Pub Date : 2024-11-01Epub Date: 2024-07-08DOI: 10.1007/s00296-024-05653-x
Gernot Kriegshäuser, Hasmik Hayrapetyan, Christian Oberkanins, Tamara Sarkisian
There is little and conflicting data on the role of the plasminogen activator inhibitor-1 (PAI-1, SERPINE1) 4G/5G polymorphism in familial Mediterranean fever (FMF). Therefore this study aimed at evaluating the impact of this polymorphism on the disease course in a cohort of 303 Armenian FMF patients. Genotyping for 12 Mediterranean fever (MEFV) gene mutations and the PAI-1 4G/5G (rs1799762) polymorphism were performed by PCR/reverse-hybridization (StripAssay) and real-time PCR, respectively. PAI-1 genotypes 4G/4G, 4G/5G, and 5G/5G could be identified in 4 (5.88%), 30 (18.63%) and 9 (12.16%) patients with erysipelas-like erythema (ELE), while this was the case for 64 (94.12%), 131 (81.37%), and 65 (87.84%) patients without ELE, respectively (P < 0.033). We have identified a significant relationship between the PAI-1 4G/5G genotype and the occurence of ELE in a relatively large cohort of Armenian FMF patients. Because of conflicting results concerning the impact of this polymorphism on the clinical course of FMF in different populations, further studies are desirable to substantiate the findings reported here.
{"title":"Plasminogen activator inhibitor-1 genotype 4G/5G associates with skin involvement in Armenian familial Mediterranean fever patients.","authors":"Gernot Kriegshäuser, Hasmik Hayrapetyan, Christian Oberkanins, Tamara Sarkisian","doi":"10.1007/s00296-024-05653-x","DOIUrl":"10.1007/s00296-024-05653-x","url":null,"abstract":"<p><p>There is little and conflicting data on the role of the plasminogen activator inhibitor-1 (PAI-1, SERPINE1) 4G/5G polymorphism in familial Mediterranean fever (FMF). Therefore this study aimed at evaluating the impact of this polymorphism on the disease course in a cohort of 303 Armenian FMF patients. Genotyping for 12 Mediterranean fever (MEFV) gene mutations and the PAI-1 4G/5G (rs1799762) polymorphism were performed by PCR/reverse-hybridization (StripAssay) and real-time PCR, respectively. PAI-1 genotypes 4G/4G, 4G/5G, and 5G/5G could be identified in 4 (5.88%), 30 (18.63%) and 9 (12.16%) patients with erysipelas-like erythema (ELE), while this was the case for 64 (94.12%), 131 (81.37%), and 65 (87.84%) patients without ELE, respectively (P < 0.033). We have identified a significant relationship between the PAI-1 4G/5G genotype and the occurence of ELE in a relatively large cohort of Armenian FMF patients. Because of conflicting results concerning the impact of this polymorphism on the clinical course of FMF in different populations, further studies are desirable to substantiate the findings reported here.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141555404","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-11-01Epub Date: 2024-07-16DOI: 10.1007/s00296-024-05661-x
Oya Koker, Sezgin Sahin, Mehmet Yildiz, Amra Adrovic, Ozgur Kasapcopur
Artificial intelligence algorithms, with roots extending into the past but experiencing a resurgence and evolution in recent years due to their superiority over traditional methods and contributions to human capabilities, have begun to make their presence felt in the field of pediatric rheumatology. In the ever-evolving realm of pediatric rheumatology, there have been incremental advancements supported by artificial intelligence in understanding and stratifying diseases, developing biomarkers, refining visual analyses, and facilitating individualized treatment approaches. However, like in many other domains, these strides have yet to gain clinical applicability and validation, and ethical issues remain unresolved. Furthermore, mastering different and novel terminologies appears challenging for clinicians. This review aims to provide a comprehensive overview of the current literature, categorizing algorithms and their applications, thus offering a fresh perspective on the nascent relationship between pediatric rheumatology and artificial intelligence, highlighting both its advancements and constraints.
{"title":"The emerging paradigm in pediatric rheumatology: harnessing the power of artificial intelligence.","authors":"Oya Koker, Sezgin Sahin, Mehmet Yildiz, Amra Adrovic, Ozgur Kasapcopur","doi":"10.1007/s00296-024-05661-x","DOIUrl":"10.1007/s00296-024-05661-x","url":null,"abstract":"<p><p>Artificial intelligence algorithms, with roots extending into the past but experiencing a resurgence and evolution in recent years due to their superiority over traditional methods and contributions to human capabilities, have begun to make their presence felt in the field of pediatric rheumatology. In the ever-evolving realm of pediatric rheumatology, there have been incremental advancements supported by artificial intelligence in understanding and stratifying diseases, developing biomarkers, refining visual analyses, and facilitating individualized treatment approaches. However, like in many other domains, these strides have yet to gain clinical applicability and validation, and ethical issues remain unresolved. Furthermore, mastering different and novel terminologies appears challenging for clinicians. This review aims to provide a comprehensive overview of the current literature, categorizing algorithms and their applications, thus offering a fresh perspective on the nascent relationship between pediatric rheumatology and artificial intelligence, highlighting both its advancements and constraints.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11424736/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141620889","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-11-01Epub Date: 2024-05-15DOI: 10.1007/s00296-024-05613-5
Sandra Garrote-Corral, Diana Botello Corzo, Jesús Loarce-Martos, Carlos de la Puente Bujidos, Loreto Carmona
Background and objective: Systemic sclerosis (SSc) is a highly heterogeneous disease whose treatment is based mainly on immunosuppressants, antifibrotics, and vasodilators. Intravenous immunoglobulin (IVIG) have proved effective in other autoimmune diseases. The objective of this study is to evaluate the efficacy and safety of IVIG in SSc.
