Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases最新文献

{"title":"Reported Triggers and Self-reported Management Strategies in Children With Familial Mediterranean Fever.","authors":"Elif Kucuk, Safak Senpolat, Feray Kaya, Lutfiye Koru, Zelal Aydin, Eda Nur Dizman, Hatice Kubra Dursun, Merve Ozen Balci, Kubra Ozturk, Fatih Haslak","doi":"10.1097/RHU.0000000000002265","DOIUrl":"https://doi.org/10.1097/RHU.0000000000002265","url":null,"abstract":"<p><strong>Objectives: </strong>Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disorder. Data regarding the reported triggers of this rare disease are scarce. This study aimed to analyze the demographic data, clinical findings during the attacks, reported triggering, and self-reported management strategies of pediatric patients with FMF with exon 10 MEFV mutations.</p><p><strong>Methods: </strong>Patients diagnosed with FMF according to the Eurofever/PRINTO classification criteria, with heterozygous, homozygous, and compound heterozygous mutations in exon 10 of the Mediterranean fever (MEFV) gene, and with a follow-up of more than 6 months were included in the study.</p><p><strong>Results: </strong>The study included 266 patients (53% female, n = 141). Reported triggers were identified in 189 patients (93.6%), and the most common trigger was fatigue (n = 141; 69.8%). The others were as follows: prolonged standing (49.5%), emotional stress (47%), cold exposure (42.6%), insomnia (36.6%), menstruation (18.5%), high-fat food consumption (15.8%), exercise (15.3%), long-term travel (13.4%), starvation (11.9%), sunlight exposure (5.4%), and physical trauma (2.5%). Self-reported management strategies were used by 89.1% (n = 180) of the patients, primarily nonsteroidal anti-inflammatory drugs (75.2%, n = 152). The others were sleep (50.5%), fluid intake (39.1%), massage (31.2%), hot water compress (30.7%), warm shower (23.3%), fat-free diet (8.4%), and sweet food consumption (5.4%). Long-term travel was found to be a significantly more commonly reported trigger for attacks with arthritis/arthralgia (p = 0.036) and erysipelas-like erythema (p = 0.001).</p><p><strong>Conclusions: </strong>This is the first study focused on reported triggers in childhood FMF. Although our study offers unique findings, the data require validation with clinical and laboratory evidence.</p>","PeriodicalId":520664,"journal":{"name":"Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-07-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144693094","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":"Pulmonary Artery Involvement and Parenchymal Lung Changes in Behçet Disease: A Comparative Cohort Thoracic Computed Tomography Imaging Study.","authors":"Cemal Bes, Rabia Deniz, Sezgi Karabulut Gök, Ceren Tansu Yavuz, Oya Altun, Ferdanur Deniz, Arda Okumuş, Duygu Sevinç Özgür, Gamze Akkuzu, Bilgin Karaalioğlu, Fatih Yıldırım, Serap Baş","doi":"10.1097/RHU.0000000000002264","DOIUrl":"https://doi.org/10.1097/RHU.0000000000002264","url":null,"abstract":"<p><strong>Background/objective: </strong>Pulmonary involvement of Behçet disease (BD) typically manifests as vascular involvement in the form of pulmonary artery thrombosis and/or aneurysm, although various parenchymal lung findings may also be observed. We aimed to analyze the indications for imaging and thoracic computed tomography (TCT) findings in BD patients.</p><p><strong>Methods: </strong>In this medical records review, single-center, comparative cohort study, 196 BD patients who underwent TCT for any reason between July 2020 and July 2024 were included. The patients' demographic data, disease-related clinical features, indications for TCT, and TCT findings were recorded.</p><p><strong>Results: </strong>The mean age of the patients was 40.0 ± 12.0 years, with a female-to-male ratio of 56/140 and disease duration of 8.9 ± 9.1 years. The most common indications for TCT imaging were suspected pulmonary vascular involvement (PVI) (139/196), unexplained acute phase elevation (46/196), and infection (36/196). PVI was present in 23 patients. Patients with PVI also exhibited additional parenchymal findings. Ground-glass opacities and atelectasis were significantly more common in patients with PVI compared with those without.</p><p><strong>Conclusion: </strong>TCT imaging is essential for identifying both vascular and parenchymal pulmonary complications in BD, especially in patients with atypical symptoms or elevated inflammatory markers.