（OR 2.36；95 % CI 1.01-5.50）后，最终模型的 AUC 为 0.70。体重指数（BMI）、慢性主诉和放射学 OA 是持续主诉的预后因素，但放射学 OA 对临床因素的额外预测价值微乎其微。
{"title":"Prognostic factors and the value of radiographic osteoarthritis for persistent complaints after referral for ankle radiography","authors":"S.E.(Sabine) Kloprogge , R.M.M.(Roshni) Lachmipersad , N.(Nienke) Katier , A.K.E.(Adinda) Mailuhu , J.(Jeanette) van Vooren , J.M.(John) van Ochten , P.J.E.(Patrick) Bindels , S.M.A.(Sita) Bierma-Zeinstra , M.(Marienke) van Middelkoop","doi":"10.1016/j.semarthrit.2024.152487","DOIUrl":"https://doi.org/10.1016/j.semarthrit.2024.152487","url":null,"abstract":"<div><h3>Background</h3><p>Ankle symptoms are a common reason to consult the general practitioner and often persist for years. In a population referred for ankle radiography, the prevalence of radiographic osteoarthritis (OA) is substantial, but its additional predictive value for persistent symptoms is unknown. Therefore, we examined the prognosis of symptoms 2–3 years after referral for ankle radiography, assessed clinical prognostic factors, and the additional predictive value of radiographic OA for persistent ankle complaints.</p></div><div><h3>Methods</h3><p>We included 893 adults referred for ankle radiography and studied the following candidate prognostic factors at baseline: age, sex, body mass index (BMI), referral for chronic complaints (>3 months), pain during activity (NRS-11) and presence of stiffness and functional loss as predominant symptom. X-rays were scored for radiographic OA. After 2–3 years participants were invited for a follow-up questionnaire including persistence of ankle complaints. To assess prognostic factors for persistent complaints, uni- and multivariable logistic regression were used.</p></div><div><h3>Results</h3><p>Of the 194 responders at follow-up, ankle complaints persisted in 71(36.6 %). BMI (OR 1.08; 95 % CI 1.01–1.15), stiffness as predominant symptom (OR 1.69; 95 % CI 0.89–3.21), and chronic complaints (OR 2.84; 95 % CI 1.45–5.57) were in the initial model for persistent complaints (AUC=0.69). After adding radiographic OA (OR 2.36; 95 % CI 1.01–5.50), the AUC of the final model became 0.70.</p></div><div><h3>Conclusion</h3><p>Ankle complaints persist in a considerable proportion of patients 2–3 years after referral for ankle radiography. BMI, chronic complaints and radiographic OA are prognostic factors for persistent complaints, but the additional predictive value of radiographic OA on top of clinical factors is negligible.</p></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":null,"pages":null},"PeriodicalIF":4.6,"publicationDate":"2024-07-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141592950","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-07-02DOI: 10.1016/j.semarthrit.2024.152507
Z. Touma , S. Kayaniyil , A. Parackal , D. Bonilla , J. Su , A. Johnston , J. Gahn , E.D. Hille , R. Ohsfeldt , S. Chandran
Background
New treatments for systemic lupus erythematosus (SLE) aim to improve tolerability and disease activity control over standard of care (SoC) treatment. SoC typically includes daily glucocorticoid (GC) which carries a risk of organ damage over time. This study sought to develop natural history models to identify predictors of long-term outcomes with current SoC SLE treatment.
Methods
Generalized linear and parametric accelerated failure time survival models (GLM) and parametric accelerated failure time (AFT) survival models were designed to identify predictors of disease activity, flare rate, GC use, organ damage, and mortality beyond the first year of treatment in patients with SLE. Models were run using a longitudinal retrospective analysis of prospectively collected Toronto Lupus Cohort (TLC) study data, collected between 1997 and 2020. Covariates of clinical and statistical significance were selected by bivariate- then multi-variate regression to find the model of best fit.
