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Real-world safety data of first-line drugs for rheumatoid arthritis: insights from the Portuguese Reuma.pt database. 类风湿性关节炎一线药物的真实世界安全性数据:来自葡萄牙Reuma.pt数据库的见解
IF 1.4 4区 医学
ARP Rheumatology Pub Date : 2025-10-01 DOI: 10.63032/XQFS6110
Joana Ramos Rodrigues, Luís Silva Pires, Luís Inês, Manuel Morgado, Maria Pontes Ferreira, Anita Cunha, Susana Almeida, Mariana Emília Santos, Ana Catarina Moniz, Daniel Melim, Miguel Bernardes, Carlos Marques Gomes, Mariana Diz Lopes, Daniel Carvalho, Joaquim Polido Pereira, João Aguiar, Inês Sopa, Cátia Duarte, Filipa Canhão André, Sara Alves Costa, Cláudia Miguel, Ana Rita Vieira, Leonor Reynolds, Marina Oliveira, Filipe Pinheiro, Catarina Abreu, Susana Matias, Ana Catarina Duarte, Ana Teodósio Chícharo, Vítor Silvestre Teixeira, Pedro Miguel Teixeira, Rita Fonseca, Maria Helena Lourenço, Catarina Rua, Catarina Silva, Margarida Oliveira, Ana Águeda, Marília Rodrigues, Helena Assunção, Patrícia Nero, Maura Couto, Helena Santos, Duarte Augusto, Maria Francisca Magalhães, João Dias, Teresa Melo, Carla Campinho Ferreia, Paulo Pereira, João Lagoas Gomes, Ana Raposo, Ana Valido, Filipa Farinha, José António Pereira Silva, António Vilar, José Tavares-Costa, Jorge Garcia, Filipe Araújo, Graça Sequeira, Ana Cordeiro, Ana Filipa Mourao
{"title":"Real-world safety data of first-line drugs for rheumatoid arthritis: insights from the Portuguese Reuma.pt database.","authors":"Joana Ramos Rodrigues, Luís Silva Pires, Luís Inês, Manuel Morgado, Maria Pontes Ferreira, Anita Cunha, Susana Almeida, Mariana Emília Santos, Ana Catarina Moniz, Daniel Melim, Miguel Bernardes, Carlos Marques Gomes, Mariana Diz Lopes, Daniel Carvalho, Joaquim Polido Pereira, João Aguiar, Inês Sopa, Cátia Duarte, Filipa Canhão André, Sara Alves Costa, Cláudia Miguel, Ana Rita Vieira, Leonor Reynolds, Marina Oliveira, Filipe Pinheiro, Catarina Abreu, Susana Matias, Ana Catarina Duarte, Ana Teodósio Chícharo, Vítor Silvestre Teixeira, Pedro Miguel Teixeira, Rita Fonseca, Maria Helena Lourenço, Catarina Rua, Catarina Silva, Margarida Oliveira, Ana Águeda, Marília Rodrigues, Helena Assunção, Patrícia Nero, Maura Couto, Helena Santos, Duarte Augusto, Maria Francisca Magalhães, João Dias, Teresa Melo, Carla Campinho Ferreia, Paulo Pereira, João Lagoas Gomes, Ana Raposo, Ana Valido, Filipa Farinha, José António Pereira Silva, António Vilar, José Tavares-Costa, Jorge Garcia, Filipe Araújo, Graça Sequeira, Ana Cordeiro, Ana Filipa Mourao","doi":"10.63032/XQFS6110","DOIUrl":"10.63032/XQFS6110","url":null,"abstract":"<p><strong>Objectives: </strong>To assess adverse events (AE) associated with first-line therapies for rheumatoid arthritis (RA) in a real-world setting.</p><p><strong>Material and methods: </strong>Retrospective multicenter cohort study of patients fulfilling classification criteria for RA and followed up in 66 rheumatology centers from the Rheumatic Diseases Portuguese Registry (Reuma.pt). All AE reports associated with first-line disease-modifying antirheumatic drugs (DMARDs) up to November 2024 were included. Demographic and clinical data were analyzed, and AE characteristics were investigated. Categorical and continuous variables were compared using chi-square tests and Mann-Whitney U tests, respectively. Statistical significance was defined as p < .05.</p><p><strong>Results: </strong>Among 1 880 AE entries, 377 (20.1%) were attributed to first-line DMARDs, most commonly methotrexate (62.9%) although no information on drug dosage was available. The median age at AE occurrence was 58.6 years (IQR: 19.32), and 82% were female. A causality assessment was available in 317 reports, with 40.3% deemed \"probable,\" 28.1% \"possible,\" and 10.6% \"definitive.