</p>","PeriodicalId":520664,"journal":{"name":"Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144602796","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":"Predictors of Persistent Disease Activity and Damage in Systemic Sclerosis: Associations With Mortality in a Single-Center Cohort Study.","authors":"Beatrice Moccaldi, Marco Binda, Salvatore Prete, Andrea Martini, Anna Cuberli, Maria Favaro, Andrea Doria, Elisabetta Zanatta","doi":"10.1097/RHU.0000000000002253","DOIUrl":"https://doi.org/10.1097/RHU.0000000000002253","url":null,"abstract":"<p><strong>Background/objective: </strong>The assessment of \"disease activity\" and \"damage\" has recently been standardized in systemic sclerosis (SSc), with the creation of composite indices. We aimed to identify predictors of persistent disease activity and moderate-severe damage in a monocentric SSc cohort and to evaluate the impact of persistent disease activity and moderate-severe damage on mortality.</p><p><strong>Methods: </strong>Adult SSc patients with a disease duration ≤7 years were enrolled in this cohort study. Disease activity was evaluated by EUSTAR-AI (European Scleroderma Trials and Research Group Activity Index), severity by Medsger Severity Scale and damage by SCTC-DI (Scleroderma Clinical Trials Consortium Damage Index). \"Persistent disease activity\" was defined as EUSTAR-AI ≥2,5 in ≥50% of follow-up visits and moderate-severe damage as SCTC-DI ≥6. Statistical analysis was performed using Jamovi computer software.</p><p><strong>Results: </strong>One hundred one SSc patients (85% females and 33% diffuse cutaneous SSc, with a median disease duration of 2 years; interquartile range, 1-5 years) were enrolled and followed up for a median time of 27 months (interquartile range, 14-48 months). Erythrocyte sedimentation rate (p = 0.004), Medsger Severity Scale (p = 0.044), and SCTC-DI at baseline (p = 0.022) were independent predictors of persistent disease activity. Severe organ involvement-interstitial lung disease, cardiac involvement, and pulmonary arterial hypertension-and diffuse cutaneous SSc were associated with moderate-severe damage at the end of follow-up. Persistent disease activity and moderate-severe damage were associated with poor survival (p = 0.0152 and p < 0.001, respectively).</p><p><strong>Conclusion: </strong>Persistent disease activity and moderate-severe damage are associated with increased mortality in SSc. Early identification of at-risk patients may help improve outcomes.</p>","PeriodicalId":520664,"journal":{"name":"Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-07-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144593777","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":"Isotretinoin-Induced Sacroiliitis: New Clinical Insights and Magnetic Resonance Imaging-Based Outcomes in a Real-Life Clinical Setting.","authors":"Kemal Erol, Ezgi Akyıldız Tezcan","doi":"10.1097/RHU.0000000000002247","DOIUrl":"https://doi.org/10.1097/RHU.0000000000002247","url":null,"abstract":"<p><strong>Background: </strong>Isotretinoin, a frequently prescribed treatment for severe acne vulgaris, is associated with rare but clinically important musculoskeletal adverse effects, notably sacroiliitis. Although previous studies reported an association, comprehensive long-term data on clinical outcomes, imaging progression, and risk of evolution to axial spondyloarthritis (axSpA) remain limited. Basically, previous studies commonly excluded patients with spondyloarthritis-related risk factors such as chronic inflammatory back pain, but this limits the generalization. This study uniquely evaluated the clinical course and progression of isotretinoin-induced sacroiliitis using objective magnetic resonance imaging (MRI) without excluding patients presenting these risk factors.</p><p><strong>Methods: </strong>In this historical cohort study, patients with MRI-confirmed sacroiliitis during isotretinoin treatment were assessed. Clinical data including spondyloarthritis-associated features were recorded. Follow-up MRI performed at least 6 months later assessed inflammation resolution. Patients were classified according to Assessment of SpondyloArthritis International Society axSpA criteria. Statistical analyses were descriptive.</p><p><strong>Results: </strong>Among the 16 patients, 14 underwent follow-up MRI; of these, 2 (14.3%) demonstrated persistent inflammation. Additionally, 1 patient met the Assessment of SpondyloArthritis International Society axSpA criteria clinically without repeat imaging. Overall, 3 patients (18.8%) progressed to axSpA, all with chronic inflammatory back pain, and 1 patient also had psoriasis. Half of the patients initially presenting with inflammatory back pain (3/6) progressed to axSpA during follow-up.