Methods: The systematic review was conducted according to the PRISMA Statement. Medline, Embase and Cochrane Library databases were searched until March 2024. We assessed the quality of included studies using the Cochrane Risk of Bias 2.0 tool (RoB 2) for randomised clinical trials and the Cochrane Risk in non-randomized studies (ROBINS-I) tool for observational studies.
Results: From 1242 studies identified, 15 studies were included, of which 14 were observational studies. In total, 361 patients with SSc were included, and 295 received treatment with IVIG. Most of the studies used a dose of 2 g/kg IVIG. Ten studies, including the clinical trial, showed high risk of bias, and five had a critical risk. Skin involvement was assessed using modified Rodnan skin score, in 11 studies and the authors reported cutaneous efficacy in 9 of them. The 6 studies that assessed muscle involvement reported an improvement. Six studies reported data on gastrointestinal efficacy. Other domains such as lung and joint involvement and steroid-sparing effect were evaluated. The most frequent adverse events were mild, including headache, abdominal pain, fever, and skin rash.
Conclusion: Treatment with IVIG in SSc patients could be helpful and safe in patients with cutaneous, muscular, or digestive manifestations.
{"title":"Efficacy and safety of intravenous immunoglobulin therapy in systemic sclerosis: a systematic review.","authors":"Sandra Garrote-Corral, Diana Botello Corzo, Jesús Loarce-Martos, Carlos de la Puente Bujidos, Loreto Carmona","doi":"10.1007/s00296-024-05613-5","DOIUrl":"10.1007/s00296-024-05613-5","url":null,"abstract":"<p><strong>Background and objective: </strong>Systemic sclerosis (SSc) is a highly heterogeneous disease whose treatment is based mainly on immunosuppressants, antifibrotics, and vasodilators. Intravenous immunoglobulin (IVIG) have proved effective in other autoimmune diseases. The objective of this study is to evaluate the efficacy and safety of IVIG in SSc.</p><p><strong>Methods: </strong>The systematic review was conducted according to the PRISMA Statement. Medline, Embase and Cochrane Library databases were searched until March 2024. We assessed the quality of included studies using the Cochrane Risk of Bias 2.0 tool (RoB 2) for randomised clinical trials and the Cochrane Risk in non-randomized studies (ROBINS-I) tool for observational studies.</p><p><strong>Results: </strong>From 1242 studies identified, 15 studies were included, of which 14 were observational studies. In total, 361 patients with SSc were included, and 295 received treatment with IVIG. Most of the studies used a dose of 2 g/kg IVIG. Ten studies, including the clinical trial, showed high risk of bias, and five had a critical risk. Skin involvement was assessed using modified Rodnan skin score, in 11 studies and the authors reported cutaneous efficacy in 9 of them. The 6 studies that assessed muscle involvement reported an improvement. Six studies reported data on gastrointestinal efficacy. Other domains such as lung and joint involvement and steroid-sparing effect were evaluated. The most frequent adverse events were mild, including headache, abdominal pain, fever, and skin rash.</p><p><strong>Conclusion: </strong>Treatment with IVIG in SSc patients could be helpful and safe in patients with cutaneous, muscular, or digestive manifestations.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140923116","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-11-01Epub Date: 2024-07-09DOI: 10.1007/s00296-024-05646-w
Kerem Yiğit Abacar, Şeyma Çolakoğlu-Özkaya, Erhan Bıyıklı, Onur Buğdaycı, Meltem Kurşun, Ayberk Denizli, Beril Koçak, Aysun Aksoy, Can Erzik, Pınar Ay, Murat Bezer, Mehmet Tuncay Duruöz, Haner Direskeneli, Pamir Atagündüz
Radiographic progression in Ankylosing spondylitis (AS) is driven by mechanical strain. A well-balanced spine provides a favorable weight distribution across the entheses. Pelvic parameters are useful in assessing the shape of the spine. The present study aimed to prospectively investigate the predictive value of pelvic parameters for radiographic progression in AS. This non-interventional, observational, and prospective study enrolled AS patients fulfilling the modified New York criteria (mNY) currently under follow-up in the MARS (MARmara Spondyloarthritis) outpatient clinics. The primary objective was to investigate the relationship between the baseline pelvic parameters and radiographic progression in the spine. Two trained radiologists (EB, OB) independently assessed the modified Stoke Ankylosing Spondylitis Spine Score (mSASSS). An orthopedic surgeon (AHA) and a radiologist (EB) derived the pelvic parameters. Patients with no bridging or bamboo spine were included in the final analysis. Risk assessment for radiographic progression, defined as a two-unit increase in mSASSS or developing a new syndesmophyte every two years, was done using uni- and multivariate logistic regression analyses. Radiographs of 69 AS patients were analyzed. The median (IQR 25-75) prospective follow-up was 47.7 (34.6-52.8) months. Only 33.3% (23/69) had radiographic progression. The pelvic tilt (PT) was lower in patients with radiographic progression (p = 0.037) and each degree of decrease in PT provided a 9% increase in risk for radiographic progression. Male patients were 7.5 times more likely to progress. Pelvic parameters provide a prognostic insight into the radiographic progression in AS. Our observations may aid in selecting patient-specific interventions in addition to anti-inflammatory treatments.