\" Severe AE were reported in 13.2% of cases, with pulmonary involvement being the most common (20.8%).Overall, 46.7% of patients discontinued treatment for any reason. Male sex was significantly associated with severe AE (OR = 2.31; 95% CI: 1.17-4.55; p = .014), and older patients were more likely to experience severe AE (median age 65.7 vs. 57.9 years; p < .001). The most affected body organ systems were gastrointestinal (9.3%), skin (8.2%), and hematological (8.2%). The median time to AE onset from treatment initiation was 1.27 years (IQR: 2.63), and from disease onset was 8.56 years (IQR: 11.76).</p><p><strong>Conclusions: </strong>AE related to first-line RA therapies can lead to significant clinical consequences, including treatment discontinuation. Male sex and advanced age were associated with increased AE severity. The most affected systems appear consistent with known drug safety profiles, particularly that of methotrexate; however, the absence of information regarding drug dosage precludes more detailed conclusions. These findings emphasize the need for individualized monitoring strategies and improved pharmacovigilance to optimize long-term treatment safety and adherence in RA management.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"4 4","pages":"267-275"},"PeriodicalIF":1.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145890319","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}
引用次数: 0
Interstitial lung disease in Sjögren's disease: the portrait of a national cohort. Sjögren疾病中的间质性肺病:一个国家队列的画像。
IF 1.4 4区 医学
ARP Rheumatology Pub Date : 2025-10-01 DOI: 10.63032/ZNHW2234
Ana Catarina Duarte, Susana Matias, Carolina Bargado, Matilde Bandeira, Vasco C Romão, Carlos Marques Gomes, Miguel Bernardes, Miguel Natal, Ana Catarina Moniz, Maria João Gonçalves, Mariana Emília Santos, Filipa Canhão André, Maria João Cadório, Sara Alves Costa, Ana Rita Vieira, Beatriz Mendonça, Filipe Barcelos, Anita Cunha, Maria Pontes Ferreira, Anabela Barcelos, Cláudia Pinto Oliveira, Rita Fonseca, Catarina Silva, Luísa Brites, Inês Almeida, Duarte Augusto, Diana Belchior Raimundo, Maria José Santos
{"title":"Interstitial lung disease in Sjögren's disease: the portrait of a national cohort.","authors":"Ana Catarina Duarte, Susana Matias, Carolina Bargado, Matilde Bandeira, Vasco C Romão, Carlos Marques Gomes, Miguel Bernardes, Miguel Natal, Ana Catarina Moniz, Maria João Gonçalves, Mariana Emília Santos, Filipa Canhão André, Maria João Cadório, Sara Alves Costa, Ana Rita Vieira, Beatriz Mendonça, Filipe Barcelos, Anita Cunha, Maria Pontes Ferreira, Anabela Barcelos, Cláudia Pinto Oliveira, Rita Fonseca, Catarina Silva, Luísa Brites, Inês Almeida, Duarte Augusto, Diana Belchior Raimundo, Maria José Santos","doi":"10.63032/ZNHW2234","DOIUrl":"10.63032/ZNHW2234","url":null,"abstract":"<p><strong>Introduction: </strong>Apart from exocrine glands involvement with sicca symptoms, several extra-glandular manifestations can occur in Sjögren's disease (SjD) such as pulmonary manifestations. Interstitial lung disease (ILD) is the most common lung manifestation in SjD. We aim to evaluate the presence of ILD in a national cohort of patients with SjD, identify variables associated with its development and progression, as well as describe the treatment used for SjD-ILD and its effectiveness and tolerability.</p><p><strong>Methods: </strong>We conducted an observational multicenter study of SjD-ILD patients prospectively followed in Reuma.pt. Demographic and clinical data were collected. We compared patient characteristics between groups using Chi-square or Fisher's exact test, Mann-Whitney or independent samples t-test, as appropriate. Logistic regression analysis was used to identify predictors of SjD- ILD. A linear mixed model with random intercept was used to compare results from pulmonary function tests (PFTs) before and after immunosuppression initiation.