ACR open rheumatology最新文献

{"title":"Giant Aortic Aneurysm in Refractory Behçet's Disease Shrank with Tocilizumab Treatment: A Case Report.","authors":"Zi-Yi Song, Xin Zhi, Jia-Yi Geng, Xing Sun, Tian Liu, Zhan-Guo Li","doi":"10.1002/acr2.70071","DOIUrl":"10.1002/acr2.70071","url":null,"abstract":"<p><p>Behçet disease (BD) is characterized by recurrent oral and genital ulcers, skin lesions, and vascular involvement. Giant aortic aneurysm, a life-threatening complication of BD, often proves refractory to conventional therapies. Here, we present the case of a 71-year-old man with a 40-year history of BD complicated by a giant aortic aneurysm, which was successfully managed using the interleukin-6 (IL-6) receptor antagonist tocilizumab (TCZ). Despite years of tight treatment with high-dose glucocorticoids and immunosuppressants, there were frequent flares of disease and aortic aneurysm formation. Following the initiation of TCZ therapy, the patient achieved remarkable clinical improvement: the aortic aneurysm diameter decreased significantly from 10.15 × 7.83 cm to 6.22 × 5.12 cm, and disease activity, as measured by the Behçet's Disease Current Activity Form score, dropped from 8 to 1, indicating sustained disease control. This case underscores the potential of IL-6 inhibition as a transformative therapeutic strategy for refractory BD with severe vascular complications, offering new hope for patients with this challenging condition.</p>","PeriodicalId":93845,"journal":{"name":"ACR open rheumatology","volume":"7 7","pages":"e70071"},"PeriodicalIF":2.9,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12226098/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144562254","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical Images: Löffler endocarditis: a rare cardiac manifestation of eosinophilic granulomatosis with polyangiitis.","authors":"Frank A Scangarello, Asmaa Naguib Ahmed, Fatima K Alduraibi","doi":"10.1002/acr2.70056","DOIUrl":"10.1002/acr2.70056","url":null,"abstract":"","PeriodicalId":93845,"journal":{"name":"ACR open rheumatology","volume":"7 7","pages":"e70056"},"PeriodicalIF":2.9,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12207645/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144531584","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical Images: Stubborn low back pain under the eyes.","authors":"William Gil, Martin Soubrier","doi":"10.1002/acr2.70090","DOIUrl":"10.1002/acr2.70090","url":null,"abstract":"","PeriodicalId":93845,"journal":{"name":"ACR open rheumatology","volume":"7 7","pages":"e70090"},"PeriodicalIF":2.9,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12238772/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144593154","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Asymptomatic hypertension and bilateral renal artery stenosis in Takayasu arteritis.","authors":"Wan-Hao Tsai, Chiao-Feng Cheng","doi":"10.1002/acr2.70077","DOIUrl":"10.1002/acr2.70077","url":null,"abstract":"","PeriodicalId":93845,"journal":{"name":"ACR open rheumatology","volume":"7 7","pages":"e70077"},"PeriodicalIF":2.9,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12207014/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144531583","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Development of Eosinophilic Granulomatosis With Polyangiitis Despite Anti-Interleukin-5 Receptor Therapy: The First Case of Bilateral Central Retinal Artery Occlusion During Benralizumab Treatment.","authors":"Elena Treppo, Lucia Finocchio, Benedetta Fazzi, Maria Pillon, Luca Quartuccio","doi":"10.1002/acr2.70073","DOIUrl":"10.1002/acr2.70073","url":null,"abstract":"<p><p>Here, we describe a rare presentation of eosinophilic granulomatosis with polyangiitis (EGPA) under benralizumab therapy manifesting as bilateral central retinal artery occlusion (CRAO). The patient, a 61-year-old man with chronic eosinophilic rhinosinusitis and severe asthma, experienced sudden bilateral visual loss and transient amaurosis. Ophthalmologic evaluations, including a fundus examination and optical coherence tomography, confirmed CRAO, and laboratory test results revealed elevated markers of inflammation and positive antimyeloperoxidase antibodies in the context of normal eosinophil counts. Intensive immunosuppressive therapy led to resolution of systemic inflammation, although significant visual impairment persisted. These findings underscore the potential limitations of anti-interleukin-5 receptor therapy in preventing vasculitic complications in EGPA.</p>","PeriodicalId":93845,"journal":{"name":"ACR open rheumatology","volume":"7 6","pages":"e70073"},"PeriodicalIF":2.9,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12133382/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144217786","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Late-onset rheumatoid arthritis versus elderly-onset rheumatoid arthritis: understanding the key differences: comment on the article by Lee et al.","authors":"Galymzhan Togizbayev, Chokan Baimukhamedov, Galiya Аssanova, Khilola Mirakhmedova, Maya Goremykina","doi":"10.1002/acr2.70058","DOIUrl":"10.1002/acr2.70058","url":null,"abstract":"","PeriodicalId":93845,"journal":{"name":"ACR open rheumatology","volume":"7 6","pages":"e70058"},"PeriodicalIF":2.9,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12176420/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144328049","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Response to letter to the editor, \"Observational studies on glucocorticoid harm should always consider confounding by indication.