ARP Rheumatology最新文献

{"title":"Wunderlich Syndrome as an unusual presentation of Microscopic Polyangiitis: A Case report with Review of Literature.","authors":"BrajaGopal Jana, Kaustav Bhowmick, Arghya Chattopadhyay, Uddalok Das, Indranil Sen, Spandan Bhadury","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Wunderlich syndrome, characterized by spontaneous perinephric hematoma with subcapsular extension has been scarcely reported in microscopic polyangiitis (MPA). We report the case of a 45-year-old woman, who presented with constitutional symptoms, left-eye episcleritis, and rapidly progressive glomerulonephritis. She developed sudden, severe left flank pain with hemoglobin drop two days after admission. Both computed tomography (CT) and non-contrast magnetic resonance imaging revealed large left-sided perinephric hematoma. CT angiography failed to demonstrate intrarenal aneurysms. A remarkable reduction in size of her perinephric hematoma was observed after three and a half months of treatment with glucocorticoids and intravenous cyclophosphamide (IV CYC) following the international guidelines. A literature review on renal vessel involvement in antineutrophil cytoplasmic antibody-associated vasculitis revealed 26 case reports and one case series with 20 cases of renal aneurysms. Eighteen cases in the case reports (69.2%) and nine in the case series (45%) ruptured their renal arteries. The majority (44.4%) were managed with IV CYC and high-dose glucocorticoids. Angioembolization, renal replacement therapy, and plasma exchange were used as adjuvant measures. Only three patients (16.7%) underwent nephrectomy, while the majority(63.6%) fully recovered.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"4 2","pages":"154-162"},"PeriodicalIF":1.4,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144592459","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}

{"title":"Bridging the gap between 2024 EULAR/PreS Recommendations for Still's Disease and practice: the need for awareness of biomarkers and timely use of IL-1/ IL-6 inhibition.","authors":"Carolina Zinterl, Carolina Ochôa Matos, Filipa Oliveira-Ramos","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Introduction: </strong>Still's Disease (SD), encompassing both systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD) is a rare condition, therefore posing unique challenges in diagnosis and management, which can lead to delayed treatment and increased morbidity. The current state of practice regarding SD management varies widely across different countries. Very recently, a EULAR/PReS taskforce published new recommendations for the diagnosis and management of SD, providing tools to accelerate its diagnosis, including new biomarkers such as IL-18 and S100 proteins and recommending early initiation of IL-1 or IL-6 inhibition to avoid prolonged systemic glucocorticoid exposure.</p><p><strong>Methods: </strong>Cross-sectional, descriptive, and observational study utilizing a structured questionnaire to collect data from healthcare professionals involved in the diagnosis and treatment of SD in Portugal.</p><p><strong>Results: </strong>We obtained 52 responses from Portuguese clinicians. Only 10% use IL-18 levels and 25% use S100 proteins to aid in the diagnosis of SD. Half of the responders expect to achieve clinically inactive disease (CID) with low-dose glucocorticoids after 3 months, but only 39% aim to achieve CID without glucocorticoids at month 6. For 95% of responders the use of glucocorticoids is part of the first line of therapy. Less than half (37%) did not include IL-1 or IL-6 inhibitors in their first line of treatment.</p><p><strong>Conclusion: </strong>The results of the questionnaire applied show that there is still a gap between clinical practice and the recent recommendations, as demonstrated by the underuse of recent biomarkers and biologic therapies, which should be bridged in order to improve health outcomes for individuals affected by SD.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"4 2","pages":"177-178"},"PeriodicalIF":1.4,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144592411","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}