</p><p><strong>Results: </strong>Of the 1532 patients enrolled in the Reuma.pt-SjD protocol, 1333 (87%) had information on the presence of pulmonary manifestations. Among these, 127 (9.5%) had documented lung involvement, with ILD being the most common manifestation (74%). Ever smoking (OR=2.175; [95%CI:1.214-3.899]; p=0.009) and older age at SjD diagnosis (OR=1.047 per year; [95%CI:1.025-1.069]; p<0.001) were predictors of ILD. Nonspecific interstitial pneumonia was the most frequent ILD pattern (45.7%). Immunosuppression was used in 62 (66%) SjD-ILD patients and antifibrotics in eight patients (in seven of them in association with immunosuppression). Among the 26 patients with serial PFTs available, 10 (38.5%) showed ILD progression. Progressors had a higher forced vital capacity (FVC 96.8 ± 22.1 vs. 71.1 ± 21.4%; p=0.007) and diffusion capacity for carbon monoxide (DLCO 75.5 ± 12.1 vs. 50.2 ± 16.6%; p=0.002) at baseline and tend to have hypergammaglobulinemia more often (80% vs. 43.8%; p=0.069). Nevertheless, immunosuppression interrupted the decline of FVC (absolute value) and DLCO (percent predicted).</p><p><strong>Conclusion: </strong>This work demonstrated that a substantial proportion of SjD-ILD patients present with progressive fibrosis. Immunosuppression seems to delay the progression of lung disease. Therefore, identifying predictors for ILD development and progression is essential for recognizing which patients will require closer monitoring and intervention.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"4 4","pages":"276-283"},"PeriodicalIF":1.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145890240","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}
引用次数: 0
Prevalence and clinical risk factors for methotrexate intolerance in patients with juvenile idiopathic arthritis. 青少年特发性关节炎患者甲氨蝶呤不耐受的患病率及临床危险因素。
IF 1.4 4区 医学
ARP Rheumatology Pub Date : 2025-10-01 DOI: 10.63032/WYVM2561
Stefan Antonije Djordjevic, Predrag Ostojic, Goran Radunovic, Natasa Mujovic, Smiljka Kovacevic, Dusica Novakovic, Dragana Lazarevic, Hristina Petrovic, Maja Bijelic, Tijana Dimkic-Tomic, Andjela Dimkic-Milenkovic, Vladimir Milenkovic, Gordana Susic
{"title":"Prevalence and clinical risk factors for methotrexate intolerance in patients with juvenile idiopathic arthritis.","authors":"Stefan Antonije Djordjevic, Predrag Ostojic, Goran Radunovic, Natasa Mujovic, Smiljka Kovacevic, Dusica Novakovic, Dragana Lazarevic, Hristina Petrovic, Maja Bijelic, Tijana Dimkic-Tomic, Andjela Dimkic-Milenkovic, Vladimir Milenkovic, Gordana Susic","doi":"10.63032/WYVM2561","DOIUrl":"10.63032/WYVM2561","url":null,"abstract":"<p><strong>Aims: </strong>Methotrexate (MTX) is a basic therapy for juvenile idiopathic arthritis (JIA). MTX intolerance can significantly impact quality of life, treatment adherence and outcomes. We aimed to assess the prevalence of MTX intolerance and to identify clinical factors associated with intolerance in children and adolescents with JIA.</p><p><strong>Methods: </strong>This cross-sectional study was conducted at a large pediatric rheumatology referral center between July 2019 and July 2021. It included 94 patients with JIA, aged up to 19 years, who had been treated with MTX (oral or subcutaneous) for at least three months. Patients with systemic JIA and those exhibiting toxic MTX effects were excluded. Demographic and clinical data were collected, and MTX intolerance was assessed using the Methotrexate Intolerance Severity Score (MISS) questionnaire. MTX intolerance was defined as a total MISS score of ≥6, including at least one anticipatory, associative, or behavioral symptom. Statistical analyses were performed to compare MTX-tolerant and MTX-intolerant groups.