Lucas Castro Pires, Alisson Pugliesi, Cleandro Pires de Albuquerque, Vitor Alves Cruz, Manoel Barros Bertolo, Ana Paula Monteiro Gomides Reis, Rina Dalva Neubarth Giorgi, Leticia Rocha Pereira, Sebastião Cezar Radominski, Ivanio Alves Pereira, Maria Fernanda Resende Guimarães, Paulo Louzada Junior, Maria de Fátima Lobato da Cunha Sauma, Karina Rossi Bonfiglioli, Claiton Viegas Brenol, Licia Maria Henrique da Mota, Geraldo da Rocha Castelar Pinheiro
{"title":"Are global definitions enough? Revisiting CDAI and SDAI remission cut-offs in Brazilian Rheumatoid Arthritis patients.","authors":"Lucas Castro Pires, Alisson Pugliesi, Cleandro Pires de Albuquerque, Vitor Alves Cruz, Manoel Barros Bertolo, Ana Paula Monteiro Gomides Reis, Rina Dalva Neubarth Giorgi, Leticia Rocha Pereira, Sebastião Cezar Radominski, Ivanio Alves Pereira, Maria Fernanda Resende Guimarães, Paulo Louzada Junior, Maria de Fátima Lobato da Cunha Sauma, Karina Rossi Bonfiglioli, Claiton Viegas Brenol, Licia Maria Henrique da Mota, Geraldo da Rocha Castelar Pinheiro","doi":"10.1186/s42358-026-00532-4","DOIUrl":"10.1186/s42358-026-00532-4","url":null,"abstract":"<p><p>BACKGROUND: Rheumatoid arthritis (RA) is a chronic systemic inflammatory disease in which achieving remission is the most effective strategy to prevent progression and optimize long-term outcomes. The performance of commonly used disease activity indices has not been well validated in the Brazilian RA population. This study aimed to evaluate the agreement between CDAI/SDAI and the revised Boolean 2.0 remission criteria, which served as the reference standard, and to identify the most accurate CDAI and SDAI remission cut-offs in this population. METHODS: We conducted a cross-sectional analysis of baseline data from a Brazilian Cohort study, which included 840 patients from 11 public hospitals in Brazil. Disease activity was assessed using DAS28-CRP, DAS28-ESR, SDAI, CDAI, and Boolean 1.0/2.0. Agreement was assessed using Cohen’s kappa, and optimal remission cut-offs were determined through ROC curve analysis. RESULTS: The study population was predominantly female (89.8%), with a mean age of 57 years and a median disease duration of 12 years. DAS28-CRP showed the highest remission rate (39.2%), whereas Boolean 1.0 showed the lowest (15.1%). Strong agreement was found between Boolean 2.0, and both the SDAI (κ = 0.775) and CDAI (κ = 0.692). ROC analysis revealed that the most accurate remission cut-offs were SDAI ≤ 4.3 and CDAI ≤ 3.9, which increased remission detection by 5.9% and 6.2%, respectively. CONCLUSION: In our cohort, SDAI ≤ 4.3 and CDAI ≤ 3.9 were the values most closely aligned with Boolean 2.0 remission. These adjusted cut-offs may help minimize overtreatment in resource-limited settings. Prospective studies assessing function, radiographic progression, and quality of life are warranted to confirm their validity in the Brazilian population. </p>","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2026-03-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13488623/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147460735","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Rheumatoid arthritis is associated with chronic dizziness, recurrent falls, and chronic imbalance: results from the Korean National Health and Nutrition Examination Survey.","authors":"Hyemin Jeong, Chan Hong Jeon, Young-Soo Chang","doi":"10.1186/s42358-026-00533-3","DOIUrl":"10.1186/s42358-026-00533-3","url":null,"abstract":"<p><p>BACKGROUND: Rheumatoid arthritis (RA) is a chronic systemic inflammatory disease that leads to various systemic complications. This study aims to assess the association between RA and chronic dizziness, recurrent falls, and chronic imbalance in the Korean adult population. METHODS: Data from 4,754 (weighted n = 17,694,932) individuals aged 40 years or older in the 2019–2020 Korean National Health and Nutrition Examination Survey were analyzed. A questionnaire including information on chronic dizziness, recurrent falls, demographic variables, and laboratory test data was evaluated. RA was defined based on patient-reported physician diagnoses. Factors potentially associated