{"title":"Interleukin biomarkers as predictive tools for lupus nephritis grade and disease activity in systemic lupus erythematosus.","authors":"Dalia S Saif, Shimaa Abdelsattar, Enas S Zahran, Mohammed Khalil, Sanaa Samir, Hend E Abo Mansour","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Background: </strong>Systemic lupus erythematosus (SLE) is an autoimmune disease that affects multiple organs, particularly the kidneys. Interleukin (IL) biomarkers including IL-10 and IL17/23 axis play an important role in SLE pathogenesis.</p><p><strong>Objectives: </strong>To investigate the predictive value of IL-17, IL-23, and IL-10 biomarkers in detecting lupus nephritis (LN) class in SLE cases.</p><p><strong>Methods: </strong>This is a case-control study involving 160 individuals: 100 patients with SLE (80 LN patients who had a recent report of kidney biopsy in the two months prior to the study +20 non renal SLE patients), and 60 age- and sex-matched healthy volunteers. All participants were subjected to clinical and laboratory studies, as well as the evaluation of their IL-17, IL-23, and IL-10 biomarkers.</p><p><strong>Results: </strong>IL-17, IL-23, and IL-10 were significantly elevated in SLE patients (p-value < 0.001), especially in cases with high disease activity (p-value < 0.001). Moreover, these biomarkers were considerably higher in LN patients (p-value < 0.001), particularly among class III and IV LN (p-value < 0.001) and in cases with high nephritis activity index (p-value < 0.001). ROC curve analysis revealed precise cutoff points of IL-17, IL-23, and IL-10 levels in each renal histopathological class with high sensitivity and specificity.</p><p><strong>Conclusion: </strong>IL-17, IL-23, and IL-10 biomarkers are higher in SLE patients and are correlated with SLE Disease Activity Index (SLEDAI). They are more prevalent in individuals with LN, particularly in cases with high activity index and with more aggressive classes (in renal classes III and IV). These biomarkers might function as indicators for detecting LN activity and as predictors of LN class.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"4 2","pages":"80-90"},"PeriodicalIF":1.4,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144592415","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}

{"title":"Prevalence of Juvenile Fibromyalgia Syndrome in Turkish Patients with Juvenile Idiopathic Arthritis: A Multicenter Study.","authors":"Gülcan Özomay Baykal, Semra Ayduran, Selen Duygu Arık, Ozge Baba, Serife Tunçez, Hafize Emine Sönmez, Kübra Öztürk, Semanur Özdel, Mukaddes Kalyoncu, Nuray Aktay Ayaz, Selçuk Yüksel, Betül Sözeri","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Objectives: </strong>To investigate the diagnostic prevalence of juvenile fibromyalgia syndrome (JFMS) causing widespread pain in patients with a diagnosis of juvenile idiopathic arthritis (JIA).</p><p><strong>Methods: </strong>Patients with JIA from seven pediatric rheumatology centers in Türkiye were included. 2010 American College of Rheumatology criteria for fibromyalgia was utilized throughout a face-to-face interview. The Pain and Symptom Assessment Tool was used, and data were analyzed using the Widespread Pain Index and the Symptom Severity Scale. Patients were stratified into two groups: Group 1 (JIA with concomitant juvenile fibromyalgia) and Group 2 (JIA without juvenile fibromyalgia).</p><p><strong>Results: </strong>A total of 313 patients with JIA were included, of whom 21 (6.7%) were found to have concomitant JFMS. In group 1, 71% (15 patients) were female and 29% (6 patients) were male, with a median age at JFMS evaluation of 16 years (range: 12.8-19). Among patients with JFMS, 62% (13 patients) were classified as having spondyloarthropathy (enthesitis-related arthritis or juvenile psoriatic arthritis), 28.5% (6 patients) as having oligoarticular JIA, and 9.5% (2 patients) as having polyarticular JIA. Seventeen patients (81%) were on medication, including five (24%) on biologics. The most common symptoms in the JFMS group were muscle pain and fatigue, followed by headache, nervousness, numbness, dizziness, acne, abdominal pain, and anorexia.</p><p><strong>Conclusion: </strong>In JIA patients with chronic musculoskeletal pain, fatigue, headache, and irritability lasting more than three months, the possible diagnosis of JFMS should be considered in the clinical evaluation.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"4 2","pages":"138-144"},"PeriodicalIF":1.4,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144592419","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}

{"title":"Juvenile dermatomyositis: a severe and atypical presentation.","authors":"Isabel Moitinho de Almeida, Marta Abreu Andrade, Miguel Bernardo, Ana Cláudia Fonseca, Ana Raquel Campanilho Marques, Filipa Oliveira Ramos, Patrícia Costa-Reis","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Introduction: </strong>Juvenile dermatomyositis (JDM) is a rare immune-mediated disease, characterised by proximal muscle weakness and typical skin rashes1. We present a patient with severe JDM, to highlight the importance of a timely diagnosis and early initiation of treatment.</p><p><strong>Case description: </strong>A 9-year-old girl presented to the hospital due to asthenia, rash, generalized oedema, and inability to walk. At observation, the patient had dysphonia, dysphagia, proximal muscle weakness, petechial rash, skin ulcers, and anasarca. The levels of creatine kinase, aldolase, transaminases, and ferritin were elevated, and the NXP-2 antibody was detected. Prednisolone and methotrexate were started, followed by intravenous immunoglobulin. During the hospitalisation, the patient had an alveolar haemorrhage. Retinal vasculitis was also detected. Mycophenolate mofetil was added to the treatment. The patient had full resolution of myositis with progressive recovery of muscle strength, healed ulcers, and completely improved vision.</p><p><strong>Discussion: </strong>This is an atypical presentation of JDM, without the typical skin lesions, but with several manifestations of severe vasculopathy, including retinopathy and alveolar haemorrhage. Early diagnosis and a multidisciplinary approach are crucial to improve prognosis.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"4 2","pages":"163-168"},"PeriodicalIF":1.4,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144592416","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}