Findings
Of the 1255 subjects included, 89 % were female 89 % and 65 % Caucasian. Mean follow-up was 10·5 years. At first visit, 51 % of patients had moderate-to-severe disease activity (SLEDAI-2 K score ≥ 6). Mean organ damage scores gradually increased over the years following diagnosis. Median survival of the cohort was ∼35 years from study entry. In the GLM models, SLEDAI-2 K yearly average, and average GC dose were key for predicting change in SLEDAI-2 K, GC use/ dose, and flare (any/rate). Together, adjusted mean SLEDAI-2 K and GC dose were shown to be predictors of mortality and damage in at least 9 of 12 organ systems considered.
Interpretation
These comprehensive, longitudinal, predictive models show that disease activity and GC use are significant predictors of organ damage and mortality in a patient population with predominantly moderate to severe SLE. This deepens understanding of SLE natural history and underscores the need for new treatment approaches that reduce disease activity and GC use with an aim to improve long-term SLE outcomes.
{"title":"Modelling long-term outcomes for patients with systemic lupus erythematosus","authors":"Z. Touma , S. Kayaniyil , A. Parackal , D. Bonilla , J. Su , A. Johnston , J. Gahn , E.D. Hille , R. Ohsfeldt , S. Chandran","doi":"10.1016/j.semarthrit.2024.152507","DOIUrl":"10.1016/j.semarthrit.2024.152507","url":null,"abstract":"<div><h3>Background</h3><p>New treatments for systemic lupus erythematosus (SLE) aim to improve tolerability and disease activity control over standard of care (SoC) treatment. SoC typically includes daily glucocorticoid (GC) which carries a risk of organ damage over time. This study sought to develop natural history models to identify predictors of long-term outcomes with current SoC SLE treatment.</p></div><div><h3>Methods</h3><p>Generalized linear and parametric accelerated failure time survival models (GLM) and parametric accelerated failure time (AFT) survival models were designed to identify predictors of disease activity, flare rate, GC use, organ damage, and mortality beyond the first year of treatment in patients with SLE. Models were run using a longitudinal retrospective analysis of prospectively collected Toronto Lupus Cohort (TLC) study data, collected between 1997 and 2020. Covariates of clinical and statistical significance were selected by bivariate- then multi-variate regression to find the model of best fit.</p></div><div><h3>Findings</h3><p>Of the 1255 subjects included, 89 % were female 89 % and 65 % Caucasian. Mean follow-up was 10·5 years. At first visit, 51 % of patients had moderate-to-severe disease activity (SLEDAI-2 K score ≥ 6). Mean organ damage scores gradually increased over the years following diagnosis. Median survival of the cohort was ∼35 years from study entry. In the GLM models, SLEDAI-2 K yearly average, and average GC dose were key for predicting change in SLEDAI-2 K, GC use/ dose, and flare (any/rate). Together, adjusted mean SLEDAI-2 K and GC dose were shown to be predictors of mortality and damage in at least 9 of 12 organ systems considered.</p></div><div><h3>Interpretation</h3><p>These comprehensive, longitudinal, predictive models show that disease activity and GC use are significant predictors of organ damage and mortality in a patient population with predominantly moderate to severe SLE. This deepens understanding of SLE natural history and underscores the need for new treatment approaches that reduce disease activity and GC use with an aim to improve long-term SLE outcomes.</p></div><div><h3>Funding</h3><p>This study was funded by AstraZeneca.</p></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":null,"pages":null},"PeriodicalIF":4.6,"publicationDate":"2024-07-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S0049017224001471/pdfft?md5=ad77581972ce52f3f9a657deb50dc9f7&pid=1-s2.0-S0049017224001471-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141637280","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-07-02DOI: 10.1016/j.semarthrit.2024.152509
Osman Aykan Kargin , Serdar Arslan , Bora Korkmazer , Sabriye Guner , Ayse Ozdede , Nursena Erener , Elif Burcu Ersungur Celik , Gulcin Baktiroglu , Rauf Hamid , Ahmet Oz , Burc Cagri Poyraz , Ugur Uygunoglu , Emire Seyahi , Osman Kizilkilic
Objectives
To evaluate the microstructural integrity of brain white matter tracts in patients with Neuro-Behcet's syndrome (NBS) and Behcet's syndrome (BS) without neurological manifestations using diffusion tensor imaging (DTI) and to investigate potential utility of DTI as a surrogate biomarker of neurocognitive functioning and disease severity.