with chronic dizziness, recurrent falls, and chronic imbalance were examined. RESULTS: The mean age was 56.7 years. The weighted prevalence of RA was 1.9%. In patients with RA, the rates of chronic dizziness (10.1% vs. 3.4%, p = 0.005), recurrent falls (3.9% vs. 0.4%, p < 0.001), and chronic imbalance (5.1% vs. 1.0%, p < 0.001) were significantly higher than in those without RA. In multivariable analysis, RA (OR 2.65, 95% CI 1.10–6.34, p = 0.030) was associated with an increased risk of chronic dizziness, alongside females, a body mass index under 25, hearing impairment, stroke, and stress. Additionally, females, hearing impairment, and RA (OR 8.29, 95% CI 2.79–24.67, p < 0.001) were associated with an increased risk of recurrent falls. For chronic imbalance, RA (OR 3.73, 95% CI 1.22–9.6, p = 0.020) was significantly associated with chronic imbalance, alongside being female, smoking, and hearing impairment. CONCLUSION: RA was found to be independently associated with an increased risk of chronic dizziness, recurrent falls, and chronic imbalance. </p>","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2026-03-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13488762/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147391038","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Alana Rubia Balbinot, Letícia de Abreu Wiedmer de Siqueira, Poliana Vieira da Silva Menolli, Rafael Andrade Menolli, Ana Paula Adame
{"title":"Incidence, prevalence and clinical features of systemic sclerosis patients from a southern Brazilian city.","authors":"Alana Rubia Balbinot, Letícia de Abreu Wiedmer de Siqueira, Poliana Vieira da Silva Menolli, Rafael Andrade Menolli, Ana Paula Adame","doi":"10.1186/s42358-026-00526-2","DOIUrl":"10.1186/s42358-026-00526-2","url":null,"abstract":"<p><p>BACKGROUND: Systemic sclerosis (SSc) is a chronic and heterogeneous disorder characterized by vascular, inflammatory, and fibrotic changes in the skin and internal organs. As epidemiological studies on SSc in Brazil are few, most reports are from international literature. This study aimed to determine the incidence, prevalence, clinical and laboratory characteristics, and therapeutic management of patients diagnosed with SSc in Cascavel, a southern Brazilian city. Methods: Cross-sectional study that collected data from all rheumatology services in the city (six private rheumatology clinics, a regional specialty center, a municipal specialty center, and an outpatient clinic at a university hospital) in 2020. After the epidemiological survey, eligible patients were invited to undergo investigation for possible orofacial manifestations. Results: We identified 57 patients who were treated for SSc in clinics, 30 of whom lived in Cascavel, Brazil. The estimated prevalence of SSc was 9 ± 0.03 cases/100,000 population, and the incidence in 2020 was 1.2 ± 0.004 cases/100,000 population. Most patients were female, and the mean age at diagnosis was 42.5 ± 17.22 years. The most frequently reported clinical feature is Raynaud’s phenomenon, followed by skin thickening. Limited SSc was the SSc syndrome most frequently identified by physicians, and the antinuclear antibody test was positive in 86.66% of the patients. Six patients participated in the orofacial alteration investigation. Half of the patients had very low hyposalivation, and all had telangiectasias and periodontal disease. Conclusions: Clinical and epidemiological data from Cascavel, Brazil corroborate the findings of previous studies. Orofacial compromise was common in patients with SSc. Increased awareness among healthcare teams managing these patients is necessary to evaluate and manage orofacial problems. </p>","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2026-02-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146776711","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
C D L Marques, G G Resende, M M Pinheiro, C G S Saad, A O Marinho, A M Soares, B E Paiva, C P Albuquerque, D L N Rodrigues, G R W Castro, G A Bulbol, J N Carneiro, J M C Fernandes, M L G Ochtrop, M B R O Gavi, M E G Veiga, M A Yazbek, N G Cavalcanti, N P Machado, O B Malheiro, R B Macedo, R M R A Vieira, R C Lage, R C Menin, R T M Golebiovski, S L E Ribeiro, T L Oliveira, V G Dinis, P D Sampaio-Barros
