Pub Date : 2025-02-01DOI: 10.1016/j.semarthrit.2024.152605
Gustavo G.M. Balbi , Pedro Gaspar , Hannah Cohen , David A. Isenberg , Doruk Erkan , Danieli Andrade , APS ACTION Extended “Damage” Working Group
Objectives
To gather the perspectives of APS ACTION members regarding the strengths and limitations of Damage Index for Antiphospholipid Syndrome (DIAPS); and establish recommendations for the improvement of DIAPS.
Methods
APS ACTION members were invited to answer a survey regarding their satisfaction with DIAPS scoring system and individual items. The level of agreement (LoA) among members with the inclusion of individual items in DIAPS was calculated (LoA of <75% was considered disagreement). Respondents’ open-ended comments about DIAPS limitations were also collected, which helped formulate our recommendations for DIAPS improvement.
Results
Forty-two APS ACTION members (58.3%) answered the survey. Of them, 26 (61.9%) were satisfied, 4 (9.5%) were neutral, and 12 (28.6%) were dissatisfied with the current DIAPS scoring system. Fifteen items (39.5%) presented a LoA <75% regarding the inclusion in DIAPS. Respondents provided comments that were grouped under six main categories related to concerns about: a) definitions and attribution of damage (including causality and temporal relationship); b) scoring system; c) overlapping items; d) specific items (exclusion of redundant items and inclusion of additional ones); e) the need to incorporate multiple events; and f) feasibility and practicality. Finally, the APS ACTION “Damage” Working Group developed 7 recommendations that should be considered for the next generation DIAPS.
Conclusion
Approximately 60% of respondents were satisfied with DIAPS and its definitions; however, our survey demonstrated that there is substantial room to improve the current damage index for APS. Efforts for updating DIAPS should consider the APS ACTION “Damage” Working Group recommendations.
{"title":"Damage Index for Antiphospholipid Syndrome (DIAPS): An Antiphospholipid Syndrome Alliance for Clinical Trials and International Networking (APS ACTION) “Damage” working group report on strengths and limitations","authors":"Gustavo G.M. Balbi , Pedro Gaspar , Hannah Cohen , David A. Isenberg , Doruk Erkan , Danieli Andrade , APS ACTION Extended “Damage” Working Group","doi":"10.1016/j.semarthrit.2024.152605","DOIUrl":"10.1016/j.semarthrit.2024.152605","url":null,"abstract":"<div><h3>Objectives</h3><div>To gather the perspectives of APS ACTION members regarding the strengths and limitations of Damage Index for Antiphospholipid Syndrome (DIAPS); and establish recommendations for the improvement of DIAPS.</div></div><div><h3>Methods</h3><div>APS ACTION members were invited to answer a survey regarding their satisfaction with DIAPS scoring system and individual items. The level of agreement (LoA) among members with the inclusion of individual items in DIAPS was calculated (LoA of <75% was considered disagreement). Respondents’ open-ended comments about DIAPS limitations were also collected, which helped formulate our recommendations for DIAPS improvement.</div></div><div><h3>Results</h3><div>Forty-two APS ACTION members (58.3%) answered the survey. Of them, 26 (61.9%) were satisfied, 4 (9.5%) were neutral, and 12 (28.6%) were dissatisfied with the current DIAPS scoring system. Fifteen items (39.5%) presented a LoA <75% regarding the inclusion in DIAPS. Respondents provided comments that were grouped under six main categories related to concerns about: a) definitions and attribution of damage (including causality and temporal relationship); b) scoring system; c) overlapping items; d) specific items (exclusion of redundant items and inclusion of additional ones); e) the need to incorporate multiple events; and f) feasibility and practicality. Finally, the APS ACTION “Damage” Working Group developed 7 recommendations that should be considered for the next generation DIAPS.</div></div><div><h3>Conclusion</h3><div>Approximately 60% of respondents were satisfied with DIAPS and its definitions; however, our survey demonstrated that there is substantial room to improve the current damage index for APS. Efforts for updating DIAPS should consider the APS ACTION “Damage” Working Group recommendations.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"70 ","pages":"Article 152605"},"PeriodicalIF":4.6,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142814218","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 : 2025-02-01DOI: 10.1016/j.semarthrit.2024.152609
Guy Katz, Claire E. Cook, Xiaoqing Fu, Andrew J. King, John H. Stone, Hyon K. Choi, Zachary S. Wallace
Objectives
Patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) face excess mortality compared with the general population. Mortality in clinical epidemiology research is often examined using death certificate diagnosis codes; however, the sensitivity of such codes in AAV is unknown.
Methods
We performed a retrospective cohort study using the Mass General Brigham AAV Cohort, including patients with AAV who died between 2002 and 2019. Causes of death were determined by electronic health record (EHR) review (reference gold standard) and via cause of death diagnosis codes on death certificates. We calculated the sensitivity of death certificate diagnosis codes for AAV.