{"title":"Rare coexistence of large vessel vasculitis and nonbacterial thrombotic endocarditis: a case report.","authors":"Ana Rita Lopes, Roberto Pereira da Costa, Filipa Costa, Bianca Paulo Correia, André Peixoto, Luís Brás Rosário, Cristina Ponte","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Large-vessel vasculitis (LVV) is an inflammatory condition affecting large arteries, often leading to complications such as vessel occlusion or aneurysm. Nonbacterial thrombotic endocarditis (NBTE), although rare, is generally linked with hypercoagulable states and malignancies, characterized by sterile vegetations on undamaged cardiac valves. We present a unique case of a 61-year-old woman with coexisting LVV and NBTE, evidenced by echocardiographic findings of vegetations on the aortic and mitral valves. Her initial presentation included syncope, fatigue, weight loss, and intermittent claudication. Investigations revealed elevated inflammatory markers, arterial wall thickening suggestive of vasculitis, and valvular masses on echocardiography. She responded well to anticoagulation and immunosuppressive therapy, achieving clinical remission with mild residual symptoms at two years. This case underscores the unusual overlap between LVV and NBTE, highlighting the importance of vigilant diagnostic evaluation in patients with vasculitis who present with valvular abnormalities. Given the rarity of LVV with NBTE, further studies are essential to guide optimal management for similar complex presentations.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"4 2","pages":"171-173"},"PeriodicalIF":1.4,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144592420","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}

{"title":"Patients', caregivers and clinicians' perspectives on education and support about methotrexate: survey to 26 European Countries.","authors":"Cristiano Matos, Polly Livermore, Mariana Ortiz-Piña, Khadija El Aoufy, Kristina Buerki, Agnes Ágoston-Szabó, Darja Batšinskaja, Jana Melicharová, Marie-Louise Karlsson, Karlien Claes, Ana Isabel Rodriguez Vargas, Ellen Moholt, Ane Ludvigsen, Una Martin, Ulrike Erstling, Angela Camon, Ana Pais, Mikaella Konstantinou, Myrto Nikoloudaki, Souzi Makri, Bruno Silva, Cláudia Paiva, Elena Nikiphorou, Andrea Marques, Ricardo J O Ferreira","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Objectives: </strong>To assess patients', carers', nurses' and physicians' perspectives and experiences regarding patient education (PE) and support of Methotrexate (MTX) treatment in Europe.</p><p><strong>Methods: </strong>An international team of researchers and clinicians, including rheumatology nurses, a pharmacist, a rheumatologist, and three patient representatives, developed a survey. Common and sample-specific questions were conceived for adult patients or carers (≥18 years) of children/young people with RMDs, nurses, and physicians working in rheumatology practice in Europe. The survey was available in English and, for patients/carers, in eight additional languages, disseminated between May 2022 and May 2023. Ethics committee approval was obtained (116_CEIPC/2022_IPC).</p><p><strong>Results: </strong>A total of 1526 patients, 145 carers, 354 nurses, and 291 physicians (96% rheumatologists), from 26 European countries participated. Only 28% of patients had a PE with nurse when starting oral MTX, with a slight increase to 42% for the subcutaneous form, with variations across Europe (Northern=69%, Eastern=52%, Western=50%, Southern=23%). Patients' perspectives align with physicians, whereas nurses reported higher access rates. Around 77% of patients had/have concerns about side effects, which were discussed with health professionals in 69% of the cases, though 46% of these concerns remained unresolved. The priority ranking of topics to be addressed in PE was similar overall for the three subgroups.</p><p><strong>Conclusion: </strong>PE and support regarding MTX are unequal across Europe and can be improved by offering opportunities to clarify concerns through more access to nursing consultations. There is an overall agreement between patients and clinicians regarding key areas of education, although a tailored approach is required.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"4 2","pages":"91-105"},"PeriodicalIF":1.4,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144592418","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}