</p><p><strong>Results: </strong>The median patient age was 9.9 years (range 2.3-18.5 years), and 69 (73.4%) were female. The median age at disease onset was 3.7 years, and the median duration of MTX therapy was 2.3 years. The prevalence of MTX intolerance was 24.5%. The most common symptom was nausea after MTX intake (38.3%), followed by behavioral problems such as irritability or restlessness. Intolerant patients were significantly older at disease onset and MTX initiation (U=577.0, p=0.04 and U=555.5, p=0.02, respectively), and MTX as first-line therapy was more frequent in this group (X2=5.78, p=0.02). There was a strong positive correlation between age at disease onset and MTX initiation (r=0.9, p<0.001).</p><p><strong>Conclusions: </strong>MTX intolerance is relatively common in pediatric patients with JIA and is associated with older age at disease onset and MTX initiation, as well as the use of MTX as first-line therapy.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"4 4","pages":"285-293"},"PeriodicalIF":1.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145890395","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}
引用次数: 0
Upadacitinib in rheumatoid nodules: beyond joint control. Upadacitinib治疗类风湿性结节:关节无法控制。
IF 1.4 4区 医学
ARP Rheumatology Pub Date : 2025-10-01 DOI: 10.63032/HZSJ4098
Margarida Lucas Rocha, Rodrigo Rei, Vítor Silvestre Teixeira
{"title":"Upadacitinib in rheumatoid nodules: beyond joint control.","authors":"Margarida Lucas Rocha, Rodrigo Rei, Vítor Silvestre Teixeira","doi":"10.63032/HZSJ4098","DOIUrl":"10.63032/HZSJ4098","url":null,"abstract":"","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"4 4","pages":"317-319"},"PeriodicalIF":1.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145890370","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}
引用次数: 0
Femoral head ochronotic pigmentation in a patient with alkaptonuria and secondary hip osteoarthritis. 尿酸尿伴继发性髋关节骨关节炎患者股骨头衰老性色素沉着1例。
IF 1.4 4区 医学
ARP Rheumatology Pub Date : 2025-10-01 DOI: 10.63032/MYMI3386
Rita Moniz, Filipe Araújo, Gonçalo Viana
{"title":"Femoral head ochronotic pigmentation in a patient with alkaptonuria and secondary hip osteoarthritis.","authors":"Rita Moniz, Filipe Araújo, Gonçalo Viana","doi":"10.63032/MYMI3386","DOIUrl":"10.63032/MYMI3386","url":null,"abstract":"<p><p>Alkaptonuria is a rare autosomal recessive disorder caused by homogentisic acid dioxygenase deficiency, leading to ochronotic pigment deposition in connective tissues and secondary osteoarthritis. We report the case of a 59-year-old female with longstanding shoulder, spine, and hip pain, diagnosed with alkaptonuria at age 45. Imaging revealed generalized osteoarthritis, most severe in the lumbar spine and right hip. Despite multimodal management, hip pain progressed, requiring total hip replacement with significant functional improvement. The surgical specimen revealed characteristic dark-bluish ochronotic pigmentation of the femoral head. This vignette highlights the disabling musculoskeletal manifestations of alkaptonuria and the need for timely recognition and surgical management in advanced disease.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"4 4","pages":"311-312"},"PeriodicalIF":1.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145890293","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}
引用次数: 0