Results
Of 684 patients in the registry, 184 died, 92 (52 %) of whom had adequate EHR data available determine cause of death and 72 (40 %) of whom had both EHR and death certificate data available. Death due to AAV, infection, cardiovascular disease, and cancer occurred in 8 %, 29 %, 5 %, and 18 %, respectively, when ascertained by manual review, as opposed to 0 %, 11 %, 25 %, and 21 %, as determined by death certificates. The sensitivity of AAV diagnosis codes for AAV was 16.6 % (95 % CI: 10.5, 22.6) among all patients with death certificate data available.
Conclusion
In a contemporary cohort of patients with AAV, infection was the most common cause of death, while death due to AAV itself was rare. We found a high degree of discordance between causes of death determined by manual review and death certificate diagnosis codes. Mortality research on AAV should include linkage to medical records data to reduce potential bias.
{"title":"Defining cause of death in a contemporary cohort with ANCA-associated vasculitis (AAV): A comparison of electronic health record and death certificate data","authors":"Guy Katz, Claire E. Cook, Xiaoqing Fu, Andrew J. King, John H. Stone, Hyon K. Choi, Zachary S. Wallace","doi":"10.1016/j.semarthrit.2024.152609","DOIUrl":"10.1016/j.semarthrit.2024.152609","url":null,"abstract":"<div><h3>Objectives</h3><div>Patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) face excess mortality compared with the general population. Mortality in clinical epidemiology research is often examined using death certificate diagnosis codes; however, the sensitivity of such codes in AAV is unknown.</div></div><div><h3>Methods</h3><div>We performed a retrospective cohort study using the Mass General Brigham AAV Cohort, including patients with AAV who died between 2002 and 2019. Causes of death were determined by electronic health record (EHR) review (reference gold standard) and via cause of death diagnosis codes on death certificates. We calculated the sensitivity of death certificate diagnosis codes for AAV.</div></div><div><h3>Results</h3><div>Of 684 patients in the registry, 184 died, 92 (52 %) of whom had adequate EHR data available determine cause of death and 72 (40 %) of whom had both EHR and death certificate data available. Death due to AAV, infection, cardiovascular disease, and cancer occurred in 8 %, 29 %, 5 %, and 18 %, respectively, when ascertained by manual review, as opposed to 0 %, 11 %, 25 %, and 21 %, as determined by death certificates. The sensitivity of AAV diagnosis codes for AAV was 16.6 % (95 % CI: 10.5, 22.6) among all patients with death certificate data available.</div></div><div><h3>Conclusion</h3><div>In a contemporary cohort of patients with AAV, infection was the most common cause of death, while death due to AAV itself was rare. We found a high degree of discordance between causes of death determined by manual review and death certificate diagnosis codes. Mortality research on AAV should include linkage to medical records data to reduce potential bias.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"70 ","pages":"Article 152609"},"PeriodicalIF":4.6,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142903417","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 : 2025-02-01DOI: 10.1016/j.semarthrit.2024.152587
Kristine A. Kuhn
None.
无。
{"title":"Microbial pathways contributing to the pathogenesis of rheumatoid arthritis","authors":"Kristine A. Kuhn","doi":"10.1016/j.semarthrit.2024.152587","DOIUrl":"10.1016/j.semarthrit.2024.152587","url":null,"abstract":"<div><div>None.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"70 ","pages":"Article 152587"},"PeriodicalIF":4.6,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142627716","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 : 2025-02-01DOI: 10.1016/j.semarthrit.2024.152590
Iris Navarro-Millán
Abstract Para
Para.
{"title":"The cause is worse than the effect: Inequities in the United States health system; how could we change them?","authors":"Iris Navarro-Millán","doi":"10.1016/j.semarthrit.2024.152590","DOIUrl":"10.1016/j.semarthrit.2024.152590","url":null,"abstract":"<div><div>Abstract Para</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"70 ","pages":"Article 152590"},"PeriodicalIF":4.6,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142639710","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 : 2025-02-01DOI: 10.1016/j.semarthrit.2024.152603
Catherine Deffendall , Sarah Green , Ashley Suh , Nikol Nikolova , Katherine Walker , Raeann Whitney , Lee Wheless , Sarah Osmundson , April Barnado
Objective
Few studies have examined peripartum maternal outcomes in systemic lupus erythematosus (SLE). Using a de-identified electronic health record (EHR) cohort of individuals with and without SLE, we compared rates of peripartum maternal outcomes including maternal infections, blood transfusions, hospital length of stay, and SLE flares.
