Saeyun Lee, Sujin Kim, Suzanne Segerstrom, Polly J Ferguson, Aleksander Lenert
{"title":"Accuracy and Performance Characteristics of Administrative Codes for the Diagnosis of Autoinflammatory Syndromes: A Discovery and Validation Study.","authors":"Saeyun Lee, Sujin Kim, Suzanne Segerstrom, Polly J Ferguson, Aleksander Lenert","doi":"10.1097/RHU.0000000000002172","DOIUrl":"10.1097/RHU.0000000000002172","url":null,"abstract":"<p><strong>Objective: </strong>The aim of this study was to evaluate and validate the accuracy and performance characteristics of administrative codes in diagnosing autoinflammatory syndromes (AISs).</p><p><strong>Methods: </strong>We identified potential AIS patients from the electronic medical records at the University of Iowa Hospital and Clinics and the Stead Family Children's Hospital using a screening filter based on the 10th edition of the International Classification of Diseases ( ICD-10 ) codes and interleukin-1 antagonists. Diagnostic criteria for adult-onset Still disease, systemic juvenile idiopathic arthritis, Behçet disease (BD), familial Mediterranean fever (FMF), cryopyrin-associated periodic syndrome (CAPS), and SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome and chronic nonbacterial osteomyelitis (SAPHO-CNO) were reviewed for each patient. Patients who did not meet the diagnostic criteria were categorized as non-AIS. In this cross-sectional study, we calculated the sensitivity, specificity, positive predictive value, negative predictive value, and area under the receiver operating characteristic curve for the ICD codes in diagnosing AIS.</p><p><strong>Results: </strong>Out of the 502 patients with potential AIS, 338 patients (67%) had a true AIS diagnosis. Sensitivity ranged from 80% (SAPHO-CNO) to 100% (BD and FMF), and positive predictive value ranged from 15% (FMF) to 80% (SAPHO-CNO). Specificity ranged from 81% (FMF) to 99% (CAPS and SAPHO-CNO), whereas negative predictive value ranged from 98% (adult-onset Still disease) to 100% (systemic juvenile idiopathic arthritis, BD, FMF, and CAPS). All ICD codes or code combinations for the diagnosis of specific AIS subtypes showed high accuracy with areas under the receiver operating characteristic curve ≥0.89.</p><p><strong>Conclusions: </strong>This study validated the accuracy of administrative codes for diagnosing AIS, supporting their use in constructing AIS cohorts for clinical outcomes research.</p>","PeriodicalId":14745,"journal":{"name":"JCR: Journal of Clinical Rheumatology","volume":" ","pages":"60-64"},"PeriodicalIF":2.4,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12109933/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143005526","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Victor R Pimentel-Quiroz, Rocío V Gamboa-Cárdenas, Zoila Rodríguez-Bellido, Risto Perich-Campos, Graciela S Alarcón, Manuel F Ugarte-Gil
{"title":"Validation of the ANCA-Associated Vasculitis Patient-Reported Outcomes Questionnaire in a Latin American Vasculitis Cohort.","authors":"Victor R Pimentel-Quiroz, Rocío V Gamboa-Cárdenas, Zoila Rodríguez-Bellido, Risto Perich-Campos, Graciela S Alarcón, Manuel F Ugarte-Gil","doi":"10.1097/RHU.0000000000002217","DOIUrl":"https://doi.org/10.1097/RHU.0000000000002217","url":null,"abstract":"<p><strong>Background/objectives: </strong>The aim of this work is to validate the ANCA-associated vasculitis patient-reported outcome (AAV-PRO) questionnaire in a Latin American (Peru) AAV cohort.</p><p><strong>Methods: </strong>We included patients from the Almenara vasculitis cohort who had at least 1 visit between December 2022 and June 2024. Sociodemographic features, disease activity (Birmingham Vasculitis Activity Score version 3 [BVASv3] score), damage (Vasculitis Damage Index [VDI] score), the AAV-PRO (Spanish version), and the Short Form 36 (SF-36) were obtained. The AAV-PRO includes 6 domains (organ-specific symptoms, systemic symptoms, treatment side effects, social and emotional impact, concerns about the future, and physical function); the score ranges from 0 to 100: the higher the value, the worse the health-related quality of life. Correlations between domains of the AAV-PRO and clinical and sociodemographic variables were evaluated using Spearman correlation.