Reumatismo最新文献

{"title":"Is there still someone laughing at Christopher Columbus?","authors":"Leonardo Punzi, Alberto Perez-Negrete, Marco Amedeo Cimmino, Andrea Doria, Roberto Gerli, Gian Domenico Sebastiani, Luigi Sinigaglia, Piero Marson","doi":"10.4081/reumatismo.2026.2253","DOIUrl":"10.4081/reumatismo.2026.2253","url":null,"abstract":"<p><p>Dear Editor, Cristopher Columbus's voyages have undoubtedly changed the history of humankind. Despite already being a public figure in his lifetime, several facts concerning Columbus's life, including his final illness, are still under debate...</p>","PeriodicalId":21222,"journal":{"name":"Reumatismo","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147779467","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Hypocomplementemic urticarial vasculitis: a rare presentation revealing systemic lupus erythematosus and Sjögren's disease.","authors":"Said Taharboucht, Chaima Mehiris, Sonia Bellil, Zahira Chikh Salah, Lydia Bentaleb","doi":"10.4081/reumatismo.2026.1930","DOIUrl":"10.4081/reumatismo.2026.1930","url":null,"abstract":"<p><p>Hypocomplementemic urticarial vasculitis (HUV) is a rare small-vessel vasculitis that may occur as a primary form or secondary to systemic disease. Its concurrent association with systemic lupus erythematosus (SLE) and Sjögren's disease (SD) remains exceptional. Diagnosis is particularly challenging due to overlapping clinical and immunological features among these entities. We report the case of a 46-year-old woman with no significant medical history, presenting with recurrent chronic urticaria resistant to antihistamines. Clinical, biological, and histological investigations led to the diagnosis of HUV associated with SLE and SD without severe organ involvement. Treatment with colchicine and hydroxychloroquine resulted in complete remission of symptoms. This case highlights the importance of considering HUV in any patient with chronic or refractory urticaria and of systematically screening for associated autoimmune diseases, particularly SLE and SD.</p>","PeriodicalId":21222,"journal":{"name":"Reumatismo","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148219536","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Telerheumatology to improve first access outcomes in outpatients: a prospective monocentric pilot study.","authors":"Maria Chiara Gerardi, Nicola Ughi, Eleonora Giacobbe, Antonella Adinolfi, Laura Belloli, Cinzia Casu, Maria Di Cicco, Davide Filippini, Clizia Gagliardi, Marina Muscarà, Elisa Verduci, Chiara De Stefani, Oscar Massimiliano Epis","doi":"10.4081/reumatismo.2026.1843","DOIUrl":"10.4081/reumatismo.2026.1843","url":null,"abstract":"<p><strong>Objective: </strong>The present pilot study aimed to assess the feasibility of a \"teletriagerheum\" service before the first rheumatology visit and identify potential benefits and disadvantages by comparing a regular visit with a first face-to-face visit preceded by the \"teletriagerheum\" service.</p><p><strong>Methods: </strong>A prospective monocentric pilot study was conducted from October to December 2021. Consecutive patients were contacted to investigate their willingness to receive a phone call (\"teletriagerheum\" service) from a physician of the Rheumatology Unit before the first rheumatology visit. The number of definite diagnoses at the first visit in the \"teletriagerheum\" group was compared with patients receiving a regular first visit, as well as socio-demographic characteristics, the reason for the visit, face-to-face visit duration, and the number of additional exams.</p><p><strong>Results: </strong>Overall, 75 patients performed the \"teletriagerheum\" (\"teletriagerheum\" group), and 74 patients were included in the control group. In the \"teletriagerheum\" group, a higher number of conclusive diagnoses was observed (72% vs. 58%, p=0.020) with a higher probability of receiving a conclusive diagnosis compared with the control group, both at univariate [odds ratio (OR) 2.28, p=0.021] and multivariate analyses adjusting for the only differences between the two groups (marital status and information and communication technologies skills; OR 2.95, p=0.007). At the first face-to-face visit, fewer additional tests were prescribed in the \"teletriagerheum\" group; however, the overall number of tests (including those prescribed during teletriage) was higher.</p><p><strong>Conclusions: </strong>A telephone-based telerheumatology service could be an effective triage tool for first visits of new outpatients, enabling more definitive diagnoses, thanks in part to the ability to perform the required investigations in a timely manner in most cases.