ARP Rheumatology最新文献

{"title":"Efficacy and Safety of Tocilizumab in Polymyalgia Rheumatica: A Systematic Review and Meta-analysis of Randomized Controlled Trials.","authors":"Brijesh Baral, Mandakini Parajuli, Juan Pinilla, Beatriz Quintanilha, Bishal Baral, Fidencio Cons Molina","doi":"10.63032/HYAU2255","DOIUrl":"10.63032/HYAU2255","url":null,"abstract":"<p><strong>Introduction: </strong>The efficacy and safety of tocilizumab in patients with polymyalgia rheumatica (PMR) is not well established.</p><p><strong>Methods: </strong>We systematically searched PubMed, Cochrane, and Scopus to identify randomized controlled trials (RCTs) evaluating the efficacy and safety of tocilizumab compared with placebo in patients with PMR. The endpoints of interest were glucocorticoid-free remission at week 24, cumulative prednisolone dose at week 24, and adverse effects like risk of infection, gastrointestinal disorders, musculoskeletal and connective tissue disorders. We analyzed binary outcomes using risk ratios (RR) and continuous outcomes using mean difference (MD) with 95% confidence intervals (CI). Statistical analysis was performed using Review Manager 8.13 (Cochrane Collaboration).</p><p><strong>Results: </strong>Three RCTs with 188 patients were included, of whom 99 (53%) received tocilizumab and 89 (47%) received a placebo. The three RCTs varied significantly regarding patient populations and clinical settings: Bonelli et al. (2022) studied patients with early PMR receiving short-term glucocorticoids (GCs), Devauchelle-Pensec et al. (2022) included patients with GC-dependent PMR and a prespecified GC tapering strategy, and Spiera et al. (2021) analyzed patients with PMR associated with giant cell arteritis (GCA). Tocilizumab was associated with higher glucocorticoid-free remission at week 24 (RR 2.64; 95% CI 1.38 to 5.06; p= 0.003) and a lower cumulative prednisolone dose at week 24 (MD -2.52mg; CI -4.00 to -1.03; p= 0.0009) compared to placebo. However, there were no significant differences between the groups regarding safety outcomes, including the risk of infections (RR 1.19; 95% CI 0.92 to 1.52, p = 0.18), gastrointestinal disorders (RR 1.17; 95% CI 0.72 to 1.89, p = 0.52), and musculoskeletal and connective tissue disorders (RR 1.13; 95% CI 0.53 to 2.42, p = 0.75).</p><p><strong>Conclusion: </strong>Our findings indicate that tocilizumab significantly improved glucocorticoid-free remission rates and reduced the cumulative prednisolone dose at week 24. Notably, safety outcomes between tocilizumab and placebo groups were comparable. These findings support the efficacy of tocilizumab in treatment of PMR.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"4 2","pages":"145-153"},"PeriodicalIF":1.4,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144592414","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":"Manifestations and predictors of neurologic involvement in Behçet's disease.","authors":"Margarida Lucas Rocha, Roberto Pereira da Costa, Ana Teodósio Chícharo, Joana Martins-Martinho, Carla Macieira, Cristina Ponte, Nikita Khmelinskii","doi":"10.63032/MYNO6766","DOIUrl":"10.63032/MYNO6766","url":null,"abstract":"<p><strong>Background: </strong>Behçet's disease (BD) is a multisystem inflammatory disorder primarily affecting mucocutaneous tissues. Central nervous system (CNS) involvement, Neuro-BD (NBD), is a potentially severe manifestation of BD with a variable prevalence (1-59%).</p><p><strong>Aims: </strong>We aimed to characterize BD patients with CNS involvement and to identify predictors of this clinical subtype.</p><p><strong>Methods: </strong>We performed a single-centre observational retrospective study using data from patients fulfilling the 2013 International Criteria for BD registered at the Rheumatic Diseases Portuguese Registry (Reuma.pt) between October 2014 and May 2023. NBD was defined according to International Consensus Recommendation Criteria for NBD diagnosis. Demographic, clinical and treatment data were collected.</p><p><strong>Results: </strong>We included 157 patients with BD, 116 (74%) of them were females and 127 (81%) Caucasian with a mean±SD age at diagnosis of 32.0±12.2 years and the median (IQR) follow-up was 11.0 (11.8) years. NBD was diagnosed in 24 (15%) patients, with two (1%) cases identified at disease onset. The mean age at NBD diagnosis was 31.6±8.7 years, occurring 4.7 (1.0) years after BD onset. Among NBD patients, 15 (63%) had parenchymal, eight (33%) non-parenchymal and one (4%) mixed CNS involvement. Compared to patients without NBD, those with NBD were more likely to be non-Caucasian (27% vs 17%, p=0.026), have genital ulcers at disease onset (68% vs 34%, p=0.002) and present with ocular manifestations during the disease course (58% vs 35%, p=0.028). NBD patients more frequently received systemic glucocorticoids (100% vs 78%, p=0.008) and conventional synthetic disease-modifying anti-rheumatic drugs (86% vs 62%, p=0.035), particularly cyclophosphamide (48% vs 1%, p<0.001). Mortality was also significantly higher among NBD (13% vs 2%, p=0.026). On multivariable analysis, genital ulcers at presentation (OR 4.50, 1.68-12.06) and ocular involvement during the disease course (OR 2.69, 1.01-6.76) were independent predictors of CNS involvement.