Methods
We identified deliveries among individuals with SLE and individuals without autoimmune disease using a previously validated algorithm. Peripartum maternal infection was assessed up to 6 weeks postpartum. Using Chi-square and Mann-Whitney U tests, we compared peripartum outcomes in SLE and control deliveries. We performed mixed effects models to estimate the association of SLE case status with peripartum outcomes. We assessed for SLE flares up to 6 months postpartum using chart review of rheumatology notes and the 2009 revised SELENA Flare Index. We evaluated SLE medications prescribed during pregnancy and at time of delivery on peripartum outcomes.
Results
We identified 185 deliveries to 142 individuals with SLE and 468 deliveries to 241 control individuals without autoimmune diseases. Mean length of hospital stay was longer for individuals with SLE compared to controls (3.1 ± 2.0 vs. 2.4 ± 1.0 days, p < 0.001). In a mixed effects model, peripartum infection was significantly associated with SLE case status (OR = 6.18, 95 % CI 2.73 – 13.98, p < 0.01), Cesarean section (OR = 5.00, 95 % CI 2.16 – 11.57, p < 0.01), and age at delivery (OR = 0.92, 95 % CI 0.86 - 0.99, p = 0.03) after adjusting for race. Transfusion was also significantly associated with SLE case status (OR = 9.05, 95 % CI 3.24–25.32, p < 0.01) and Black race (OR = 6.64, 95 % CI 1.47 – 30.02, p = 0.01) after adjusting for Cesarean section and age at delivery. We observed a postpartum flare rate of 32 % among individuals with SLE with 13 % characterized as mild, 41 % moderate, and 46 % severe. Antimalarial use in the postpartum period was associated with lower flare rate (43 % vs. 63 %, p = 0.04).
Conclusions
Individuals with SLE have increased rates of blood transfusions, longer hospital stays, and more frequent infections compared to control individuals in the peripartum period. We observed a postpartum flare rate of 32 %, and antimalarial use was associated with lower flare rate. Our findings demonstrate that the peripartum period remains a high-risk time for individuals with SLE with an ongoing need for close monitoring.
目的：很少有研究对系统性红斑狼疮（SLE）围生期产妇的预后进行调查。使用一个去识别的电子健康记录（EHR）队列，有和没有SLE的个体，我们比较围产期产妇结局的比率，包括产妇感染、输血、住院时间和SLE发作。方法：我们使用先前验证的算法确定SLE患者和无自身免疫性疾病患者的分娩情况。围产期产妇感染评估至产后6周。使用卡方检验和Mann-Whitney U检验，我们比较了SLE和对照组分娩的围生期结局。我们采用混合效应模型来估计SLE病例状态与围产期结局的关系。我们使用风湿病学记录的图表回顾和2009年修订的SELENA耀斑指数来评估产后6个月的SLE耀斑。我们评估了怀孕期间和分娩时SLE药物对围产期结果的影响。结果：我们确定了185例给142例SLE患者接生，468例给241例没有自身免疫性疾病的对照患者接生。SLE患者的平均住院时间比对照组更长（3.1±2.0天比2.4±1.0天，p < 0.001）。在混合效应模型中，围生期感染与SLE病例状态（OR = 6.18, 95% CI 2.73 - 13.98, p < 0.01）、剖宫产（OR = 5.00, 95% CI 2.16 - 11.57, p < 0.01）和分娩年龄（OR = 0.92, 95% CI 0.86 - 0.99, p = 0.03）相关。在调整剖宫产和分娩年龄后，输血也与SLE病例状态（OR = 9.05, 95% CI 3.24-25.32, p < 0.01）和黑人种族（OR = 6.64, 95% CI 1.47 - 30.02, p = 0.01）显著相关。我们观察到SLE患者的产后爆发率为32%，其中13%为轻度，41%为中度，46%为重度。产后使用抗疟药与较低的发作率相关（43%对63%，p = 0.04）。结论：与对照组相比，围产期SLE患者输血率增加，住院时间延长，感染更频繁。我们观察到产后爆发率为32%，抗疟药的使用与较低的爆发率相关。我们的研究结果表明，围产期仍然是SLE患者的高风险时期，需要持续密切监测。
{"title":"Peripartum maternal outcomes in individuals with systemic lupus erythematosus in a real-world electronic health record cohort","authors":"Catherine Deffendall , Sarah Green , Ashley Suh , Nikol Nikolova , Katherine Walker , Raeann Whitney , Lee Wheless , Sarah Osmundson , April Barnado","doi":"10.1016/j.semarthrit.2024.152603","DOIUrl":"10.1016/j.semarthrit.2024.152603","url":null,"abstract":"<div><h3>Objective</h3><div>Few studies have examined peripartum maternal outcomes in systemic lupus erythematosus (SLE). Using a de-identified electronic health record (EHR) cohort of individuals with and without SLE, we compared rates of peripartum maternal outcomes including maternal infections, blood transfusions, hospital length of stay, and SLE flares.</div></div><div><h3>Methods</h3><div>We identified deliveries among individuals with SLE and individuals without autoimmune disease using a previously validated algorithm. Peripartum maternal infection was assessed up to 6 weeks postpartum. Using Chi-square and Mann-Whitney U tests, we compared peripartum outcomes in SLE and control deliveries. We performed mixed effects models to estimate the association of SLE case status with peripartum outcomes. We assessed for SLE flares up to 6 months postpartum using chart review of rheumatology notes and the 2009 revised SELENA Flare Index. We evaluated SLE medications prescribed during pregnancy and at time of delivery on peripartum outcomes.