强直性脊柱炎(AS)的放射学进展是由机械应变驱动的。平衡良好的脊柱能使重量在各关节间得到良好的分配。骨盆参数有助于评估脊柱的形态。本研究旨在前瞻性地调查骨盆参数对强直性脊柱炎放射学进展的预测价值。这项非干预性、观察性和前瞻性研究招募了符合改良纽约标准(mNY)的强直性脊柱炎患者,他们目前正在MARS(MARmara Spondyloarthritis)门诊接受随访。主要目的是研究骨盆基线参数与脊柱放射学进展之间的关系。两名训练有素的放射科医生(EB、OB)独立评估改良斯托克强直性脊柱炎脊柱评分(mSASSS)。一名骨科医生(AHA)和一名放射科医生(EB)得出骨盆参数。无桥接或竹节状脊柱的患者被纳入最终分析。采用单变量和多变量逻辑回归分析对放射学进展进行风险评估,即 mSASSS 每两年增加两个单位或出现新的联合骨赘。对 69 名 AS 患者的 X 光片进行了分析。前瞻性随访的中位数(IQR 25-75)为 47.7(34.6-52.8)个月。只有 33.3%(23/69)的患者出现放射学进展。影像学进展患者的骨盆倾斜度(PT)较低(P = 0.037),PT每降低1度,影像学进展的风险就增加9%。男性患者病情恶化的几率是前者的 7.5 倍。骨盆参数提供了对强直性脊柱炎放射学进展的预后洞察力。除了抗炎治疗外,我们的观察结果可能有助于选择针对患者的干预措施。
{"title":"Pelvic parameters as prognostic factors of radiographic progression in classical Ankylosing Spondylitis: A prospective follow-up data.","authors":"Kerem Yiğit Abacar, Şeyma Çolakoğlu-Özkaya, Erhan Bıyıklı, Onur Buğdaycı, Meltem Kurşun, Ayberk Denizli, Beril Koçak, Aysun Aksoy, Can Erzik, Pınar Ay, Murat Bezer, Mehmet Tuncay Duruöz, Haner Direskeneli, Pamir Atagündüz","doi":"10.1007/s00296-024-05646-w","DOIUrl":"10.1007/s00296-024-05646-w","url":null,"abstract":"<p><p>Radiographic progression in Ankylosing spondylitis (AS) is driven by mechanical strain. A well-balanced spine provides a favorable weight distribution across the entheses. Pelvic parameters are useful in assessing the shape of the spine. The present study aimed to prospectively investigate the predictive value of pelvic parameters for radiographic progression in AS. This non-interventional, observational, and prospective study enrolled AS patients fulfilling the modified New York criteria (mNY) currently under follow-up in the MARS (MARmara Spondyloarthritis) outpatient clinics. The primary objective was to investigate the relationship between the baseline pelvic parameters and radiographic progression in the spine. Two trained radiologists (EB, OB) independently assessed the modified Stoke Ankylosing Spondylitis Spine Score (mSASSS). An orthopedic surgeon (AHA) and a radiologist (EB) derived the pelvic parameters. Patients with no bridging or bamboo spine were included in the final analysis. Risk assessment for radiographic progression, defined as a two-unit increase in mSASSS or developing a new syndesmophyte every two years, was done using uni- and multivariate logistic regression analyses. Radiographs of 69 AS patients were analyzed. The median (IQR 25-75) prospective follow-up was 47.7 (34.6-52.8) months. Only 33.3% (23/69) had radiographic progression. The pelvic tilt (PT) was lower in patients with radiographic progression (p = 0.037) and each degree of decrease in PT provided a 9% increase in risk for radiographic progression. Male patients were 7.5 times more likely to progress. Pelvic parameters provide a prognostic insight into the radiographic progression in AS. Our observations may aid in selecting patient-specific interventions in addition to anti-inflammatory treatments.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141564190","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-11-01Epub Date: 2024-08-13DOI: 10.1007/s00296-024-05664-8
Kristian Vogt, Christian Bijan Fink, Teresa Maria Schreibing, Stefan Krämer, Sebastian Reinartz, Thomas Rauen
ANCA-associated vasculitides (AAV) comprise granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis. All forms may involve different organ systems, yet kidney and lung involvement are common and fatal in many cases. Here, we aimed to determine the predictive value of pulmonary disease manifestation and individual CT findings in AAV patients. Available CT scans and clinical information on mortality, renal outcomes, occurrence of relapses and damage scores were analysed retrospectively from a tertiary rheumatology center in Germany. We included a total of 94 AAV patients (49 with GPA, 41 with MPA). Forty-four patients had lung involvement with available CT scans, 70.5% of which with GPA and 72.7% with renal involvement. Nodule formation and cavities were more frequent among GPA patients, whereas ground-glass opacities (GGO), ILD and pleural effusion were observed predominantly in MPA patients. Over a median follow-up of 37 months, GPA patients had a slightly higher overall mortality, whereas end-stage kidney failure rates were significantly increased in MPA patients. Relapse frequencies were comparable between both entities. The presence of GGO and pleural effusion were associated with higher relapse rates, whereas nodules were negatively correlated with relapses. Notably, RTX-treated patients had less infections as compared to individuals under different therapies. Our data demonstrate the outstanding importance of characteristic CT patterns in AAV diagnosis assessment. Especially certain CT patterns including GGO and pleura effusion may help to identify patients who are at higher risk for relapsing disease.