</p>","PeriodicalId":21222,"journal":{"name":"Reumatismo","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148302966","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"From anatomy to therapy: the historical journey to cortisone.","authors":"Ernesto Damiani, Piero Marson, Francesco M Galassi","doi":"10.4081/reumatismo.2025.1935","DOIUrl":"10.4081/reumatismo.2025.1935","url":null,"abstract":"<p><strong>Objective: </strong>To comprehensively trace the historical journey of adrenal gland research from the first anatomical descriptions in the 16th century to the development and clinical application of cortisone in the mid-20th century.</p><p><strong>Methods: </strong>This review examines key phases in adrenal gland research, including anatomical discoveries, microscopic studies, experimental physiology, biochemical advancements, and clinical applications. We analyzed primary historical sources, scientific papers, and medical records to construct a chronological narrative of adrenal gland understanding and cortisone development.</p><p><strong>Results: </strong>The review highlights significant milestones, beginning with Bartolomeo Eustachio's 1564 discovery of the adrenal glands. It details the crucial microscopic phase, initiated by Moritz Nagel's 1836 study, which revealed the cortex-medulla distinction. Julius Arnold's 1866 description of the three-zone cortical structure and the identification of chromaffin cells are also discussed. The experimental physiology phase, featuring groundbreaking work by Thomas Addison and Charles-Édouard Brown-Séquard, established the vital role of the adrenal cortex. The biochemical phase, marked by the isolation and synthesis of cortisone, is examined in depth, with particular focus on the contributions of Edward Calvin Kendall, Tadeus Reichstein, and others. Finally, the clinical phase is detailed, emphasizing Philip Showalter Hench's revolutionary application of cortisone in rheumatoid arthritis treatment in 1948.</p><p><strong>Conclusions: </strong>This historical journey demonstrates how advancements in anatomy, histology, physiology, and biochemistry synergistically contributed to our understanding of the adrenal glands. The development of cortisone, culminating with this collective knowledge, revolutionized the treatment of inflammatory diseases, marking a significant milestone in medical history and setting the stage for modern endocrinology and rheumatology.</p>","PeriodicalId":21222,"journal":{"name":"Reumatismo","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-03-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145557884","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Long-term efficacy of intra-articular viscosupplementation in delaying hip osteoarthritis radiographic progression: a retrospective, non-controlled study from the ANTIAGE registry.","authors":"Alberto Migliore, Nazzareno Iannarelli, Antonio Devito, Raffaele Saporito, Luca Saccone, Irene Beriotto, Michele Grassi, Eugenio Cunego, Annamaria Paglionico, Sandro Tormenta","doi":"10.4081/reumatismo.2026.1917","DOIUrl":"10.4081/reumatismo.2026.1917","url":null,"abstract":"<p><strong>Objective: </strong>Viscosupplementation is a recognized treatment for improving pain and joint function in the long term. The study aimed to analyze the radiological changes after 2 years of treatment with HyalOne® (Hyalubrix® 60 Italian brand name) in patients with symptomatic hip osteoarthritis (OA). The primary objective was to evaluate the ability of delaying hip OA progression, measured by the percentage of patients showing no radiological Kellgren-Lawrence (K-L) grade change ≥1 at 2 years. Secondary objectives included variations in pain at rest, Lequesne index, and non-steroidal anti-inflammatory drug (NSAID) intake after 2 years of treatment.</p><p><strong>Methods: </strong>Data of patients suffering from symptomatic hip OA treated with HyalOne®/Hyalubrix® 60 were selected from the ANTIAGE national registry. The analysis included 78 patients aged 50 to 80 years with radiological and clinical OA (K-L grade 1-3), body mass index ≤35, and at least 2 years of radiological follow-up. Patients with significant comorbidities or who received previous treatment within two years after HyalOne®/Hyalubrix® 60 were excluded.</p><p><strong>Results: </strong>This retrospective observational non-controlled study showed that intra-articular injections with HyalOne®/Hyalubrix® 60 were well tolerated and may help maintain clinical and radiographic stability in patients with hip OA. At 2-year follow-up, the K-L grade remained stable in 72 hips (92.31%). Symptom reduction was also observed, supporting lower NSAID consumption and the avoidance of the related adverse events.