</p><p><strong>Results: </strong>Eighty-two patients were included; 60 (73.2%) of them were female. Their age and disease duration were 55.3 (14.3) and 5.7 (5.2) years, respectively. The BVASv3 and the VDI scores were 6.1 (9.0) and 2.4 (1.7), respectively. Overall, every domain of the AAV-PRO correlated strongly and inversely with the global scores of the SF-36 (physical component summary and mental component summary) (all r < -0.4 and p < 0.001). Physical function, role physical, role emotional, and physical component summary correlated inversely with the BVASv3, whereas the organ-specific symptoms score correlated positively with the VDI.</p><p><strong>Conclusions: </strong>The Spanish version of the AAV-PRO questionnaire correlated with the SF-36 in AAV patients from a Peruvian cohort. These findings may support the use of this instrument in other Latin American populations.</p>","PeriodicalId":14745,"journal":{"name":"JCR: Journal of Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2025-02-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143501394","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 Curmudgeon Rheumatologist Looks at \"Burnout\".","authors":"Robert W Ike","doi":"10.1097/RHU.0000000000002203","DOIUrl":"10.1097/RHU.0000000000002203","url":null,"abstract":"","PeriodicalId":14745,"journal":{"name":"JCR: Journal of Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2025-02-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143492074","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}
Shay Brikman, Hani Tannous, Irina Novofastovski, Ran Abuhasira, Reuven Mader, Amir Bieber
{"title":"Association Between Vascular Calcifications on Joint Radiographs and Calcium Pyrophosphate Crystal Arthritis: A Medical Records Review Study.","authors":"Shay Brikman, Hani Tannous, Irina Novofastovski, Ran Abuhasira, Reuven Mader, Amir Bieber","doi":"10.1097/RHU.0000000000002214","DOIUrl":"https://doi.org/10.1097/RHU.0000000000002214","url":null,"abstract":"<p><strong>Objective: </strong>Previous studies have shown an association between chondrocalcinosis (CC) and vascular calcifications (VCs). This study aimed to investigate the association of VCs detected on joint radiographs (XRs) of older patients diagnosed with calcium pyrophosphate (CPP) arthritis compared with a control group with osteoarthritis (OA).</p><p><strong>Methods: </strong>A medical records review study of joint radiographs (knee and wrist) was conducted. CPP crystal arthritis was diagnosed based on at least 1 documented episode of arthritis with synovial fluid analysis positive for CPP crystals or imaging showing CC at 1 or more sites, with no alternative inflammatory arthritis diagnosis. The control group comprised patients with OA and no CC, matched 1:1 for age and sex. All participants were over 60 years of age. XRs were reviewed for CC, OA, and VCs at the affected joint by 2 independent observers.</p><p><strong>Results: </strong>A total of 98 patients were enrolled in both the CPP arthritis group and the OA group. VCs adjacent to the affected joint were detected in 69 patients of the CPP group and 19 patients of the control group (70.4% vs 19.4%, p < 0.001). Among patients aged 60 to 80 years, the presence of VCs on XRs was highly indicative of CPP, demonstrating a specificity of 89.2% (95% confidence interval: 79.1%-95.6%). In the CPP group, patients with VCs had a significantly higher prevalence of cardiovascular (CV) comorbidities.</p><p><strong>Conclusions: </strong>The detection of VCs on XRs was strongly associated with CPP crystal arthritis. The presence of VCs may further serve as a biomarker for an increased burden of CV comorbidities.