{"title":"Distinct pulmonary patterns in ANCA-associated vasculitides: insights from a retrospective single center cohort study.","authors":"Kristian Vogt, Christian Bijan Fink, Teresa Maria Schreibing, Stefan Krämer, Sebastian Reinartz, Thomas Rauen","doi":"10.1007/s00296-024-05664-8","DOIUrl":"10.1007/s00296-024-05664-8","url":null,"abstract":"<p><p>ANCA-associated vasculitides (AAV) comprise granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis. All forms may involve different organ systems, yet kidney and lung involvement are common and fatal in many cases. Here, we aimed to determine the predictive value of pulmonary disease manifestation and individual CT findings in AAV patients. Available CT scans and clinical information on mortality, renal outcomes, occurrence of relapses and damage scores were analysed retrospectively from a tertiary rheumatology center in Germany. We included a total of 94 AAV patients (49 with GPA, 41 with MPA). Forty-four patients had lung involvement with available CT scans, 70.5% of which with GPA and 72.7% with renal involvement. Nodule formation and cavities were more frequent among GPA patients, whereas ground-glass opacities (GGO), ILD and pleural effusion were observed predominantly in MPA patients. Over a median follow-up of 37 months, GPA patients had a slightly higher overall mortality, whereas end-stage kidney failure rates were significantly increased in MPA patients. Relapse frequencies were comparable between both entities. The presence of GGO and pleural effusion were associated with higher relapse rates, whereas nodules were negatively correlated with relapses. Notably, RTX-treated patients had less infections as compared to individuals under different therapies. Our data demonstrate the outstanding importance of characteristic CT patterns in AAV diagnosis assessment. Especially certain CT patterns including GGO and pleura effusion may help to identify patients who are at higher risk for relapsing disease.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141971755","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-11-01Epub Date: 2024-08-24DOI: 10.1007/s00296-024-05684-4
María Jesús Garcia-Villanueva, Sandra Garrote-Corral, Jose María Pego-Reigosa, Norman Jiménez Otero, Esther Uriarte Isazelaia, Alejandro Olivé Marqué, Clara Sangüesa Gómez, Mercedes Freire González, Elena Aurrecoechea Aguinaga, Enrique Raya Álvarez, Eva Tomero Muriel, Carlos Montilla Morales, María Galindo Izquierdo, Jaime Calvo-Alén, Raúl Menor-Almagro, Belén Serrano Benavente, Julia Martinez-Barrio, Jose Angel Hernández-Beriain, Mónica Ibañez Barceló, Gema Bonilla Hernan, Jose Rosas, Eva Salgado Pérez, Antonio Fernández-Nebro, Iñigo Rua-Figueroa
Introduction: Diffuse alveolar hemorrhage (DAH) is a rare complication with high mortality in patients with systemic lupus erythematosus (SLE). Early diagnosis and treatment are essential to improve patient prognosis. To determine the characteristics of patients with DAH and their mortality in a Spanish cohort of patients with SLE.
Methods: Patients from the RELESSER (Spanish Society of Rheumatology Lupus Register) who had had at least one confirmed episode of DAH were included. Epidemiological, clinical, and laboratory characteristics were analyzed.
Results: 4024 patients were included in the RELESSER register, 37 (0.9%), had at least one recorded episode of DAH. Only further data for 14 patients could be analyzed. In total, 92.9% were women, and for 4 (28.6%) DAH coincided with the debut of SLE. More than 80% of patients had renal involvement and thrombocytopenia. The most frequent manifestations were dyspnea (85.7%) and hypoxemia (100%), with the classic triad of hemoptysis, anemia and pulmonary infiltrates, appearing in 6 (46.2%) patients. The most frequently used treatments were glucocorticoids (85.7%) and cyclophosphamide (69.2%); plasmapheresis was utilized in 5 patients (35.7%) and 8, (57.1%) received intravenous immunoglobulins; 12 (85.7%) patients required admission to the ICU and 5 (35.7%) died. Tobacco use, history of lupus nephritis (LN), concomitant infection, and treatment with cyclophosphamide were more frequent in patients who died.
Conclusions: DAH is rare in patients with SLE; in up to one-third of patients, it may appear at the onset of the disease. Some factors, such as smoking, a history of LN, treatment with cyclophosphamide, or concomitant infection, are more prevalent in patients with an unfavorable outcome.