</div></div><div><h3>Results</h3><div>We identified 185 deliveries to 142 individuals with SLE and 468 deliveries to 241 control individuals without autoimmune diseases. Mean length of hospital stay was longer for individuals with SLE compared to controls (3.1 ± 2.0 vs. 2.4 ± 1.0 days, <em>p</em> < 0.001). In a mixed effects model, peripartum infection was significantly associated with SLE case status (OR = 6.18, 95 % CI 2.73 – 13.98, <em>p</em> < 0.01), Cesarean section (OR = 5.00, 95 % CI 2.16 – 11.57, <em>p</em> < 0.01), and age at delivery (OR = 0.92, 95 % CI 0.86 - 0.99, <em>p</em> = 0.03) after adjusting for race. Transfusion was also significantly associated with SLE case status (OR = 9.05, 95 % CI 3.24–25.32, <em>p</em> < 0.01) and Black race (OR = 6.64, 95 % CI 1.47 – 30.02, <em>p</em> = 0.01) after adjusting for Cesarean section and age at delivery. We observed a postpartum flare rate of 32 % among individuals with SLE with 13 % characterized as mild, 41 % moderate, and 46 % severe. Antimalarial use in the postpartum period was associated with lower flare rate (43 % vs. 63 %, <em>p</em> = 0.04).</div></div><div><h3>Conclusions</h3><div>Individuals with SLE have increased rates of blood transfusions, longer hospital stays, and more frequent infections compared to control individuals in the peripartum period. We observed a postpartum flare rate of 32 %, and antimalarial use was associated with lower flare rate. Our findings demonstrate that the peripartum period remains a high-risk time for individuals with SLE with an ongoing need for close monitoring.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"70 ","pages":"Article 152603"},"PeriodicalIF":4.6,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142786938","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 : 2025-01-31DOI: 10.1016/j.semarthrit.2025.152651
Yongpeng Ge, Wei Jiang, Xin Lu, Guochun Wang , Xiaoming Shu
Objectives
The purpose of this study was to analyze the characteristics of an ulcerative rash in dermatomyositis (DM) patients with anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody and to assess the efficacy of tadalafil in the management of refractory cutaneous ulcers.
Methods
Medical records of DM patients with anti-MDA5 antibody were reviewed. Skin rash data, radiologic characteristic and serum markers were collected. Patients with refractory cutaneous ulcers treated with tadalafil were reviewed retrospectively.
Results
Eighty-two consecutive cases with anti-MDA5-antibody positive DM (anti-MDA5 DM) were identified, including 62 (75.6 %) female patients. Approximately 95.0 % of the patients had interstitial lung disease (ILD) and 45.1 % had rapidly progressive ILD (RP-ILD). All patients (100 %) had typical skin rashes, such as Gottron's sign (87.8 %), heliotrope sign (69.5 %), and the holster sign (24.4 %). Twenty-six patients (31.7 %) had cutaneous ulcers. Univariate analysis showed that ulcer patients were prone to Gottron's sign (p = 0.026), perlungual erythematosus (p = 0.007), and arthritis (p = 0.031), while shortness of breath after exercise (p = 0.027) and RP-ILD (p = 0.024) are more likely to appear in patients with non-skin ulcers. Among the anti-MDA5 DM patients with skin ulcers, 10 (38.5 %) were refractory cutaneous ulcers, including five male patients. After tadalafil was added, eight (80 %) patients showed improvement in the physician global assessment (PGA), Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) score and the patient's pain score using the visual analog score (p-VAS), within one to two months. In addition, the refractory cutaneous ulcers of five patients completely resolved after treatment.
Conclusions
This study found that ulcerative rash in anti-MDA5 DM may not be associated with RP-ILD. The results also suggest that tadalafil may be a useful therapy for refractory cutaneous ulcers.