</p><p><strong>Conclusions: </strong>These results are clinically meaningful and support the treatment with HyalOne® as a valuable background therapy for maintaining clinical and radiographic stability in patients with hip OA.</p>","PeriodicalId":21222,"journal":{"name":"Reumatismo","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-03-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147309591","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"IgG4-related disease: findings from a retrospective cohort in Colombia.","authors":"Diana N Rincón-Riaño, Daniel E Rodríguez-Ariza, Cristhyan Pacheco, Laura J Garcia-Ballesteros, Luisa Fernanda Galindo, Fabio A Torres","doi":"10.4081/reumatismo.2025.1887","DOIUrl":"10.4081/reumatismo.2025.1887","url":null,"abstract":"<p><strong>Objective: </strong>IgG4-related disease (IgG4-RD) is a recently described fibroinflammatory disorder. The most common presentations include salivary and lacrimal gland hypertrophy, orbitary disease, autoimmune pancreatitis, retroperitoneal fibrosis, and tubulointerstitial nephritis. Lymphoplasmacytic infiltration, fibrosis, IgG4+ cell hyperplasia, and elevated IgG4 serum levels are the primary pathophysiological findings related to the disease. We describe for the first time the epidemiology and clinical manifestations in our country.</p><p><strong>Methods: </strong>A descriptive study of patients from a retrospective cohort based on clinical records of adults with IgG4-RD treated between 2014 and 2021 in a high-complexity national center.</p><p><strong>Results: </strong>23,381 patients from 12 centers nationwide until December 2021 provided demographic data. We limited the search through ICD-10 coding: M358, M359, and M368, other specified systemic involvement of connective tissue; L948, other specified localized connective tissue disorders; D472, monoclonal gammopathy; K861, other chronic pancreatitis; H051, H059, and H063, chronic inflammatory disease of orbit; and C488, malignant neoplasm of overlapping sites of retroperitoneum and peritoneum. Thirty-three patients were identified with IgG4-RD based on comprehensive diagnostic criteria. A definitive diagnosis was obtained in 48.48% of patients, a probable diagnosis in 27.7%, and a possible diagnosis in 24.24%. A total of 21 patients were female, with a male/female (M/F) ratio of 1/1.75. The median age at diagnosis was 53.87 years (interquartile range of 27.06), the minimum age of diagnosis was 11.53 years, and the maximum was 79.18 years. Regarding clinical presentation at diagnosis, ocular/orbitary involvement was present in 16 patients (48.48%), followed by head and neck in 10 patients (30.30%), and biliary tract/gastrointestinal in 9 (27.27%). A single-organ compromise was identified in 15 patients (45.45%), and 18 patients (54.55%) had two or more organs affected, with lymphatic and ocular/orbitary being the most commonly reported.</p><p><strong>Conclusions: </strong>Epidemiologic and demographic data on IgG4-RD in our country are similar to those in the world medical literature. The higher frequency of the disease in males above 65 years and females under 65 years suggests distinct pathophysiologic factors related to sex and age. This work has a limitation of subreports or misreports, which physicians can make when registering ICD-10 codes in clinical records. Nonetheless, it is the legal record in Colombia and requires an analysis like the one made in this study.</p>","PeriodicalId":21222,"journal":{"name":"Reumatismo","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-03-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145445737","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Computed tomography of interstitial lung disease in systemic sclerosis: dataset and deep learning model for pulmonary lesion segmentation.","authors":"Amir M Vahdani, Aida Mohamadi, Sepehr Nayebirad, Shayan Forghani, Elaheh Karimi, Yousef Vojgani, Zahra Tamartash, Mehrnaz Asadi, Leila Aghaghazvini, Farhad Gharibdoost, Hoda Kavosi","doi":"10.4081/reumatismo.2026.1920","DOIUrl":"10.4081/reumatismo.2026.1920","url":null,"abstract":"<p><strong>Objective: </strong>Systemic sclerosis-associated interstitial lung disease (SSc-ILD) is a major cause of morbidity and mortality in systemic sclerosis patients. High-resolution computed tomography (HRCT) plays a crucial role in SSc-ILD diagnosis and management. In this study, we aimed to develop and evaluate a deep learning-based automated segmentation model for quantifying SSc-ILD lesions in HRCT images and assess its clinical relevance.