</p><p><strong>Conclusion: </strong>Although most isotretinoin-induced sacroiliitis resolves spontaneously, approximately one-fifth of cases may progress to axSpA, especially in patients with inflammatory back pain or other spondyloarthritis features. Clinicians should closely monitor isotretinoin-treated patients developing new or worsening back pain. Longitudinal studies are warranted to establish risk factors and optimize screening strategies.</p>","PeriodicalId":520664,"journal":{"name":"Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-06-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144201317","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 Role of Positron Emission Tomography/Computed Tomography in Diagnosing Osseous Sarcoidosis.","authors":"Jenna Davison, Courtney Arment","doi":"10.1097/RHU.0000000000002200","DOIUrl":"https://doi.org/10.1097/RHU.0000000000002200","url":null,"abstract":"","PeriodicalId":520664,"journal":{"name":"Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases","volume":"31 4","pages":"e29"},"PeriodicalIF":0.0,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144129465","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":"Dysphagia Due to Diffuse Idiopathic Skeletal Hyperostosis.","authors":"Eaman Alhassan,&nbsp;Stratos Christianakis","doi":"10.1097/RHU.0000000000001919","DOIUrl":"https://doi.org/10.1097/RHU.0000000000001919","url":null,"abstract":"","PeriodicalId":520664,"journal":{"name":"Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases","volume":" ","pages":"e707-e708"},"PeriodicalIF":3.4,"publicationDate":"2022-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40719616","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":"Atypical Presentation of Leukocytoclastic Vasculitis With Koebner Phenomenon.","authors":"Neenu Sukumaran,&nbsp;Aryanna Sousa","doi":"10.1097/RHU.0000000000001926","DOIUrl":"https://doi.org/10.1097/RHU.0000000000001926","url":null,"abstract":"","PeriodicalId":520664,"journal":{"name":"Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases","volume":" ","pages":"e705-e706"},"PeriodicalIF":3.4,"publicationDate":"2022-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40719615","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":"Multiple Hereditary Exostoses-Family \"Bone History\" Revealed.","authors":"Susana P Silva,&nbsp;Gisela Eugénio","doi":"10.1097/RHU.0000000000001911","DOIUrl":"https://doi.org/10.1097/RHU.0000000000001911","url":null,"abstract":"","PeriodicalId":520664,"journal":{"name":"Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases","volume":" ","pages":"e697-e698"},"PeriodicalIF":3.4,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40382154","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":"Mycobacterium avium complex Tenosynovitis Mimicking Synovial Chondromatosis.","authors":"Seth J VanDerVeer,&nbsp;Casey Sabbag","doi":"10.1097/RHU.0000000000001898","DOIUrl":"https://doi.org/10.1097/RHU.0000000000001898","url":null,"abstract":"","PeriodicalId":520664,"journal":{"name":"Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases","volume":" ","pages":"e695-e696"},"PeriodicalIF":3.4,"publicationDate":"2022-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40423768","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":"Isolated Myeloperoxidase-Antineutrophil Cytoplasmic Antibody-Associated Hypertrophic Pachymeningitis.","authors":"Jeffrey Maneval,&nbsp;Bronwyn Bryant,&nbsp;Adam Ulano,&nbsp;Waqar Waheed,&nbsp;Alana Nevares","doi":"10.1097/RHU.0000000000001845","DOIUrl":"https://doi.org/10.1097/RHU.0000000000001845","url":null,"abstract":"<p><strong>Abstract: </strong>A 65-year-old man with no pertinent medical history presented with 1 month of progressive holocephalic positional headaches (worse supine), photophobia, progressive gait instability resulting in multiple falls (ambulatory to walker in only 2 months), and weight loss. Testing found positive ANCA 1:160 perinuclear patter, myeloperoxidase >8.0. Cerebrospinal fluid found lymphocytic pleocytosis. We present his neuroimaging of isolated hypertrophic pachymeningitis with clinicoradiographic resolution after immunomodulatory pharmacotherapy along with histology from his meningeal biopsy. Isolated vasculitic myeloperoxidase-antineutrophil cytoplasmic antibody hypertrophic pachymeningitis is quite rare.</p>","PeriodicalId":520664,"journal":{"name":"Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases","volume":" ","pages":"e672-e674"},"PeriodicalIF":3.4,"publicationDate":"2022-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40320282","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}