{"title":"Impact of sex in axial spondyloarthritis: insights from the Brazilian Registry of Spondyloarthritis.","authors":"C D L Marques, G G Resende, M M Pinheiro, C G S Saad, A O Marinho, A M Soares, B E Paiva, C P Albuquerque, D L N Rodrigues, G R W Castro, G A Bulbol, J N Carneiro, J M C Fernandes, M L G Ochtrop, M B R O Gavi, M E G Veiga, M A Yazbek, N G Cavalcanti, N P Machado, O B Malheiro, R B Macedo, R M R A Vieira, R C Lage, R C Menin, R T M Golebiovski, S L E Ribeiro, T L Oliveira, V G Dinis, P D Sampaio-Barros","doi":"10.1186/s42358-026-00525-3","DOIUrl":"10.1186/s42358-026-00525-3","url":null,"abstract":"<p><p>BACKGROUND: Sex differences in axial spondyloarthritis (axSpA) are increasingly recognized, with women often reporting higher disease burden despite similar objective inflammatory markers. This study aimed to compare clinical features, disease activity, function, quality of life, and treatment patterns between men and women with axSpA and identify sex-specific predictors of disease outcomes using data from the Brazilian Registry of Spondyloarthritis (RBE). METHODS: This was a cross-sectional, observational study based on data from the RBE, a nationwide multicenter cohort including 828 patients (568 men and 260 women) from 17 referral centers across Brazil. Standardized clinical and demographic data were collected using the REDCap platform. Disease activity (ASDAS-CRP, BASDAI), physical function (BASFI), spinal mobility (BASMI), and quality of life (ASQoL) were assessed with validated instruments. Sex-stratified multivariable linear regression models were constructed to identify independent predictors of each outcome. RESULTS: Women presented higher disease activity (median BASDAI 4.5 vs. 3.2; ASDAS-CRP 2.2 vs. 1.9), greater functional limitation (BASFI 5.0 vs. 4.0), and poorer quality of life (ASQoL 9.0 vs. 7.0) compared to men, despite similar CRP levels. Psychological distress was more frequent in women, while men had worse spinal mobility (BASMI 4.0 vs. 3.5) and higher HLA-B27 positivity. Regression models revealed that shoulder and hip pain were relevant predictors of disease activity in both sexes, but psychological factors and work activity more strongly influenced outcomes in women. Men’s disease burden was more associated with structural damage and cardiometabolic comorbidities. CONCLUSIONS: This study highlights distinct sex-related clinical patterns in axSpA. Women reported higher symptom burden, functional limitations, and reduced quality of life, largely influenced by subjective symptoms and comorbidities. Conversely, men presented greater structural impairment and different comorbidity profiles. These findings support the need for sex-informed clinical assessments and individualized management strategies in axSpA to tailor the care of these sex-specific disease trajectories, which may enhance equity and outcomes in real-world settings. </p>","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2026-02-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13488764/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146182994","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Luisa Fernanda Caro Forero, Paula Santana Marra, Ricardo Nobrega Machado, Clara Ribeiro Doria, Sebastian Durán Cordoba, Lia Vineyard Steuer, Matheus Santos França, Sylvia Costa Lima Farhat, Izabel Mantovani Buscatti, Gleice Clemente Souza Russo, Maria Teresa Terreri, Mayra Siqueira Dantas, Luciana M Carvalho, Francisco H R Gomes, Natalia Maronese, Adriana R Fonseca, Marta C F Rodrigues, Andreia Watanabe, Lucia M A Campos, Adriana Maluf Elias, Verena Andrade Balbi, Nadia Emi Aikawa, Vivianne S L Viana, Lara R C Melo, Claudia A A Strabelli, Magda Maria Sales Carneiro-Sampaio, Katia Tomie Kozu, Clovis Artur Silva