Real-world efficacy and retention of guselkumab in psoriatic arthritis: insights from a 12-month multicenter study. guselkumab在银屑病关节炎中的实际疗效和保留:来自一项为期12个月的多中心研究的见解
IF 1.4 4区 医学
ARP Rheumatology Pub Date : 2025-07-01 DOI: 10.63032/IEXQ7963
Nicola Farina, Nicola Boffini, Antonella Adinolfi, Alberto Batticciotto, Alessandro Tomelleri, Adriana Cariddi, Stefania Calvisi, Elena Baldissera, Marco Matucci-Cerinic, Antonella Cappelli, Oscar Epis, Lorenzo Dagna
{"title":"Real-world efficacy and retention of guselkumab in psoriatic arthritis: insights from a 12-month multicenter study.","authors":"Nicola Farina, Nicola Boffini, Antonella Adinolfi, Alberto Batticciotto, Alessandro Tomelleri, Adriana Cariddi, Stefania Calvisi, Elena Baldissera, Marco Matucci-Cerinic, Antonella Cappelli, Oscar Epis, Lorenzo Dagna","doi":"10.63032/IEXQ7963","DOIUrl":"10.63032/IEXQ7963","url":null,"abstract":"<p><strong>Background: </strong>Psoriatic arthritis (PsA) is a chronic inflammatory disease that affects joints, skin, and other structures. Guselkumab, an IL-23 inhibitor, has shown efficacy in clinical trials, but real-world data on its long-term use in PsA are limited. This study aimed to assess the efficacy, safety, and retention rate of guselkumab in a real-world cohort of PsA patients over 12 months.</p><p><strong>Methods: </strong>This retrospective study included PsA patients treated with guselkumab for at least 12 months across three medical centers. Patients were assessed at baseline and at 12 months using PsA disease activity scores. Retention rate at 12 months and reasons for discontinuation were recorded. Statistical analyses included descriptive statistics, Mann-Whitney tests for changes in disease activity, and Cox regression for identifying factors associated with treatment discontinuation.</p><p><strong>Results: </strong>We included 70 PsA patients. Significant reductions in disease activity were observed at 12 months for DAS28, DAPSA, MASES and ASDAS. The 12-month retention rate was 79%, with discontinuation primarily due to inefficacy. No significant adverse events were reported. Cox regression analysis found no significant associations between baseline characteristics and treatment discontinuation.</p><p><strong>Conclusions: </strong>Guselkumab demonstrated significant efficacy in reducing disease activity and a favorable retention rate over 12 months in a real-world PsA cohort. These findings support guselkumab as an effective treatment for PsA, although further prospective studies are needed to confirm long-term safety and efficacy.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"4 3","pages":"215-220"},"PeriodicalIF":1.4,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145201647","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}
引用次数: 0
Severe tertiary hyperparathyroidism as a rare mimicker of sacroiliitis. 严重的三级甲状旁腺功能亢进是一种罕见的骶髂炎的模拟。
IF 1.4 4区 医学
ARP Rheumatology Pub Date : 2025-07-01 DOI: 10.63032/HXJM4300
Carla Ferreira-Campinho, Hugo Gonçalves, Paulo Pereira, Ana Margarida Correia, Joana Leite Silva
{"title":"Severe tertiary hyperparathyroidism as a rare mimicker of sacroiliitis.","authors":"Carla Ferreira-Campinho, Hugo Gonçalves, Paulo Pereira, Ana Margarida Correia, Joana Leite Silva","doi":"10.63032/HXJM4300","DOIUrl":"10.63032/HXJM4300","url":null,"abstract":"<p><p>A 42-year-old female patient was referred for rheumatology evaluation due to suspected sacroiliitis, incidentally detected on a pelvic computerized tomography (CT). Her medical history was notable for chronic kidney disease (CKD) complicated with tertiary hyperparathyroidism. A few months before rheumatologic evaluation, she underwent total parathyroidectomy. CT imaging was reviewed revealing prominent erosions of the iliac articular