{"title":"Refractory cutaneous ulcers in anti-MDA5 dermatomyositis were not associated with rapidly progressive interstitial lung disease, and responded to tadalafil","authors":"Yongpeng Ge, Wei Jiang, Xin Lu, Guochun Wang , Xiaoming Shu","doi":"10.1016/j.semarthrit.2025.152651","DOIUrl":"10.1016/j.semarthrit.2025.152651","url":null,"abstract":"<div><h3>Objectives</h3><div>The purpose of this study was to analyze the characteristics of an ulcerative rash in dermatomyositis (DM) patients with anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody and to assess the efficacy of tadalafil in the management of refractory cutaneous ulcers.</div></div><div><h3>Methods</h3><div>Medical records of DM patients with anti-MDA5 antibody were reviewed. Skin rash data, radiologic characteristic and serum markers were collected. Patients with refractory cutaneous ulcers treated with tadalafil were reviewed retrospectively.</div></div><div><h3>Results</h3><div>Eighty-two consecutive cases with anti-MDA5-antibody positive DM (anti-MDA5 DM) were identified, including 62 (75.6 %) female patients. Approximately 95.0 % of the patients had interstitial lung disease (ILD) and 45.1 % had rapidly progressive ILD (RP-ILD). All patients (100 %) had typical skin rashes, such as Gottron's sign (87.8 %), heliotrope sign (69.5 %), and the holster sign (24.4 %). Twenty-six patients (31.7 %) had cutaneous ulcers. Univariate analysis showed that ulcer patients were prone to Gottron's sign (<em>p</em> = 0.026), perlungual erythematosus (<em>p</em> = 0.007), and arthritis (<em>p</em> = 0.031), while shortness of breath after exercise (<em>p</em> = 0.027) and RP-ILD (<em>p</em> = 0.024) are more likely to appear in patients with non-skin ulcers. Among the anti-MDA5 DM patients with skin ulcers, 10 (38.5 %) were refractory cutaneous ulcers, including five male patients. After tadalafil was added, eight (80 %) patients showed improvement in the physician global assessment (PGA), Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) score and the patient's pain score using the visual analog score (p-VAS), within one to two months. In addition, the refractory cutaneous ulcers of five patients completely resolved after treatment.</div></div><div><h3>Conclusions</h3><div>This study found that ulcerative rash in anti-MDA5 DM may not be associated with RP-ILD. The results also suggest that tadalafil may be a useful therapy for refractory cutaneous ulcers.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"71 ","pages":"Article 152651"},"PeriodicalIF":4.6,"publicationDate":"2025-01-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143139438","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}
Pulmonary function tests (PFTs) and extent of ILD on HRCT predict mortality in systemic sclerosis associated interstitial lung disease (SSc-ILD). It is not known whether location and type in addition to extent of affected lung parenchyma are associated with PFTs changes.
Methods
SSc patients from a targeted healthcare program were included when PFTs and visually scored concomitant chest HRCT and PFTs at one year follow-up were available. Lung parenchyma of SSc patients was semi-quantitatively scored by visual assessment (reticulation, ground glass opacities, emphysema and disease extent) at five levels from apex to base. Regression analysis after linearity check and excluding multicollinearity was used to predict changes in PFT parameters (TLC, total lung capacity; FVC, forced vital capacity; DLCO, diffusion capacity for carbon monoxide).
Results
A total of 185 patients were included (85% female, mean age at first symptoms 40 years). All HRCT variables correlated with PFT parameters cross-sectionally. Disease extent and reticulation at the apices (level 1), reticulation at level 2, disease extent and reticulation at level 3 all correlated significantly with TLC (r 0.151–0.17, p < 0.05). Of these HRCT variables disease extent at level 1 predicted change in TLC (adjusted R2 0.024, p 0.021) and when excluding patients with emphysema or pulmonary hypertension, reticulation at level 3 predicted change in TLC (adjusted R2 0.026, p 0.020).
Conclusions
In patients with systemic sclerosis and lung involvement, disease extent and reticulation at the mid-upper zones predicted change in TLC which may be of clinical importance.