{"title":"Prolonged skin involvement distinguishes adolescent from childhood-onset IgA vasculitis: a large multicenter study with 687 patients.","authors":"Luisa Fernanda Caro Forero, Paula Santana Marra, Ricardo Nobrega Machado, Clara Ribeiro Doria, Sebastian Durán Cordoba, Lia Vineyard Steuer, Matheus Santos França, Sylvia Costa Lima Farhat, Izabel Mantovani Buscatti, Gleice Clemente Souza Russo, Maria Teresa Terreri, Mayra Siqueira Dantas, Luciana M Carvalho, Francisco H R Gomes, Natalia Maronese, Adriana R Fonseca, Marta C F Rodrigues, Andreia Watanabe, Lucia M A Campos, Adriana Maluf Elias, Verena Andrade Balbi, Nadia Emi Aikawa, Vivianne S L Viana, Lara R C Melo, Claudia A A Strabelli, Magda Maria Sales Carneiro-Sampaio, Katia Tomie Kozu, Clovis Artur Silva","doi":"10.1186/s42358-025-00516-w","DOIUrl":"10.1186/s42358-025-00516-w","url":null,"abstract":"<p><p>BACKGROUND: Immunoglobulin A vasculitis (IgAV), also known as Henoch- Schönlein purpura (HSP), is the most common systemic vasculitis in childhood. Potential differences in demographic characteristics, clinical presentation, laboratory findings, and treatments approaches across age groups, remain poorly explored. To the best of our knowledge, no multicenter study in Latin America has systematically addressed these features. We aimed to assess demographic, clinical and laboratory features, and treatments in children versus adolescents with (IgAV)/ (HSP) in a large multicenter study. METHODS: A multicenter study involving four tertiary centers evaluated 687 children and adolescents (≤ 18 years-old) with IgAV/HSP (EULAR/PRINTO/PRES classification criteria) at first 3 months after diagnosis. The charts were retrospectively assessed for demographic data, initial clinical manifestations, laboratory tests and treatments. Data were compared between children (< 10 years-old) and adolescents (≥ 10 years-old), according to WHO definition. RESULTS: IgAV/HSP was diagnosed in 599/687(87%) children [5.33(0.88–9.91) years-old] and 88/687(13%) adolescents [11.33(10-17.5) years-old]. The median duration of purpura/petechiae was significantly lower in children compared to adolescents [14(1-120) vs. 15(2–90) days, p = 0.04]. The frequency of persistent purpura/petechiae (≥ 6 weeks of duration) was significantly reduced in the former group (7.2% vs. 19.5%, p = 0.002), likewise the frequency of gastrointestinal bleeding (17% vs. 34.1%, p = 0.01) and proteinuria (49.7% vs. 84%, p = 0.002). In contrast, the frequencies of arthritis/arthralgia (82.7% vs. 73%, p = 0.03) and orchitis(16.6% vs. 4.8%, p = 0.04) were significantly higher in children. Further analysis of laboratory tests showed that the median value of serum IgA was significantly lower in children than in adolescents [179.1(40-1002.0) vs. 279.0(104.0-488.0) mg/dL, p = 0.01], whereas thrombocytosis was higher (40.1% vs. 23%, p = 0.007). Logistic regression demonstrated that persistent purpura/petechiae after IgAV/HSP diagnosis (OR = 18.337; 95%CI 1.245-270.137; p = 0.034) was the only independently associated variable with dependent variable (adolescent). CONCLUSIONS: In this large multicenter cohort, IgAV/HSP onset occurred rarely at adolescence, with a more prominent cutaneous involvement. Prolonged purpura/petechiae after IgAV/HSP diagnosis was associated with adolescent-onset IgAV/HSP, reinforcing the need for vigilant monitoring in this subgroup. </p>","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2026-02-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13488647/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146183057","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Clinical features, treatment strategies, and long-term outcomes of Blau syndrome: a 10-year experience from a Chinese cohort.","authors":"Jingyuan Zhang, Min Shen","doi":"10.1186/s42358-026-00528-0","DOIUrl":"10.1186/s42358-026-00528-0","url":null,"abstract":"<p><p>OBJECTIVES: Blau syndrome (BS) is a rare autoinflammatory disorder characterized by the clinical triad of uveitis, dermatitis, and arthritis. While the clinical spectrum is well-documented, data on the longitudinal efficacy and durability of tumor necrosis factor-α (TNF-α) inhibitors remain limited. This study aimed to evaluate the longitudinal treatment trajectories, safety, and long-term outcomes of TNF-α inhibitors in a Chinese cohort. METHODS: This longitudinal observational cohort study analyzed clinical data from 13 Chinese patients diagnosed with BS at Peking Union