{"title":"Diffuse alveolar hemorrhage in patients with systemic lupus erythematosus: data from the Spanish society of rheumathology Lupus Register (RELESSER).","authors":"María Jesús Garcia-Villanueva, Sandra Garrote-Corral, Jose María Pego-Reigosa, Norman Jiménez Otero, Esther Uriarte Isazelaia, Alejandro Olivé Marqué, Clara Sangüesa Gómez, Mercedes Freire González, Elena Aurrecoechea Aguinaga, Enrique Raya Álvarez, Eva Tomero Muriel, Carlos Montilla Morales, María Galindo Izquierdo, Jaime Calvo-Alén, Raúl Menor-Almagro, Belén Serrano Benavente, Julia Martinez-Barrio, Jose Angel Hernández-Beriain, Mónica Ibañez Barceló, Gema Bonilla Hernan, Jose Rosas, Eva Salgado Pérez, Antonio Fernández-Nebro, Iñigo Rua-Figueroa","doi":"10.1007/s00296-024-05684-4","DOIUrl":"10.1007/s00296-024-05684-4","url":null,"abstract":"<p><strong>Introduction: </strong> Diffuse alveolar hemorrhage (DAH) is a rare complication with high mortality in patients with systemic lupus erythematosus (SLE). Early diagnosis and treatment are essential to improve patient prognosis. To determine the characteristics of patients with DAH and their mortality in a Spanish cohort of patients with SLE.</p><p><strong>Methods: </strong> Patients from the RELESSER (Spanish Society of Rheumatology Lupus Register) who had had at least one confirmed episode of DAH were included. Epidemiological, clinical, and laboratory characteristics were analyzed.</p><p><strong>Results: </strong> 4024 patients were included in the RELESSER register, 37 (0.9%), had at least one recorded episode of DAH. Only further data for 14 patients could be analyzed. In total, 92.9% were women, and for 4 (28.6%) DAH coincided with the debut of SLE. More than 80% of patients had renal involvement and thrombocytopenia. The most frequent manifestations were dyspnea (85.7%) and hypoxemia (100%), with the classic triad of hemoptysis, anemia and pulmonary infiltrates, appearing in 6 (46.2%) patients. The most frequently used treatments were glucocorticoids (85.7%) and cyclophosphamide (69.2%); plasmapheresis was utilized in 5 patients (35.7%) and 8, (57.1%) received intravenous immunoglobulins; 12 (85.7%) patients required admission to the ICU and 5 (35.7%) died. Tobacco use, history of lupus nephritis (LN), concomitant infection, and treatment with cyclophosphamide were more frequent in patients who died.</p><p><strong>Conclusions: </strong> DAH is rare in patients with SLE; in up to one-third of patients, it may appear at the onset of the disease. Some factors, such as smoking, a history of LN, treatment with cyclophosphamide, or concomitant infection, are more prevalent in patients with an unfavorable outcome.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142047121","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-11-01Epub Date: 2023-08-19DOI: 10.1007/s00296-023-05396-1
Donya Nemati, Daniel Quintero, Thomas M Best, Navin Kaushal
Knee osteoarthritis (KOA) is a chronic disease accompanied by debilitating symptoms including pain, stiffness, and limited physical functionality, which have been shown to be associated with pain catastrophizing. Previous studies have revealed racial discrepancies in pain catastrophizing, notably between Hispanics and non-Hispanics while pointing to potential health disparities. Using a conceptual model, this study aimed to investigate racial differences in associations between KOA symptoms with specific pain catastrophizing domains (rumination, magnification, and helplessness). Patients with KOA (n = 253; 147 Hispanics, 106 non-Hispanic Whites) completed a survey that included measures of knee symptoms, pain catastrophizing, and demographic variables. Structural equation modeling revealed that among Hispanics, each pain catastrophizing domain (rumination, magnification, and helplessness) was associated with at least two symptomatic experiences, including pain severity and difficulty in physical function. Specifically, pain severity was associated with (a) rumination: β = 0.48, p < 0.001, (b) magnification: β = 0.31, p = 0.003; and (c) helplessness: β = 0.39, p < 0.001). Additionally, a lower score in physical function was associated with higher magnification (β = 0.26, p = 0.01), and helplessness (β = 0.25, p = 0.01). Among non-Hispanic White patients, pain severity was further associated with two domains of pain catastrophizing, including rumination (β = 0.39, p < 0.001) and helplessness (β = 0.35, p = 0.01). In addition, association pathways for demographic variables revealed that older Hispanics experienced greater challenges with higher pain severity (β = 0.26, p = 0.01) and greater difficulty with physical function (β = 0.31, p < 0.001) while Hispanics females experienced higher pain (β = 0.19, p = 0.03). These findings highlight the importance of designing tailored interventions that consider key demographic factors such as age, and gender, to improve physical function that might alleviate pain catastrophizing among Hispanics with KOA.