surfaces of the sacroiliac joints. Given the absence of inflammatory low back pain and the patients history of severe hyperparathyroidism, the sacroiliac changes were interpreted as most likely secondary to metabolic bone disease rather than inflammatory spondyloarthropathy. CT findings in hyperparathyroidism may include widening of joint spaces and irregularity of the articular surfaces. Involvement of the sacroiliac joints often manifests as erosions on the iliac sides, occasionally accompanied by reactive sclerosis. These features can mimic those seen in spondylarthritis, making the differential diagnosis challenging.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"4 3","pages":"249-250"},"PeriodicalIF":1.4,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145201659","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}
引用次数: 0
Eosinophilic granulomatosis with polyangiitis treated with Mepolizumab and Rituximab combination therapy - a case report. 美珠单抗和利妥昔单抗联合治疗嗜酸性肉芽肿病合并多血管炎1例报告
IF 1.4 4区 医学
ARP Rheumatology Pub Date : 2025-07-01 DOI: 10.63032/OWCP6172
Ana Catarina Moniz, Laura Gago, Mariana Emília Santos, Daniel Melim, Sara Dias Rodrigues, Paula Araújo, Jaime Cunha Branco, Maria João Gonçalves
{"title":"Eosinophilic granulomatosis with polyangiitis treated with Mepolizumab and Rituximab combination therapy - a case report.","authors":"Ana Catarina Moniz, Laura Gago, Mariana Emília Santos, Daniel Melim, Sara Dias Rodrigues, Paula Araújo, Jaime Cunha Branco, Maria João Gonçalves","doi":"10.63032/OWCP6172","DOIUrl":"10.63032/OWCP6172","url":null,"abstract":"<p><p>Eosinophilic granulomatosis with polyangiitis (EGPA) is a disease characterized by vasculitis affecting small blood vessels, primarily in the lungs, skin, and peripheral nervous system. Both rituximab and mepolizumab are recommended for EGPA treatment, but their combined use is uncommon. There have been only a few reported cases of using both drugs together. We present a 46-year-old man with a history of asthma and chronic rhinosinusitis presented with dyspnea, numbness in the feet, and skin lesions. He was diagnosed with ANCA-negative EGPA and treated with corticosteroids and immunosuppressants. Despite initial improvement, he developed respiratory symptoms and osteoporosis. Treatment with mepolizumab helped reduce corticosteroid dosage but was not effective in joint involvement. A combination of mepolizumab and rituximab was then initiated, resulting in significant improvement in joint symptoms and overall disease control. This case demonstrates the challenges of managing EGPA and the need for tailored treatment approaches. The combination of rituximab and mepolizumab proved effective in controlling different disease manifestations. Further studies are needed to evaluate the safety and efficacy of this combination therapy in EGPA.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"4 3","pages":"251-252"},"PeriodicalIF":1.4,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145201508","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}
引用次数: 0
Hypocomplementemic paraneoplastic vasculitis: a rare case of B-cell malignancy. 缺补性副肿瘤血管炎:罕见的b细胞恶性肿瘤病例。
IF 1.4 4区 医学
ARP Rheumatology Pub Date : 2025-07-01 DOI: 10.63032/QGLK2956
Omer Atakan Sogur, Dilara Bulut Gokten, Ridvan Mercan