</p><p><strong>Conclusion: </strong>In summary, our cohort showed a 15% prevalence of CNS involvement, with parenchymal affection being the predominant form. Genital ulcers at onset and ocular manifestations emerged as independent predictors of NBD, serving as potential clinical markers for identifying high-risk patients. The high mortality rate observed among these patients underscores the severity of NBD and the critical need for prompt recognition.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"4 2","pages":"174-176"},"PeriodicalIF":1.4,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144592417","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":"AI in medical research: boosting discovery or weakening critical search skills?","authors":"Annika Nack, Diego Benavent","doi":"10.63032/HWCR2841","DOIUrl":"10.63032/HWCR2841","url":null,"abstract":"","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"4 2","pages":"76-79"},"PeriodicalIF":1.4,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144592410","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":"Pregnancy outcomes in connective tissue diseases: a 30-year study of 465 cases from a single-center Spanish registry with insights on hydroxychloroquine use.","authors":"Cristiana Sieiro Santos, José Ordás Martínez, Clara Moriano Morales, Carolina Alvarez Castro, Elvira Díez Álvarez","doi":"10.63032/RMXO6650","DOIUrl":"10.63032/RMXO6650","url":null,"abstract":"<p><strong>Introduction: </strong>Pregnancy in women with connective tissue diseases (CTDs), including systemic lupus erythematosus (SLE), systemic sclerosis (SSc), primary Sjögren's syndrome (pSS), and undifferentiated connective tissue disease (UCTD), poses significant risks for adverse outcomes. Evaluating these risks and outcomes is essential to improve maternal and fetal health.</p><p><strong>Objectives: </strong>This study aimed to assess pregnancy outcomes in patients with CTDs, identify factors associated with adverse outcomes, and evaluate the protective effects of hydroxychloroquine (HCQ) treatment.</p><p><strong>Methods: </strong>A study covering the period from 1990 to 2022 was conducted. Data were collected from medical records of childbearing-age women with SLE, SSc, pSS, and UCTD who were under care at our clinic. Obstetric, maternal, and fetal outcomes were analyzed across different diagnoses. Statistical analyses were performed to identify associations between disease activity, treatments, and pregnancy outcomes.</p><p><strong>Results: </strong>A total of 295 patients (125 with SLE, 50 with SSc, 80 with pSS, and 40 with UCTD) and 465 pregnancies were included. The mean age at first pregnancy was 29.1±9.1 years. Pregnancy loss occurred in 21% of cases, while 77% resulted in live births. Adverse outcomes included preterm delivery (8%), postpartum hemorrhage (6%), and preeclampsia (5%). SLE diagnosis (OR 1.5, 95% CI [1.1-4.8], p = 0.03), double/triple antiphospholipid antibody (APL) positivity (OR 2.3, 95% CI [1.1-3.9], p = 0.04), and active disease (OR 3.4, 95% CI [1.8-5.2], p = 0.004) were identified as risk factors for adverse pregnancy outcomes. HCQ treatment demonstrated a protective effect (OR 0.34, 95% CI [0.05-0.72], p = 0.0004).</p><p><strong>Conclusion: </strong>Two-thirds of pregnancies in women with CTDs resulted in live births, though SLE was associated with significantly higher risks. Active disease during pregnancy emerged as a major risk factor. Importantly, the use of HCQ was associated with a notable reduction in these risks, underscoring its protective role in improving pregnancy outcomes. These findings highlight the critical importance of preconception counseling, careful disease management, and the proactive use of HCQ to minimize complications and optimize outcomes in pregnancies complicated by CTDs.