</p><p><strong>Methods: </strong>We developed a convolutional neural network model to segment normal lung, established fibrosis (EF), and ground-glass opacity (GGO) in HRCT scans from 40 SSc-ILD patients. The model was trained and evaluated using 8-fold cross-validation. Segmentation performance was assessed using the Dice similarity coefficient (DSC). Correlations between predicted lesion volumes and pulmonary function test (PFT) metrics were analyzed using Spearman's ρ.</p><p><strong>Results: </strong>Our model achieved a total lesion (EF+GGO) DSC of 78%. Class-wise segmentation performance was lower for EF (DSC: 70%) compared to GGO (DSC: 73%). Predicted lesion volumes showed significant negative correlations with forced expiratory volume in one second (FEV1) (ρ=-0.64, p<0.001) and FEV1/forced vital capacity (ρ=-0.73, p<0.001). We also created the SICCS dataset, a public dataset of SSc-ILD HRCT images with expert-annotated segmentation labels.</p><p><strong>Conclusions: </strong>Deep learning-based automated segmentation can help quantify SSc-ILD lesions in HRCT images and provide clinically relevant information. The model's performance is comparable to previous studies, and the predicted lesion volumes correlate significantly with PFT metrics. This approach shows promise for aiding in SSc-ILD diagnosis, monitoring, and clinical decision-making, although further validation with larger datasets is needed.</p>","PeriodicalId":21222,"journal":{"name":"Reumatismo","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-03-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147309604","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The Italian Society for Rheumatology guidelines for the treatment of patients with rheumatoid arthritis and interstitial lung disease.","authors":"Chiara Crotti, Nicola Ughi, Carlo Alberto Scirè, Fabrizio Luppi, Fabiola Atzeni, Silvia Bosello, Roberto Caporali, Marina Carotti, Greta Carrara, Giulia Cassone, Lorenzo Cavagna, Lucia Dardani, Enrico De Lorenzis, Gian Luca Erre, Angelo Fassio, Roberto Gerli, Sergio Alfonso Harari, Florenzo Iannone, Gianpiero Landolfi, Andrea Lo Monaco, Silvia Mancuso, Marco Matucci-Cerinic, Maria Antonietta Mazzei, Venerino Poletti, Massimo Radin, Davide Rozza, Fausto Salaffi, Stefano Salvioli, Gian Domenico Sebastiani, Silvia Tonolo, Carlo Vancheri, Andreina Manfredi, Marco Sebastiani","doi":"10.4081/reumatismo.2025.1865","DOIUrl":"10.4081/reumatismo.2025.1865","url":null,"abstract":"<p><strong>Objective: </strong>In the absence of national and European guidelines on the treatment of rheumatoid arthritis (RA) with interstitial lung disease (ILD), the Italian Society of Rheumatology decided to develop national clinical practice guidelines on the management of patients with RA-ILD in accordance with the requirements of the National Guideline System of the National Institute of Health.</p><p><strong>Methods: </strong>The development process included a systematic review of the available evidence and its adaptability to the Italian context, followed by a consultation with experts in rheumatology, respiratory diseases, radiology, and representatives of the health professions and patients.</p><p><strong>Results: </strong>The panel decided to develop recommendations in three main scenarios. The first section of recommendations is focused on drugs indicated for RA to assess their safety and efficacy in RA-ILD. The second set of recommendations covered the drugs indicated for the treatment of ILD in patients with RA-ILD (to assess their efficacy and safety in patients with RA). The third part of these guidelines dealt with drugs indicated for the treatment of RA-ILD upon first-line failure. Moreover, the lack or absence of scientific evidence in literature on certain topics, such as the value of a multidisciplinary treatment approach and lung transplantation, led to the decision to proceed through expert consensus to develop good clinical practice guidelines.</p><p><strong>Conclusions: </strong>These guidelines represent a fundamental step towards improving the health management of patients with rheumatological diseases in Italy by providing specific and evidence-based guidelines for the management of RA-ILD. Their use is intended to promote health and reduce the burden of morbidity and mortality in this vulnerable population.