{"title":"Investigating the association between knee osteoarthritis symptoms with pain catastrophizing domains between Hispanics and non-Hispanic Whites.","authors":"Donya Nemati, Daniel Quintero, Thomas M Best, Navin Kaushal","doi":"10.1007/s00296-023-05396-1","DOIUrl":"10.1007/s00296-023-05396-1","url":null,"abstract":"<p><p>Knee osteoarthritis (KOA) is a chronic disease accompanied by debilitating symptoms including pain, stiffness, and limited physical functionality, which have been shown to be associated with pain catastrophizing. Previous studies have revealed racial discrepancies in pain catastrophizing, notably between Hispanics and non-Hispanics while pointing to potential health disparities. Using a conceptual model, this study aimed to investigate racial differences in associations between KOA symptoms with specific pain catastrophizing domains (rumination, magnification, and helplessness). Patients with KOA (n = 253; 147 Hispanics, 106 non-Hispanic Whites) completed a survey that included measures of knee symptoms, pain catastrophizing, and demographic variables. Structural equation modeling revealed that among Hispanics, each pain catastrophizing domain (rumination, magnification, and helplessness) was associated with at least two symptomatic experiences, including pain severity and difficulty in physical function. Specifically, pain severity was associated with (a) rumination: β = 0.48, p < 0.001, (b) magnification: β = 0.31, p = 0.003; and (c) helplessness: β = 0.39, p < 0.001). Additionally, a lower score in physical function was associated with higher magnification (β = 0.26, p = 0.01), and helplessness (β = 0.25, p = 0.01). Among non-Hispanic White patients, pain severity was further associated with two domains of pain catastrophizing, including rumination (β = 0.39, p < 0.001) and helplessness (β = 0.35, p = 0.01). In addition, association pathways for demographic variables revealed that older Hispanics experienced greater challenges with higher pain severity (β = 0.26, p = 0.01) and greater difficulty with physical function (β = 0.31, p < 0.001) while Hispanics females experienced higher pain (β = 0.19, p = 0.03). These findings highlight the importance of designing tailored interventions that consider key demographic factors such as age, and gender, to improve physical function that might alleviate pain catastrophizing among Hispanics with KOA.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10018101","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-11-01Epub Date: 2024-09-09DOI: 10.1007/s00296-024-05717-y
Agustín Hernández-López, Yatzil Reyna-Juárez, María José Ostos-Prado, Beatriz Alcalá-Carmona, Jiram Torres-Ruiz, Silvia Méndez-Flores, Salvador Escobar-Ceballos, Braulio Martínez-Benitez, Diana Gómez-Martín
Anti-synthetase syndrome (AS) is a subset of idiopathic inflammatory myopathy (IIM) characterized by the presence of anti-aminoacyl-transfer RNA synthetase accompanied by myositis, interstitial lung disease and other clinical features. According to a recent multicentric study, 31% of AS patients present skin lesions compatible with dermatomyositis, but sclerodermiform features are rare. Therefore, we aimed to report the case of a patient with simultaneous diagnosis of AS, deep morphea, vasculitic neuropathy, and myelodysplastic syndrome and review the current literature regarding these uncommon associations. A 57 year old man with axial and symmetrical proximal muscle weakness, skin thickening and B symptoms, later diagnosed with PL7 + AS, deep morphea, myelodysplastic syndrome (MDS) and vasculitic neuropathy documented by histopathologic studies and immunologic assessments. Since both AS and deep morphea share the vasculopathic changes and type II interferon-induced inflammation, we hypothesize that they may share pathogenic mechanisms. The muscle biopsy of the patient was consistent with AS and showed focal neutrophil infiltration. The patient received intensive immunosuppressive therapy for AS and vasculitic neuropathy, with high dose steroids, intravenous immunoglobulin (IVIg) and rituximab. Nonetheless, he suffered an unfavorable evolution with a fatal outcome due to septic shock. Albeit sclerodermiform features are rare in patients with AS, we propose a pathogenic link among AS, deep morphea and the autoimmune/autoinflammatory signs of MDS. The vasculopathic changes along with the activation of the innate and adaptive immune system leading to the production of proinflammatory cytokines may have been one of the contributing factors for the coexisting diagnosis of the patient.
抗合成酶综合征(AS)是特发性炎症性肌病(IIM)的一个分支,其特点是存在抗氨基酸酰转移核糖核酸合成酶,并伴有肌炎、间质性肺病和其他临床特征。根据最近的一项多中心研究,31%的强直性脊柱炎患者出现了与皮肌炎相符的皮肤病变,但硬皮样特征却很少见。因此,我们旨在报告一例同时被诊断为强直性脊柱炎、深部病变、血管神经病和骨髓增生异常综合征的患者,并回顾目前有关这些不常见关联的文献。一名 57 岁的男子患有轴性和对称性近端肌无力、皮肤增厚和 B 症状,后经组织病理学研究和免疫学评估确诊为 PL7 + AS、深部病变、骨髓增生异常综合征(MDS)和血管炎性神经病。由于强直性脊柱炎和深度病变都具有血管病理变化和II型干扰素诱导的炎症,我们推测它们可能具有相同的致病机制。患者的肌肉活检结果与强直性脊柱炎一致,并显示局灶性中性粒细胞浸润。患者因强直性脊柱炎和血管性神经病接受了强化免疫抑制治疗,包括大剂量类固醇、静脉注射免疫球蛋白(IVIg)和利妥昔单抗。然而,他的病情发展并不乐观,最终因脓毒性休克而死亡。尽管硬皮样特征在强直性脊柱炎患者中很少见,但我们认为强直性脊柱炎、深部病变和MDS的自身免疫/自体炎症症状之间存在致病联系。血管病理变化以及先天性和适应性免疫系统的激活导致了促炎细胞因子的产生,这可能是导致患者同时被诊断为强直性脊柱炎的原因之一。