\"","authors":"Patricia Katz, Kaleb Michaud","doi":"10.1002/acr2.70037","DOIUrl":"10.1002/acr2.70037","url":null,"abstract":"","PeriodicalId":93845,"journal":{"name":"ACR open rheumatology","volume":"7 6","pages":"e70037"},"PeriodicalIF":2.9,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12188616/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144487415","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical Images: Unilateral skin induration and pigmentation: a case of generalized morphea.","authors":"Wan-Hao Tsai, Ko-Jen Li","doi":"10.1002/acr2.70060","DOIUrl":"10.1002/acr2.70060","url":null,"abstract":"","PeriodicalId":93845,"journal":{"name":"ACR open rheumatology","volume":"7 6","pages":"e70060"},"PeriodicalIF":2.9,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12118400/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144176225","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Unveiling VEXAS Syndrome: When Skin Manifestations and Monoclonal Gammopathy Precede Myeloid-Lineage Hematologic Abnormality.","authors":"Laura Di Centa, Simone Longhino, Valeria Manfrè, Stefania Sacco, Luca Quartuccio","doi":"10.1002/acr2.70064","DOIUrl":"10.1002/acr2.70064","url":null,"abstract":"<p><p>VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a rare disorder caused by somatic UBA1 gene mutations, characterized by autoinflammation and hematologic abnormalities, particularly affecting myeloid-lineage progenitors. Sensitive markers include macrocytic anemia, vacuolization of bone marrow precursors, and myelodysplasia. Here, we report the first case of VEXAS syndrome presenting with neutrophilic dermatosis and a serum monoclonal component, without myeloid-lineage hematologic abnormalities atonset. This case underscores the importance of including VEXAS syndrome in the differential diagnosis when monoclonal gammopathy is associated with rheuamtologic features, particularly in older patients presenting with unexplained cutaneous inflammatory manifestations and a serum clonal component, as such presentations may precede the development of classical hematologic abnormalities.</p>","PeriodicalId":93845,"journal":{"name":"ACR open rheumatology","volume":"7 6","pages":"e70064"},"PeriodicalIF":2.9,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12125272/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144192587","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Muscle Vasculitis: A Novel Delineation of Distinct Subsets of Disease.","authors":"Thomas Khoo, Sarah Saxon, Barbara Koszyca, Bernice Gutschmidt, Vidya Limaye","doi":"10.1002/acr2.70062","DOIUrl":"10.1002/acr2.70062","url":null,"abstract":"<p><strong>Objective: </strong>The objective of this study was to assess the clinical and histopathologic features of muscle vasculitis.</p><p><strong>Methods: </strong>The electronic database of SA Pathology, wherein all muscle biopsy samples in South Australia are assessed, was searched (2000-2023), identifying cases of vasculitis. Cases were classified as systemic vasculitis (SV) or isolated muscle vasculitis (IMV). IMV occurred as either localized muscle disease (IMV-LMD) or with known connective tissue disease (IMV-CTD). Clinical and histologic comparisons between muscle vasculitis and biopsy-proven idiopathic inflammatory myopathy (IIM) were made.</p><p><strong>Results: </strong>From 3,090 muscle biopsy samples, 59 showed vasculitis (47.5% female, median age 64.1 [interquartile range (IQR) 51.5-75.3] years). SV was present in 32 of 59, IMV-LMD was present in 15 of 59, and IMV-CTD was present in 10 of 59. Patients with SV and IMV-LMD frequently had normal creatine kinase (CK) levels (SV: median 58.5 [IQR 25-152] IU/L; IMV-LMD: median 34.5 [IQR 21.5-88.5] IU/L). Antineutrophil cytoplasmic antibodies (ANCAs) were restricted to those with SV (myeloperoxidase-ANCA [13 of 32] vs proteinase 3-ANCA [2 of 32]). Compared to patients with IIM, patients with muscle vasculitis had less frequent weakness (29 of 59 vs 305 of 369; P < 0.01) but more distal muscle symptoms (21 of 59 vs 74 of 360; P = 0.02) and elevated C-reactive protein levels (35 of 59 vs 20 of 82; P < 0.01). Salient histologic features of IIM (lymphocytic infiltration of myofibers, myofiber necrosis, major histocompatibility complex class I up-regulation) occurred less frequently in muscle vasculitis than IIM (P < 0.01), suggesting myofiber integrity despite pathology in the vasculature.</p><p><strong>Conclusion: </strong>Muscle vasculitis can occur with SV or be isolated to the muscles and is rarely associated with CTD. Distinct from IIM, muscle vasculitis frequently lacks myofiber necrosis and/or infiltration; consequent preservation of muscle power and normal CK levels should not deter from muscle biopsy.</p>","PeriodicalId":93845,"journal":{"name":"ACR open rheumatology","volume":"7 6","pages":"e70062"},"PeriodicalIF":2.9,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12169899/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144310927","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}