</p>","PeriodicalId":21222,"journal":{"name":"Reumatismo","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-03-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145542480","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Macrophage activation syndrome and rapidly progressive lung disease as life-threatening manifestations of anti-MDA5 antibody disease. A case report.","authors":"Filippo Gozzi, Chiara Nani, Caterina Vacchi, Luca Caffarri, Dario Andrisani, Marco Sebastiani, Cecilia Burattini","doi":"10.4081/reumatismo.2025.1912","DOIUrl":"10.4081/reumatismo.2025.1912","url":null,"abstract":"<p><p>Anti-melanoma differentiation-associated protein 5 (MDA5) antibody-positive syndrome is a severe condition classified among the idiopathic inflammatory myopathies associated with rapidly progressive interstitial lung disease (RP-ILD). The present case report describes a complex patient with RP-ILD in the context of anti-MDA5-positive dermatomyositis (DM). The patient showed severe lung and skin manifestations involving hands, arms, and face, and the disease was first complicated by acute renal failure and then by macrophage activation syndrome (MAS). Finally, infectious risk delayed an effective treatment, probably increasing the rapid progression of lung involvement. A pathogenetic link between DM and MAS has been recently suggested; anti-MDA5 positive patients present higher serum soluble CD163 levels, which is a scavenger receptor for the hemoglobin/haptoglobin complex expressed on macrophages and a marker of macrophage activation in various conditions, including MAS. The patient has been treated according to the American College of Rheumatology/American College of Chest Physicians guidelines, adapting the strategy to his infectious complications. Beyond the complex clinical picture and treatment strategy, this case highlights the challenging decision-making process involved in the management of acute respiratory failure in patients with acute exacerbation of interstitial lung disease. The patient underwent helmet non-invasive ventilation (NIV), with a favorable clinical response. Helmet NIV has demonstrated advantages over mask interfaces, including better patient comfort, reduced air leaks, and more consistent delivery of positive end-expiratory pressure. These features contribute to lower inspiratory effort and improved clinical outcomes, including reduced intubation and mortality rates. In conclusion, patients with anti-MDA5 antibody disease need a multidisciplinary approach, including expert rheumatologists, pulmonologists, and radiologists for both monitoring and disease management.</p>","PeriodicalId":21222,"journal":{"name":"Reumatismo","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2026-03-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145810835","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Neonatal Behçet's syndrome: a case report and literature review.","authors":"Sandra Catarina Lino Ferreira Ferraz, David Rabiço-Costa, António Andrade, Pedro Barros, Mariana Rodrigues, Iva Brito","doi":"10.4081/reumatismo.2026.1902","DOIUrl":"10.4081/reumatismo.2026.1902","url":null,"abstract":"<p><p>Behçet's syndrome is a rare, chronic inflammatory disorder characterized by recurrent oral and genital ulcers, skin lesions, and eye inflammation. While most children born to mothers with Behçet's syndrome remain asymptomatic, some newborns may develop symptoms due to unclear pathological mechanisms. A male newborn, born at 39 weeks to a mother with a 14-year history of Behçet's syndrome, developed oral lesions on the 10th day of life. The lesions progressed, becoming ulcerated and causing painful feeding. Additional findings included a few facial pustules, pathergy, leukocytosis, and elevated inflammatory markers. Despite multiple therapies, the condition persisted. A biopsy of the tongue lesion was performed, and a diagnosis of neonatal Behçet's syndrome was made based on the clinical course, negative microbiological studies, and maternal history. The patient was treated with intravenous methylprednisolone, followed by oral steroids, with resolution within 3 months. Neonatal Behçet's syndrome is a very rare condition, with only a few cases reported in the literature. Most cases have mild clinical presentations, resolving spontaneously or with corticosteroids. However, severe cases with airway obstruction or neurological complications have been reported. This case demonstrates a favorable and self-limiting course. The underlying mechanisms causing symptoms in newborns of mothers with Behçet's syndrome remain unclear. Reporting these rare cases can help identify risk factors and improve early diagnosis.</p>","PeriodicalId":21222,"journal":{"name":"Reumatismo","volume":" ","pages":""},"PeriodicalIF":1.2,"publicationDate":"2026-03-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146181962","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}