{"title":"Anti-synthetase and myelodysplastic syndromes with deep morphea: an example of shared immunopathogenesis? A case-based review.","authors":"Agustín Hernández-López, Yatzil Reyna-Juárez, María José Ostos-Prado, Beatriz Alcalá-Carmona, Jiram Torres-Ruiz, Silvia Méndez-Flores, Salvador Escobar-Ceballos, Braulio Martínez-Benitez, Diana Gómez-Martín","doi":"10.1007/s00296-024-05717-y","DOIUrl":"10.1007/s00296-024-05717-y","url":null,"abstract":"<p><p>Anti-synthetase syndrome (AS) is a subset of idiopathic inflammatory myopathy (IIM) characterized by the presence of anti-aminoacyl-transfer RNA synthetase accompanied by myositis, interstitial lung disease and other clinical features. According to a recent multicentric study, 31% of AS patients present skin lesions compatible with dermatomyositis, but sclerodermiform features are rare. Therefore, we aimed to report the case of a patient with simultaneous diagnosis of AS, deep morphea, vasculitic neuropathy, and myelodysplastic syndrome and review the current literature regarding these uncommon associations. A 57 year old man with axial and symmetrical proximal muscle weakness, skin thickening and B symptoms, later diagnosed with PL7 + AS, deep morphea, myelodysplastic syndrome (MDS) and vasculitic neuropathy documented by histopathologic studies and immunologic assessments. Since both AS and deep morphea share the vasculopathic changes and type II interferon-induced inflammation, we hypothesize that they may share pathogenic mechanisms. The muscle biopsy of the patient was consistent with AS and showed focal neutrophil infiltration. The patient received intensive immunosuppressive therapy for AS and vasculitic neuropathy, with high dose steroids, intravenous immunoglobulin (IVIg) and rituximab. Nonetheless, he suffered an unfavorable evolution with a fatal outcome due to septic shock. Albeit sclerodermiform features are rare in patients with AS, we propose a pathogenic link among AS, deep morphea and the autoimmune/autoinflammatory signs of MDS. The vasculopathic changes along with the activation of the innate and adaptive immune system leading to the production of proinflammatory cytokines may have been one of the contributing factors for the coexisting diagnosis of the patient.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142154944","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-11-01Epub Date: 2024-09-08DOI: 10.1007/s00296-024-05708-z
Güllü Sandal Uzun, Özay Gököz, Betül Oğüt, Aylin Heper, Servet Güreşçi, Rıza Can Kardaş, Mehmet Akif Öztürk, Emine Uslu, Aşkın Ateş, Berkan Armağan, Ahmet Omma, Levent Kılıc, Omer Karadag
Histopathological findings associated with definite vasculitis in temporal artery biopsy (TAB) defined in 2022 ACR/EULAR classification criteria for Giant Cell Arteritis (GCA) was published in 2022. We aimed to evaluate the TAB of our GCA patients for histopathological findings associated with definite vasculitis. Patients who were diagnosed with GCA by clinicians and underwent TAB between January 2012 and May 2022 were included. Hospital electronic records and patients' files were reviewed retrospectively. A total of 90 patients' pathology reports were evaluated by a pathologist and a rheumatologist. In cases where microscopic findings were not specified in the pathology reports, histopathologic specimens were re-evaluated (n = 36). A standard checklist was used for histopathological findings of definite vasculitis. Patients were divided into two groups; (i) definite vasculitis-GCA and (ii) non-definite-GCA group, and the clinical and demographic characteristics for all patients were compared. The mean age of patients was 69.8 (± 8.5) years and 52.2% were female. In the first evaluation, 66 (73.3%) patients had a diagnosis of vasculitis according to pathology reports. In the re-evaluation of biopsy specimens, at least one definite finding of vasculitis was observed in TAB of 10/24 (41.6%) patients whose microscopic findings were not specified in the pathology reports. The ROC analysis showed that biopsy length had diagnostic value in predicting the diagnosis of definite vasculitis (AUC: 0.778, 95% CI: 0.65-0.89, p < 0.001). In those with a biopsy length of ≥ 1 cm, sensitivity was 76.5%, specificity was 64.3%, and PPV value was 92. In multivariate analysis, the most significant factor associated with definite vasculitis was biopsy length (OR: 1.18 (1.06-1.31), p = 0.002). Microscopic findings were reported in over 70% of patients. Reinterpretation of results according to a standard check-list improved the impact of TAB in the diagnosis of GCA. A biopsy length ≥ 1 cm was found to contribute towards a definitive histopathological vasculitis diagnosis.