{"title":"Hypocomplementemic paraneoplastic vasculitis: a rare case of B-cell malignancy.","authors":"Omer Atakan Sogur, Dilara Bulut Gokten, Ridvan Mercan","doi":"10.63032/QGLK2956","DOIUrl":"10.63032/QGLK2956","url":null,"abstract":"<p><p>Pemphigus foliaceus (PF) is a rare autoimmune blistering disease, occasionally associated with lymphoproliferative disorders. Urticarial vasculitis (UV) is classified as normocomplementemic or hypocomplementemic (HUV), the latter linked to systemic involvement and increased risk of malignancy. We present a rare case of atypical HUV syndrome in a 55-year-old female with a 24-year history of PF. She presented with recurrent heat, redness, and discoloration of the right hand. Examination revealed an erythematous-violaceous, edematous lesion with irregular but well-defined borders over the thenar region, without urticarial lesions. Laboratory evaluation showed marked hypocomplementemia (C3 0.73 g/L, C4 0.01 g/L), thrombocytopenia (32×10³/µL), leukocytosis, and positive ANA. Hepatomegaly was noted on systemic examination. Hematology consultation revealed a CD5-negative, CD19-positive B-cell lymphoproliferative disorder. Genetic testing excluded hereditary cancer mutations. This case underscores the clinical importance of recognizing atypical HUV presentations without urticaria and highlights the association of hypocomplementemia and thrombocytopenia with underlying hematologic malignancy. In patients with autoimmune background and cutaneous vasculitic lesions, hematologic malignancies should be considered, and thorough evaluation is essential to exclude paraneoplastic processes.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"4 3","pages":"256-257"},"PeriodicalIF":1.4,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145201573","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}
引用次数: 0
Clinical spectrum of cervical involvement in calcium pyrophosphate deposition disease. 焦磷酸钙沉积病累及颈椎的临床谱。
IF 1.4 4区 医学
ARP Rheumatology Pub Date : 2025-07-01 DOI: 10.63032/RZLZ8816
Carla Ferreira-Campinho, Hugo Gonçalves, Paulo Pereira, Ana Margarida Correia, Joana Leite Silva, Emanuel Costa
{"title":"Clinical spectrum of cervical involvement in calcium pyrophosphate deposition disease.","authors":"Carla Ferreira-Campinho, Hugo Gonçalves, Paulo Pereira, Ana Margarida Correia, Joana Leite Silva, Emanuel Costa","doi":"10.63032/RZLZ8816","DOIUrl":"10.63032/RZLZ8816","url":null,"abstract":"<p><p>Calcium pyrophosphate deposition (CPPD) disease is a common microcrystalline arthropathy in the elderly, The clinical spectrum includes both acute and chronic inflammatory arthritis, but crystals depositions may also occur without symptoms, with chondrocalcinosis identified incidentally on imaging. Axial involvement is less frequent than peripheral but has been increasingly recognized, particularly in the cervical spine. Its manifestations are heterogeneous and may mimic infectious, inflammatory, neoplastic or degenerative disorders, often leading to misdiagnosis. We report four cases of cervical CPPD disease that exemplify the main clinical phenotypes, from incidental crystals deposition to crowned dens syndrome, retro-odontoid pseudotumor and inflammatory discitis. They illustrate the diversity of cervical involvement and its potential for severe neurological complications. Diagnosis relies on clinical evaluation supported by imaging, with CT being the modality of choice for detecting calcifications, and MRI useful for assessing soft tissue masses, cord compression, or discitis changes. Management remains symptomatic, mainly with colchicine, glucocorticoids or NSAIDs, while surgery may be required in severe myelopathy. Awareness of cervical CPPD is essential to avoid unnecessary antibiotics or invasive procedures and to ensure timely and targeted management.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"4 3","pages":"241-246"},"PeriodicalIF":1.4,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145201558","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}
引用次数: 0
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