{"title":"Semi-quantitatively scored apical extent of disease predicts change in total lung capacity in patients with systemic sclerosis and early interstitial lung disease.","authors":"K.M.C. van Doorn-Hogervorst , E.R. (Emiel) Marges , A.A. (Anne) Schouffoer , L.J.M. (Lucia) Kroft , T.W.J. (Thomas) Huizinga , J.J.M. (Miranda) Geelhoed , J.K. (Jeska) de Vries-Bouwstra , M.K. (Maarten) Ninaber","doi":"10.1016/j.semarthrit.2025.152650","DOIUrl":"10.1016/j.semarthrit.2025.152650","url":null,"abstract":"<div><h3>Background</h3><div>Pulmonary function tests (PFTs) and extent of ILD on HRCT predict mortality in systemic sclerosis associated interstitial lung disease (SSc-ILD). It is not known whether location and type in addition to extent of affected lung parenchyma are associated with PFTs changes.</div></div><div><h3>Methods</h3><div>SSc patients from a targeted healthcare program were included when PFTs and visually scored concomitant chest HRCT and PFTs at one year follow-up were available. Lung parenchyma of SSc patients was semi-quantitatively scored by visual assessment (reticulation, ground glass opacities, emphysema and disease extent) at five levels from apex to base. Regression analysis after linearity check and excluding multicollinearity was used to predict changes in PFT parameters (TLC, total lung capacity; FVC, forced vital capacity; DLCO, diffusion capacity for carbon monoxide).</div></div><div><h3>Results</h3><div>A total of 185 patients were included (85% female, mean age at first symptoms 40 years). All HRCT variables correlated with PFT parameters cross-sectionally. Disease extent and reticulation at the apices (level 1), reticulation at level 2, disease extent and reticulation at level 3 all correlated significantly with TLC (r 0.151–0.17, <em>p</em> < 0.05). Of these HRCT variables disease extent at level 1 predicted change in TLC (adjusted R<sup>2</sup> 0.024, p 0.021) and when excluding patients with emphysema or pulmonary hypertension, reticulation at level 3 predicted change in TLC (adjusted R<sup>2</sup> 0.026, p 0.020).</div></div><div><h3>Conclusions</h3><div>In patients with systemic sclerosis and lung involvement, disease extent and reticulation at the mid-upper zones predicted change in TLC which may be of clinical importance.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"71 ","pages":"Article 152650"},"PeriodicalIF":4.6,"publicationDate":"2025-01-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143290936","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 : 2025-01-31DOI: 10.1016/j.semarthrit.2025.152638
Julia Spierings , Giulia Bandini , Yannick Allanore , Nicoletta Del Papa , Christopher P Denton , Oliver Distler , Daniel E. Furst , Raffaella Greco , Dinesh Khanna , Masataka Kuwana , Marco Matucci-Cerinic , Mandana Nikpour , Anna van Rhenen , Jacob M van Laar , Michael Hughes
Objective
Autologous stem cell transplantation (AHSCT) is an established treatment in diffuse cutaneous systemic sclerosis (dcSSc). Optimal management of disease progression after AHSCT in dcSSc has not been defined. The aim of this study was to explore the experience and preferences of SSc experts on post-AHSCT management.
Methods
An online questionnaire study was conducted containing 17 questions concerning respondent demographics, definition of SSc progression after AHSCT, diagnostic work-up and treatment preferences.
Results
In total, 69 respondents from 21 countries completed the questionnaire. The majority (89.7 %) works at a university hospital, and were involved in decisions regarding AHSCT in patients with SSc (71 %). Most have 1 to 5 patients who underwent AHSCT under their care. They defined failure to improve after AHSCT as: an increase in mRSS, new onset or worsening of interstitial lung disease (ILD), new onset scleroderma renal crisis (SRC) or inflammatory arthritis. Progression after initial response was defined as: increase in mRSS, new or worsening of ILD, new SRC, inflammatory arthritis, new pulmonary arterial hypertension, digital vasculopathy or impaired physical functioning. The most frequent therapy in case of AHSCT failure was mycophenolate mofetil (N = 55, 88.7 %), rituximab (N = 54, 87.1 %), nintedanib (N = 39, 62.9 %) or/and tocilizumab (N = 36, 58.1 %). Combination therapy with more than one of these agents was considered by most respondents (N = 61, 88.4 %).
Conclusion
Our study benchmarks the unique combined experiences of post-AHSCT management among SSc experts. We summarize preferences regarding definition of AHSCT failure and progression after response, as well as approach to diagnostic work-up and treatment.