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"4 1","pages":"29-36"},"PeriodicalIF":1.4,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143765270","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":"Implementing systematic screening of fracture risk and osteoporosis treatment in Portugal: a feasibility study protocol.","authors":"Ricardo J O Ferreira, Andréa Marques, Rafael Prieto-Moreno, Tiago Silva, Ana Pinho, Georgina Pimentel, Glenn Haugeberg, José Ap da Silva","doi":"10.63032/ORQW2127","DOIUrl":"10.63032/ORQW2127","url":null,"abstract":"","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"4 1","pages":"69-71"},"PeriodicalIF":1.4,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143765268","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":"The role of bone histomorphometry in the management of metabolic bone disease.","authors":"Rafaela Nicolau, Luciano Pereira, Georgina Terroso, Carlos Vaz, Iva Brito, João Frazão","doi":"10.63032/FYPZ8890","DOIUrl":"10.63032/FYPZ8890","url":null,"abstract":"<p><strong>Purpose: </strong>Bone biopsy is the only technique capable of comprehensively assessing all bone parameters, including turnover, microarchitecture, and mineralization, yet its clinical utility is debated. This study evaluates its role in routine diagnostic and therapeutic applications.</p><p><strong>Methods: </strong>A retrospective analysis was conducted on 22 horizontal transiliac bone biopsies from 20 patients referred for rheumatology consultation between August 2016 and May 2022. Diagnoses included osteoporosis, adynamic bone disease, hyperparathyroidism-related bone disease, and osteomalacia.</p><p><strong>Results: </strong>Histopathological findings led to therapeutic strategies differing from standard anti-osteoporotic treatment in over one-third of cases. In certain cases, bone biopsy provided critical diagnostic insights that guided therapeutic decisions.</p><p><strong>Conclusion: </strong>Although advancements in non-invasive diagnostics exist, bone biopsy remains indispensable for a subset of patients, offering essential diagnostic and therapeutic insights that significantly influence clinical management.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"4 1","pages":"37-43"},"PeriodicalIF":1.4,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143765287","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":"Pregnancy outcomes in systemic sclerosis: experience of a rheumatology-obstetric multidisciplinary clinic.","authors":"Ana Teodósio Chícharo, Ana Rita Lopes, Sofia Barreira, Patrícia Martins, Ana Rita Cruz-Machado, Luísa Pinto, Susana Capela","doi":"10.63032/RMXO6650","DOIUrl":"10.63032/RMXO6650","url":null,"abstract":"","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"4 1","pages":"66-68"},"PeriodicalIF":1.4,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143765271","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":"Crab-like hands as an unusual presentation of a rheumatic disease.","authors":"Catarina Rua, Regina Cardoso, Tiago Beirão, Catarina Silva, Tiago Meirinhos, Patrícia Pinto, Taciana Videira, Romana Vieira, Joana Aleixo-Santos, Diogo Fonseca, Ana Sofia Pinto, Beatriz Samões, Flávio Costa","doi":"10.63032/PPIF6463","DOIUrl":"10.63032/PPIF6463","url":null,"abstract":"<p><p>Tophaceous gout can present with aggressive tophi causing destruction of the soft tissues, joints, and bones (1,2). A 68-year-old male presented with intense pain in the upper and lower limbs persisting for several weeks. He reported no limb weakness or history of trauma. The patient's medical history included severe tophaceous gout, chronic renal insufficiency, and heart failure. The patient had a poor social condition. On physical examination, he exhibited poor hygiene, cachexia, and dehydration. Active drainage of gout tophi was observed. The normal anatomy of the hand was lost due to joint destruction and fusion of the interdigital spaces (see figure 1 - panel A). Also, a giant tophus can be observed in the first finger of the left hand as well. The patient displayed limited range of motion in the wrists, fingers, and elbows, with hindered mobilization of knees, ankles, and feet due to pain. Subsequent blood tests revealed anemia, leucocytosis with neutrophilia, serum creatinine of 2.68 mg/dL, blood urea nitrogen of 147 mg/dL, and C-reactive protein (CRP) of 15.4 mg/dL. X-rays of the hands (figure 2), elbows, and feet revealed multiple \"punched-out\" erosions with sclerotic margins, indicative of massive bone and joint destruction. Appropriate wound dressing was provided, and the patient was initiated on antibiotics, corticosteroids, and urate-lowering therapy. While CRP and anemia showed slight improvement, patient began to refuse nutrition, his renal function declined, and he died 10 days after admission. To the best of our knowledge, this represents an extremely rare case of joint fusion resulting from tophaceous gout, attributed to untreated aggressive disease and challenging social circumstances. In such instances, a multidisciplinary approach that includes healthcare professionals, social workers, and mental health experts is essential.