Medical College Hospital between 2015 and 2025. Whole-exome sequencing was performed in all cases. The cohort was comprehensively evaluated in terms of demographic characteristics, clinical manifestations, genetic findings, genotype-phenotype correlations, and treatment outcomes (specifically focusing on the long-term durability and treatment switching of TNF-α inhibitors). Statistical analyses were performed to assess long-term efficacy and safety. RESULTS: The cohort included 7 (53.8%) females and 6 (46.2%) males. The median age of onset was 2 years (IQR: 1–5 years), while the median age at diagnosis was 23 years (IQR: 18.5–26.5 years). The substantial diagnostic delay (median: 19 years, range: 7–29 years, IQR: 18.5–21 years) likely contributed to the high prevalence of clinical manifestations, including arthritis (13,100%), ocular involvement (12, 92.3%), joint deformity (10, 76.9%), and the full classical triad (9, 69.2%). Genetic analysis revealed 8 NOD2 substitutions, with p.R334W being the most recurrent (6, 46.2%). Regarding treatment, 12 (92.3%) patients received TNF-α inhibitors, either as monotherapy or in combination with conventional DMARDs or corticosteroids. These agents demonstrated robust initial efficacy: complete remission was consistently achieved with infliximab (4 patients, 30.8%), etanercept (3 patients, 23.1%), and adalimumab (3 patients, 23.1%). However, long-term follow-up revealed the complexity of management. While 6 (46.2%) patients achieved sustained stability, relapse or secondary loss of efficacy occurred in 6 (46.2%) patients, necessitating treatment adjustments. Additionally, alternative therapies (tofacitinib, canakinumab) showed promise in refractory cases. Safety was generally manageable, with only one patient (7.7%) experiencing adverse effects from TNF-α inhibitors. Stratified analysis based on the NOD2 genotype revealed no significant differences in clinical severity or treatment efficacy. CONCLUSIONS: This study provides real-world observations from a single-center cohort of Chinese patients with Blau syndrome. Our findings suggest that early recognition and timely therapeutic intervention are essential to prevent irreversible organ involvement. TNF-α inhibitors represent a feasible therapeutic option for achieving symptom control, particularly in cases refractory to conventional treatments. However, observed variability in therapeu","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2026-02-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13488730/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146167482","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Incidence and determinants of anxiety and depression in childhood-onset systemic lupus erythematosus: a systematic review and meta-analysis of proportion.","authors":"Yuanfei Tang, Qiqi Lu, Qianli Liu","doi":"10.1186/s42358-026-00524-4","DOIUrl":"10.1186/s42358-026-00524-4","url":null,"abstract":"<p><p>BACKGROUND: Childhood-onset systemic lupus erythematosus (cSLE) is a rare and severe autoimmune disorder with impactful physical and psychological impacts. While depression and anxiety are well-recognized in adult SLE, their incidence and determinants in cSLE remain less clear. METHODS: We performed a systematic review and meta-analysis following PRISMA guidelines. We searched five databases from inception to January 2025. Our inclusion criteria were studies of pediatric patients (≤18 years) meeting 1997 ACR, and 2021 SLICC diagnostic criteria for SLE and reporting anxiety or depression rates. Data of the demographics, disease characteristics, and medications were extracted, and meta-regression explored potential determinants. RESULTS: 36 studies fulfilled inclusion criteria. Pooled analyses revealed a 14% prevalence of anxiety and 19% prevalence of depression. Exploratory meta-regression indicated that immunosuppressive therapies were associated with modest but statistically significant reductions in both depression and anxiety. By contrast, hematologic complications showed a positive association with risk of mood disorders. Oral steroids showed inconsistent relationships with psychological outcomes. CONCLUSIONS: Anxiety and depression are common in cSLE, we need to integrate screening for them in rheumatologic and mental health care. Close monitoring, early screening, and targeted interventions can help detect the psychological burden and potentially improve adherence to therapy and long-term outcomes. TRIAL REGISTRATION NUMBER: Not applicable. </p>","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2026-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13488828/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146127140","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Fernando Henrique Carlos de Souza, Renata Miossi, Daniel Brito de Araujo, Jean Marcos de Souza, Simone Appenzeller, Luiz Felipe Adsuara de Sousa, Alexandre Moura Dos Santos, Lorenza Rosa Silverio da Silva, Marlise Sitima Mendes Simões Faria, Juliana Almeida Vieira, Laurindo Ferreira da Rocha Junior, Luiz Samuel Gomes Machado, Ana Cecília Diniz Oliveira, Juliana Markus, Ana Gabriela da Silva, Flávia Patrícia Sena Teixeira Santos, Samuel Katsuyuki Shinjo
{"title":"Registry of idiopathic inflammatory myopathies (REMAS) from nine Brazilian research centers linked to tertiary care and teaching hospitals.","authors":"Fernando Henrique Carlos de Souza, Renata Miossi, Daniel Brito de Araujo, Jean Marcos de Souza, Simone Appenzeller, Luiz Felipe Adsuara de Sousa, Alexandre Moura Dos Santos, Lorenza Rosa Silverio da Silva, Marlise Sitima Mendes Simões Faria, Juliana Almeida Vieira, Laurindo Ferreira da Rocha Junior, Luiz Samuel Gomes Machado, Ana Cecília Diniz Oliveira, Juliana Markus, Ana Gabriela da Silva, Flávia Patrícia Sena Teixeira Santos, Samuel Katsuyuki Shinjo","doi":"10.1186/s42358-026-00522-6","DOIUrl":"10.1186/s42358-026-00522-6","url":null,"abstract":"<p><p>OBJECTIVES: Idiopathic inflammatory myopathies (IIMs) are a group of rare chronic inflammatory diseases that affect the muscle, skin, and other organs. There is a severe lack of evidence for current treatment protocols for IIMs. The rarity of these conditions requires multicenter collaboration is vital to facilitate studies of pathogenesis, demographic, clinical, laboratory manifestations, comorbidities, treatment, and disease outcomes from that research group we established a national registry (REMAS) that aims to improve knowledge, facilitate research and clinical trials, and ultimately improve outcomes for these patients. METHODS: A Brazilian network of nine centers and a research group was established to include, since December 2022, consecutive patients with dermatomyositis (DM), clinically amyopathic DM (CADM), inclusion body myositis (IBM), polymyositis (PM), immune-mediated necrotizing myopathy (IMNM), and anti-synthetase syndrome (ASyS). RESULTS: Currently, the study has recruited 284 patients, the majority were diagnosed with DM (53.1%), were female (77.1%), and Caucasian (44%). The median age was 51 years (IQR: 41–62), the median age at the disease onset was 43 years (IQR: 33–55), and the median duration of disease was 6 years (2–11). The median of the manual muscle test 8 (MMT-8) was 77 (74–80), the patient visual analogue scale (VAS) was 3 (0–5), physician VAS was 2 (0–5) and the Health Assessment Questionnaire of 0.33 (0.00–1.00), whereas median creatine kinase levels at the inclusion were 145 U/L (74–327). Among those positive for myositis-specific autoantibody, anti-Jo-1 was the most frequent (10.5%), followed by anti-Ro-52 (10.1%). Regarding treatment, 87% of the patients received prednisone with a median dose of 5 mg/day (0–20), 32.7% received pulse therapy with methylprednisolone, and 18.3% received human intravenous immunoglobulin. CONCLUSIONS: This establishment of this multicenter registry has facilitated research and advanced knowledge on a complex group of rare musculoskeletal diseases. The establishment of the REMAS has highlighted important local findings, despite limited representation from some regions of Brazil. </p>","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2026-02-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13488733/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146114694","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}