{"title":"Management of disease progression after autologous hematopoietic stem cell transplantation in systemic sclerosis: Results from an international questionnaire-based study","authors":"Julia Spierings , Giulia Bandini , Yannick Allanore , Nicoletta Del Papa , Christopher P Denton , Oliver Distler , Daniel E. Furst , Raffaella Greco , Dinesh Khanna , Masataka Kuwana , Marco Matucci-Cerinic , Mandana Nikpour , Anna van Rhenen , Jacob M van Laar , Michael Hughes","doi":"10.1016/j.semarthrit.2025.152638","DOIUrl":"10.1016/j.semarthrit.2025.152638","url":null,"abstract":"<div><h3>Objective</h3><div>Autologous stem cell transplantation (AHSCT) is an established treatment in diffuse cutaneous systemic sclerosis (dcSSc). Optimal management of disease progression after AHSCT in dcSSc has not been defined. The aim of this study was to explore the experience and preferences of SSc experts on post-AHSCT management.</div></div><div><h3>Methods</h3><div>An online questionnaire study was conducted containing 17 questions concerning respondent demographics, definition of SSc progression after AHSCT, diagnostic work-up and treatment preferences.</div></div><div><h3>Results</h3><div>In total, 69 respondents from 21 countries completed the questionnaire. The majority (89.7 %) works at a university hospital, and were involved in decisions regarding AHSCT in patients with SSc (71 %). Most have 1 to 5 patients who underwent AHSCT under their care. They defined failure to improve after AHSCT as: an increase in mRSS, new onset or worsening of interstitial lung disease (ILD), new onset scleroderma renal crisis (SRC) or inflammatory arthritis. Progression after initial response was defined as: increase in mRSS, new or worsening of ILD, new SRC, inflammatory arthritis, new pulmonary arterial hypertension, digital vasculopathy or impaired physical functioning. The most frequent therapy in case of AHSCT failure was mycophenolate mofetil (<em>N</em> = 55, 88.7 %), rituximab (<em>N</em> = 54, 87.1 %), nintedanib (<em>N</em> = 39, 62.9 %) or/and tocilizumab (<em>N</em> = 36, 58.1 %). Combination therapy with more than one of these agents was considered by most respondents (<em>N</em> = 61, 88.4 %).</div></div><div><h3>Conclusion</h3><div>Our study benchmarks the unique combined experiences of post-AHSCT management among SSc experts. We summarize preferences regarding definition of AHSCT failure and progression after response, as well as approach to diagnostic work-up and treatment.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"71 ","pages":"Article 152638"},"PeriodicalIF":4.6,"publicationDate":"2025-01-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143139224","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 : 2025-01-30DOI: 10.1016/j.semarthrit.2025.152632
Ji Hyoun Kim , Yo Han Im , Ji Hyun Noh , Jin-Sung Yuk
Objectives
This retrospective cohort study aimed to investigate the influence of menopausal hormone therapy (MHT) on the occurrence of systemic lupus erythematosus (SLE) in postmenopausal women. Additionally, the study aimed to examine the specific effects of individual MHT drugs.
Methods
In this population-based cohort study conducted in Korea, a total of 452,124 women aged >40 years seeking healthcare for menopause were assessed from January 1, 2011, to December 31, 2014. After employing propensity score matching, 139,331 pairs were included in the MHT and non-MHT groups. Follow-up of participants continued until December 31, 2020. The diagnosis of SLE was based on the International Classification of Diseases 10th edition criteria.
Results
The median follow-up in the study was 7.9 [6.9–8.9] years. SLE developed in 134 (0.1 %) of the 139,197 participants in the MHT group and 143 (0.1 %) of the 139,188 of the non-MHT group, individually. The risk of SLE in the MHT group did not show a significant increase compared to the non-MHT group {hazard ratio (HR) 1.114, 95 % confidence interval (CI) 0.88–1.41}. Subgroup analysis results indicated no significant differences based on the type of MHT or the duration of MHT use, except tibolone. In the group that used tibolone within 3 years, the HR for SLE risk was 1.45 (95 % confidence interval: 1.051–2.001).
Conclusion
The utilization of MHT did not demonstrate a substantial impact on the development of SLE in postmenopausal women. Caution is required in the early stages of tibolone use.
{"title":"The effects of menopausal hormone therapy for the risk of systemic lupus erythematosus: A nationwide cohort study in Korea","authors":"Ji Hyoun Kim , Yo Han Im , Ji Hyun Noh , Jin-Sung Yuk","doi":"10.1016/j.semarthrit.2025.152632","DOIUrl":"10.1016/j.semarthrit.2025.152632","url":null,"abstract":"<div><h3>Objectives</h3><div>This retrospective cohort study aimed to investigate the influence of menopausal hormone therapy (MHT) on the occurrence of systemic lupus erythematosus (SLE) in postmenopausal women. Additionally, the study aimed to examine the specific effects of individual MHT drugs.</div></div><div><h3>Methods</h3><div>In this population-based cohort study conducted in Korea, a total of 452,124 women aged >40 years seeking healthcare for menopause were assessed from January 1, 2011, to December 31, 2014. After employing propensity score matching, 139,331 pairs were included in the MHT and non-MHT groups. Follow-up of participants continued until December 31, 2020. The diagnosis of SLE was based on the International Classification of Diseases 10th edition criteria.</div></div><div><h3>Results</h3><div>The median follow-up in the study was 7.9 [6.9–8.9] years. SLE developed in 134 (0.1 %) of the 139,197 participants in the MHT group and 143 (0.1 %) of the 139,188 of the non-MHT group, individually. The risk of SLE in the MHT group did not show a significant increase compared to the non-MHT group {hazard ratio (HR) 1.114, 95 % confidence interval (CI) 0.88–1.41}. Subgroup analysis results indicated no significant differences based on the type of MHT or the duration of MHT use, except tibolone. In the group that used tibolone within 3 years, the HR for SLE risk was 1.45 (95 % confidence interval: 1.051–2.001).</div></div><div><h3>Conclusion</h3><div>The utilization of MHT did not demonstrate a substantial impact on the development of SLE in postmenopausal women. Caution is required in the early stages of tibolone use.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"71 ","pages":"Article 152632"},"PeriodicalIF":4.6,"publicationDate":"2025-01-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143290927","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 : 2025-01-30DOI: 10.1016/j.semarthrit.2025.152644
Andre S. Franco, Igor H. Murai, Thomas H. Yang, Liliam Takayama, Virginia L.N. Bonoldi, Valeria F. Caparbo, Lissiane K.N. Guedes, Diogo S. Domiciano, Sandra G. Pasoto, Camille P. Figueiredo, Rosa M.R. Pereira
Background
Sjögren's disease (SjD) arthritis is non-erosive, but joint involvement in this disease remains unclear. This study investigated the association between bone erosions and clinical parameters using high-resolution peripheral quantitative computed tomography (HR-pQCT) in patients with SjD.