{"title":"The impact of histopathological criteria for definite vasculitis in giant cell arteritis: retrospective analysis of temporal artery biopsies.","authors":"Güllü Sandal Uzun, Özay Gököz, Betül Oğüt, Aylin Heper, Servet Güreşçi, Rıza Can Kardaş, Mehmet Akif Öztürk, Emine Uslu, Aşkın Ateş, Berkan Armağan, Ahmet Omma, Levent Kılıc, Omer Karadag","doi":"10.1007/s00296-024-05708-z","DOIUrl":"10.1007/s00296-024-05708-z","url":null,"abstract":"<p><p>Histopathological findings associated with definite vasculitis in temporal artery biopsy (TAB) defined in 2022 ACR/EULAR classification criteria for Giant Cell Arteritis (GCA) was published in 2022. We aimed to evaluate the TAB of our GCA patients for histopathological findings associated with definite vasculitis. Patients who were diagnosed with GCA by clinicians and underwent TAB between January 2012 and May 2022 were included. Hospital electronic records and patients' files were reviewed retrospectively. A total of 90 patients' pathology reports were evaluated by a pathologist and a rheumatologist. In cases where microscopic findings were not specified in the pathology reports, histopathologic specimens were re-evaluated (n = 36). A standard checklist was used for histopathological findings of definite vasculitis. Patients were divided into two groups; (i) definite vasculitis-GCA and (ii) non-definite-GCA group, and the clinical and demographic characteristics for all patients were compared. The mean age of patients was 69.8 (± 8.5) years and 52.2% were female. In the first evaluation, 66 (73.3%) patients had a diagnosis of vasculitis according to pathology reports. In the re-evaluation of biopsy specimens, at least one definite finding of vasculitis was observed in TAB of 10/24 (41.6%) patients whose microscopic findings were not specified in the pathology reports. The ROC analysis showed that biopsy length had diagnostic value in predicting the diagnosis of definite vasculitis (AUC: 0.778, 95% CI: 0.65-0.89, p < 0.001). In those with a biopsy length of ≥ 1 cm, sensitivity was 76.5%, specificity was 64.3%, and PPV value was 92. In multivariate analysis, the most significant factor associated with definite vasculitis was biopsy length (OR: 1.18 (1.06-1.31), p = 0.002). Microscopic findings were reported in over 70% of patients. Reinterpretation of results according to a standard check-list improved the impact of TAB in the diagnosis of GCA. A biopsy length ≥ 1 cm was found to contribute towards a definitive histopathological vasculitis diagnosis.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142154947","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-11-01Epub Date: 2023-09-21DOI: 10.1007/s00296-023-05468-2
Yeo-Jin Lee, Sang Hoon Lee, Soo-Min Ahn, Seokchan Hong, Ji-Seon Oh, Chang-Keun Lee, Bin Yoo, Yong-Gil Kim
We aimed to identify when magnetic resonance imaging (MRI) would be useful to diagnose patients with suspected axial spondyloarthropathy (AxSpA) without evidence of sacroiliitis on radiographs. We retrospectively reviewed electronic medical records of patients who underwent pelvis MRI after radiographs at the rheumatology clinic in a single tertiary center in Korea. Patients underwent imaging from January 2020 to July 2022. We collected data including complete blood count, erythrocyte sedimentation rate, C-reactive protein (CRP), human leukocyte antigen (HLA)-B27, history of acute anterior uveitis (AAU), peripheral arthritis, dactylitis, inflammatory bowel disease (IBD), enthesopathy, and psoriasis. A total of 105 patients who showed no evidence of sacroiliitis on radiographs were included. The median age of patients was 41.0 years, and 44.8% were male. Of them, 34 showed sacroiliitis on MRI (group 1), and 71 showed no evidence of sacroiliitis even on MRI (group 2). Known AxSpA-related clinical features including AAU, peripheral arthritis, dactylitis, IBD, enthesopathy, and psoriasis were not different between the two groups. HLA-B27 positivity (79.4% vs. 40.0%, p < 0.001), median white blood cell count (7700 vs. 6300, p = 0.007), mean platelet count (307.7 ± 69.7 vs. 265.3 ± 68.9 × 103/µL, p = 0.005), and median CRP level (0.38 vs. 0.10, p = 0.001) showed significant differences between the two groups. In a multivariate analysis, HLA-B27 positivity and platelet count were significantly associated with sacroiliitis on MRI. In our cohort, sacroiliitis was observed on MRI in one-third of patients without radiographic evidence. MRI could be recommended to evaluate sacroiliitis in patients with positive HLA-B27 and a high platelet count.
{"title":"When MRI would be useful in patients without evidence of sacroiliitis on radiographs?","authors":"Yeo-Jin Lee, Sang Hoon Lee, Soo-Min Ahn, Seokchan Hong, Ji-Seon Oh, Chang-Keun Lee, Bin Yoo, Yong-Gil Kim","doi":"10.1007/s00296-023-05468-2","DOIUrl":"10.1007/s00296-023-05468-2","url":null,"abstract":"<p><p>We aimed to identify when magnetic resonance imaging (MRI) would be useful to diagnose patients with suspected axial spondyloarthropathy (AxSpA) without evidence of sacroiliitis on radiographs. We retrospectively reviewed electronic medical records of patients who underwent pelvis MRI after radiographs at the rheumatology clinic in a single tertiary center in Korea. Patients underwent imaging from January 2020 to July 2022. We collected data including complete blood count, erythrocyte sedimentation rate, C-reactive protein (CRP), human leukocyte antigen (HLA)-B27, history of acute anterior uveitis (AAU), peripheral arthritis, dactylitis, inflammatory bowel disease (IBD), enthesopathy, and psoriasis. A total of 105 patients who showed no evidence of sacroiliitis on radiographs were included. The median age of patients was 41.0 years, and 44.8% were male. Of them, 34 showed sacroiliitis on MRI (group 1), and 71 showed no evidence of sacroiliitis even on MRI (group 2). Known AxSpA-related clinical features including AAU, peripheral arthritis, dactylitis, IBD, enthesopathy, and psoriasis were not different between the two groups. HLA-B27 positivity (79.4% vs. 40.0%, p < 0.001), median white blood cell count (7700 vs. 6300, p = 0.007), mean platelet count (307.7 ± 69.7 vs. 265.3 ± 68.9 × 10<sup>3</sup>/µL, p = 0.005), and median CRP level (0.38 vs. 0.10, p = 0.001) showed significant differences between the two groups. In a multivariate analysis, HLA-B27 positivity and platelet count were significantly associated with sacroiliitis on MRI. In our cohort, sacroiliitis was observed on MRI in one-third of patients without radiographic evidence. MRI could be recommended to evaluate sacroiliitis in patients with positive HLA-B27 and a high platelet count.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41139521","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}