{"title":"Reliability and validity of the European Portuguese version of the ABILHAND questionnaire in Patients with Systemic Sclerosis.","authors":"Fernando Albuquerque, Marcelo Neto, Rodrigo Patrício, Margarida Vieira, Rúben Fernandes, Anabela Silva, Tânia Santiago, Maria João Salvador","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Systemic sclerosis (SSc) frequently affects hands, impairing its function and impacting quality of life. The ABILHAND-SSc is a patient-reported outcome measure (PROM) specifically designed to assess manual ability in SSc. Previously, an European Portuguese version has been translated and culturally adapted, but its psychometric properties remain to be validated. Therefore, we aim to validate the European Portuguese version of the ABILHAND-SSc in patients with systemic sclerosis (SSc) using Rasch analysis and classical test theory. A cross-sectional validation study was conducted in a tertiary rheumatology centre, including 53 Portuguese-speaking adults fulfilling ACR/EULAR 2013 classification criteria for SSc or VEDOSS classification criteria. Patients completed the ABILHAND-SSc along with other PROMs: HAQ-DI, SHAQ, SF-36v2, EQ-5D-5L, and ScleroID. Rasch analysis assessed item fit, dimensionality, targeting, and reliability. Test-retest reliability was evaluated in a stable subsample. Construct validity was examined via hypothesis testing and correlation with external instruments. The ABILHAND-SSc showed excellent internal consistency and Rasch-based reliability. Item fit statistics were within acceptable ranges, and no floor or ceiling effects were observed. Rasch and PCA analyses supported unidimensionality. Test-retest reliability was good. ABILHAND-SSc scores showed good correlation with related PROMs. Patients with diffuse cutaneous SSc had significantly lower manual ability than those with VEDOSS. In conclusion, the European Portuguese version of the ABILHAND-SSc is a valid, reliable, and feasible tool for assessing manual ability in patients with SSc. Its use in clinical practice and research may support patient-centred assessment and monitoring of hand function. Further validation in longitudinal and multicentre studies is warranted.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"4 2","pages":"123-137"},"PeriodicalIF":1.4,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144592457","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}

{"title":"Translation and cross-cultural adaptation of the mSQUASH into European Portuguese.","authors":"Ana Catarina Moniz, Mariana Emília Santos, Kaylene de Freitas, Filipa Tojal, Joelene de Freitas, Manuela Costa, Paula Araújo, Suzanne Arends, Sofia Ramiro, Fernando Pimentel-Santos, Jaime Cunha Branco, Alexandre Sepriano","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Background: </strong>Regular physical activity is recommended for all patients with axial spondyloarthritis (axSpA), but measuring its frequency, duration and intensity can be challenging. The modified Short Questionnaire to ASsess Health-enhancing physical activity (mSQUASH) is a patient-reported outcome measurement designed to assess daily physical activity in patients with axSpA. This study aimed to translate the mSQUASH into European Portuguese and to perform field testing with cognitive debriefing interviews in patients with axSpA in Portugal.</p><p><strong>Methods: </strong>The mSQUASH was translated into European Portuguese following the Beaton method. Two bilingual translators independently translated the questionnaire from English to European Portuguese, which was then harmonized into a consensus version. Two other translators back translated the synthesized version into English. Translation discrepancies were resolved within a scientific committee, resulting in a preliminary version. The preliminary version was field-tested through semi-structured one-to-one interviews with 10 patients with axSpA with a broad range of socio-demographic and clinical characteristics.</p><p><strong>Results: </strong>The translation process was completed without major issues and minor disagreements were resolved in consensus meetings. During field testing, all participants found the questionnaire clear and appropriate. The median time to complete the questionnaire was 4 minutes and 15 seconds. Patient's feedback led to the correction of minor spelling errors and the addition of examples to the item \"Home maintenance\", which was misinterpreted as household activities by half of patients. The final version of the questionnaire, incorporating patient feedback, was approved by the scientific committee.</p><p><strong>Conclusion: </strong>The European Portuguese version of the mSQUASH demonstrated good linguistic properties and performed well in a field test with axSpA patients. Further studies are needed to evaluate its psychometric properties.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"4 2","pages":"115-122"},"PeriodicalIF":1.4,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144592458","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}

{"title":"Bywaters lesions.","authors":"Catarina Abreu, Susana Matias, Maria Margarida Cunha, Alice Morais Castro","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"4 2","pages":"169-170"},"PeriodicalIF":1.4,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144592412","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}