Methods
In this cross-sectional study at a quaternary hospital in Brazil, 106 female patients with SjD, aged 18–65 years, who met ACR/EULAR 2016 criteria were evaluated. Exclusion criteria were the use of osteoporosis medication and other bone or autoimmune diseases. Disease activity, damage, functional disability, and laboratory tests were assessed. HR-pQCT was used to identify bone erosions and osteophytes in the 2nd and 3rd metacarpophalangeal and proximal interphalangeal joints, and functional impairment was measured by the handgrip test. Associations between bone erosions, osteophytes, and clinical indices were analysed using logistic regression and generalised linear models.
Findings
Between July 2022 and October 2023, 368 patients were screened, and 106 were included. Bone erosions were present in 56.7 % of patients, predominantly in the 3rd metacarpal head, with a mean volume of 5.71 mm³. Osteophytes were identified in 7.2 % of cases. Erosion volume was significantly higher in older and postmenopausal women, in those with greater damage and in those with osteophytes. Erosion volume increased by 7 % per year of age, and postmenopausal women had a 3.54-fold increase in erosion volume compared to premenopausal women. No significant associations were observed between erosions and disease indices or clinical outcomes.
Interpretation
This study shows a higher prevalence of bone erosions in SjD than previously described, particularly in older, postmenopausal women. These findings highlight the potential of HR-pQCT as a sensitive tool to detect joint damage in SjD, challenging its non-erosive classification.
{"title":"Associations of local bone involvement with disease activity, damage and functional disability in Sjögren's disease: A cross-sectional study","authors":"Andre S. Franco, Igor H. Murai, Thomas H. Yang, Liliam Takayama, Virginia L.N. Bonoldi, Valeria F. Caparbo, Lissiane K.N. Guedes, Diogo S. Domiciano, Sandra G. Pasoto, Camille P. Figueiredo, Rosa M.R. Pereira","doi":"10.1016/j.semarthrit.2025.152644","DOIUrl":"10.1016/j.semarthrit.2025.152644","url":null,"abstract":"<div><h3>Background</h3><div>Sjögren's disease (SjD) arthritis is non-erosive, but joint involvement in this disease remains unclear. This study investigated the association between bone erosions and clinical parameters using high-resolution peripheral quantitative computed tomography (HR-pQCT) in patients with SjD.</div></div><div><h3>Methods</h3><div>In this cross-sectional study at a quaternary hospital in Brazil, 106 female patients with SjD, aged 18–65 years, who met ACR/EULAR 2016 criteria were evaluated. Exclusion criteria were the use of osteoporosis medication and other bone or autoimmune diseases. Disease activity, damage, functional disability, and laboratory tests were assessed. HR-pQCT was used to identify bone erosions and osteophytes in the 2nd and 3rd metacarpophalangeal and proximal interphalangeal joints, and functional impairment was measured by the handgrip test. Associations between bone erosions, osteophytes, and clinical indices were analysed using logistic regression and generalised linear models.</div></div><div><h3>Findings</h3><div>Between July 2022 and October 2023, 368 patients were screened, and 106 were included. Bone erosions were present in 56.7 % of patients, predominantly in the 3rd metacarpal head, with a mean volume of 5.71 mm³. Osteophytes were identified in 7.2 % of cases. Erosion volume was significantly higher in older and postmenopausal women, in those with greater damage and in those with osteophytes. Erosion volume increased by 7 % per year of age, and postmenopausal women had a 3.54-fold increase in erosion volume compared to premenopausal women. No significant associations were observed between erosions and disease indices or clinical outcomes.</div></div><div><h3>Interpretation</h3><div>This study shows a higher prevalence of bone erosions in SjD than previously described, particularly in older, postmenopausal women. These findings highlight the potential of HR-pQCT as a sensitive tool to detect joint damage in SjD, challenging its non-erosive classification.</div></div>","PeriodicalId":21715,"journal":{"name":"Seminars in arthritis and rheumatism","volume":"71 ","pages":"Article 152644"},"PeriodicalIF":4.6,"publicationDate":"2025-01-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143123474","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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