Methods
This cross-sectional study comprised 34 NBS patients and 32 BS patients without neurological involvement, identified based on the International Study Group of the Behcet's disease (ISGBD) and the International Consensus Recommendation (ICR) criteria, as well as 33 healthy controls. Cognitive functions, including attention, memory, language, abstraction, executive control, visuospatial skills, and sensorimotor performance were assessed using standardized questionnaires. DTI data were analyzed using tract-based spatial statistics (TBSS) and automated probabilistic tractography to investigate inter-group differences. Subsequently, correlations between tensor-derived parameters of white matter tracts, neurocognitive test scores, and disease severity measures were examined.
Results
DTI revealed decreased fractional anisotropy and increased radial diffusivity, mean diffusivity, and axial diffusivity in both supratentorial and infratentorial white matter in NBS patients, indicating widespread loss of microstructural integrity. Moreover, this loss of integrity was also observed in BS patients without neurological manifestations, albeit to a lesser extent. In NBS patients, certain white matter tracts, including cingulum bundle, were associated with poor cognitive performance across multiple domains and disease severity.
Discussion
DTI findings might potentially serve as a neuroimaging marker to predict the extent of neurocognitive impairment and disease severity associated with central nervous system involvement in BS.
{"title":"Brain white matter microstructural alterations in Behcet's syndrome correlate with cognitive impairment and disease severity: A diffusion tensor imaging study","authors":"Osman Aykan Kargin , Serdar Arslan , Bora Korkmazer , Sabriye Guner , Ayse Ozdede , Nursena Erener , Elif Burcu Ersungur Celik , Gulcin Baktiroglu , Rauf Hamid , Ahmet Oz , Burc Cagri Poyraz , Ugur Uygunoglu , Emire Seyahi , Osman Kizilkilic","doi":"10.1016/j.semarthrit.2024.152509","DOIUrl":"https://doi.org/10.1016/j.semarthrit.2024.152509","url":null,"abstract":"<div><h3>Objectives</h3><p>To evaluate the microstructural integrity of brain white matter tracts in patients with Neuro-Behcet's syndrome (NBS) and Behcet's syndrome (BS) without neurological manifestations using diffusion tensor imaging (DTI) and to investigate potential utility of DTI as a surrogate biomarker of neurocognitive functioning and disease severity.</p></div><div><h3>Methods</h3><p>This cross-sectional study comprised 34 NBS patients and 32 BS patients without neurological involvement, identified based on the International Study Group of the Behcet's disease (ISGBD) and the International Consensus Recommendation (ICR) criteria, as well as 33 healthy controls. Cognitive functions, including attention, memory, language, abstraction, executive control, visuospatial skills, and sensorimotor performance were assessed using standardized questionnaires. DTI data were analyzed using tract-based spatial statistics (TBSS) and automated probabilistic tractography to investigate inter-group differences. Subsequently, correlations between tensor-derived parameters of white matter tracts, neurocognitive test scores, and disease severity measures were examined.</p></div><div><h3>Results</h3><p>DTI revealed decreased fractional anisotropy and increased radial diffusivity, mean diffusivity, and axial diffusivity in both supratentorial and infratentorial white matter in NBS patients, indicating widespread loss of microstructural integrity. Moreover, this loss of integrity was also observed in BS patients without neurological manifestations, albeit to a lesser extent. In NBS patients, certain white matter tracts, including cingulum bundle, were associated with poor cognitive performance across multiple domains and disease severity.</p></div><div><h3>Discussion</h3><p>DTI findings might potentially serve as a neuroimaging marker to predict the extent of neurocognitive impairment and disease severity associated with central nervous system involvement in BS.</p></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":null,"pages":null},"PeriodicalIF":4.6,"publicationDate":"2024-07-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141606426","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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