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"4 1","pages":"65"},"PeriodicalIF":1.4,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143765266","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":"A systemic lupus erythematosus patient with Mycobacterium haemophilum infection under treatment: a case report.","authors":"Eva Rojas-Rojas, Wendy Rosario Cinencio-Chávez, Julia Dolores Estrada-Guzmán, Rafael Laniado-Laborín","doi":"10.63032/ZTJA1939","DOIUrl":"10.63032/ZTJA1939","url":null,"abstract":"<p><p>Environmental mycobacteria, or nontuberculous mycobacteria (NTM), include over 170 species, with few frequently affecting humans. M. haemophilum, a slowly growing acid-fast bacillus, is known to cause various infections in immunocompromised patients. We present a case of a 29-year-old female with systemic lupus erythematosus, under immunosuppressive treatment, who developed disseminated skin lesions. Initially presenting with painful erythematous nodules that ulcerated, her condition was initially attributed to lupus. A skin biopsy revealed chronic granulomatous inflammation positive for acid-fast bacilli, leading to treatment for what was thought an infection by M. abscessus. Molecular sequencing later identified M. haemophilum, prompting a switch to a regimen of rifampicin, isoniazid, ethambutol, and levofloxacin, resulting in clinical improvement and lesion remission. M. haemophilum infections are more common in severely immunocompromised patients, often involve dermal lesions, and require accurate diagnosis through a combination of histological, molecular, and culture methods. Immunosuppressive therapy in autoimmune diseases predisposes patients to NTM infections. While tuberculosis infection can be screened and managed preemptively, no equivalent protocols exist for NTM, making close monitoring of immunosuppressed patients crucial for early detection and treatment.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"4 1","pages":"54-56"},"PeriodicalIF":1.4,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143765265","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":"A feasibility study of a short lung ultrasound protocol as a screening method for fibrotic interstitial lung disease.","authors":"Sara Braga, André Carvalho, Márcio Rodrigues, Pedro Madureira, Raquel Miriam Ferreira, André Terras Alexandre, Natália Melo, Patrícia Caetano Mota, António Morais, Hélder Novais Bastos","doi":"10.63032/GQAM5525","DOIUrl":"10.63032/GQAM5525","url":null,"abstract":"<p><strong>Aims: </strong>Interstitial lung disease (ILD) represents a common complication of connective tissue diseases (CTD). Early detection of ILD is critical since patients often remain asymptomatic during the initial stages. While numerous studies have proposed various ultrasound protocols for ILD evaluation, we find these protocols to be time-consuming in our clinical practice. The aim of this study is to introduce a concise lung ultrasound protocol for the screening of pulmonary fibrosis.</p><p><strong>Methods: </strong>We conducted a prospective observational pilot study involving 28 patients followed in consultation for ILDs. By implementing this streamlined protocol, we established correlations between ultrasound findings at specific locations and their corresponding tomographic patterns. Following the assessment of the protocol's diagnostic accuracy, a multidisciplinary meeting was convened to scrutinize ultrasound images from several patients and evaluate the level of agreement among rheumatologists, pulmonologists, and radiologists.</p><p><strong>Results: </strong>Our simplified protocol revealed significant correlations between ultrasonographic pleural irregularity, discontinuity and the number of B-lines, and the tomographic findings of reticulation and honeycombing in both the upper and lower lung regions. This protocol demonstrated high sensitivity and positive predictive value in identifying reticulation and honeycombing, as well as remarkable specificity and negative predictive value for the latter. The results obtained between the specialties showed strong agreement.</p><p><strong>Conclusion: </strong>Preliminary results suggest a role of ultrasound to detect peripheral manifestations of fibrotic ILDs, such as reticulation and honeycombing. This assessment protocol can be adopted effectively by clinicians, such as rheumatologists, to evaluate lung involvement by CTDs. A larger study is necessary to achieve more robust and generalizable results as a screening tool.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"4 1","pages":"20-28"},"PeriodicalIF":1.4,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143765264","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}