</p>","PeriodicalId":14745,"journal":{"name":"JCR: Journal of Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2025-02-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143492077","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":"Pneumomediastinum and Subcutaneous Emphysema in a Patient with Dermatomyositis Dermatomyositis.","authors":"Hirotaka Yamamoto, Yoshinori Taniguchi","doi":"10.1097/RHU.0000000000002218","DOIUrl":"https://doi.org/10.1097/RHU.0000000000002218","url":null,"abstract":"","PeriodicalId":14745,"journal":{"name":"JCR: Journal of Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2025-02-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143492086","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 Strengths-Based, Positive Approaches Within Rheumatology Fellowship Training: A Realist Evaluation of Appreciative Inquiry-Based Interventions.","authors":"Bharat Kumar, Alick Feng, Patricia Bruffey Thoene, Erica Sigwarth, Kristina Cobb, Melissa Swee, Manish Suneja","doi":"10.1097/RHU.0000000000002212","DOIUrl":"https://doi.org/10.1097/RHU.0000000000002212","url":null,"abstract":"<p><strong>Objective: </strong>Appreciative Inquiry (AIn) is a strengths-based organizational framework to promote engagement and change. It has shown promise in graduate medical education settings, but how, why, and for whom AIn may drive educational outcomes is underexplored. This realist evaluation examines the causal relationships between contexts, mechanisms, and outcomes at a rheumatology fellowship program in a large tertiary care center that implemented a set of AIn-based interventions. We generate recommendations for leaders in rheumatology fellowship programs on the implementation of AIn-based interventions.</p><p><strong>Methods: </strong>The realist evaluation was conducted in 3 phases. In phase 1, a scoping review informed the initial program theory. In phase 2, realist interviews were conducted to identify and refine causal relationships between contexts, mechanisms, and outcomes, yielding a final program theory. In phase 3, the final program theory was utilized to generate recommendations for implementation.</p><p><strong>Results: </strong>The final program theory identified 15 contexts, 10 mechanisms, and 10 outcomes along with 43 context-mechanism-outcome configurations. Through analysis of the final program theory, 3 recommendations were generated: (1) programs must first create permission structures for critical self-reflection through strengths-based feedback, (2) programs must consistently and synergistically apply AIn principles at multiple levels, and (3) programs can sustain AIn-based interventions through the deliberate co-design of virtuous cycles.</p><p><strong>Conclusions: </strong>This realist evaluation has generated a theory on how AIn may be implemented into rheumatology fellowship programs to drive educational outcomes. Because of the intricate causal relationships, leaders are well-advised to tailor AIn-based interventions based on the context of their training programs.</p>","PeriodicalId":14745,"journal":{"name":"JCR: Journal of Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2025-02-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143449114","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":"Skin Lesions in Acute Hemorrhagic Edema of Infancy.","authors":"Lucy Emery, Derek Ross Soled","doi":"10.1097/RHU.0000000000002215","DOIUrl":"https://doi.org/10.1097/RHU.0000000000002215","url":null,"abstract":"","PeriodicalId":14745,"journal":{"name":"JCR: Journal of Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2025-02-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143440975","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}
Pasoon Hellamand, Marleen G H van de Sande, Rianne E van Bentum, Frank D Verbraak, Jos W R Twisk, Irene van der Horst Bruinsma
{"title":"Early Axial Spondyloarthritis Detection: Two-Year Follow-up of the Sp-EYE Study on Acute Anterior Uveitis and Chronic Back Pain Screening.","authors":"Pasoon Hellamand, Marleen G H van de Sande, Rianne E van Bentum, Frank D Verbraak, Jos W R Twisk, Irene van der Horst Bruinsma","doi":"10.1097/RHU.0000000000002202","DOIUrl":"https://doi.org/10.1097/RHU.0000000000002202","url":null,"abstract":"<p><strong>Objective: </strong>In the previous Spondyloarthritis EYE study, we confirmed the potential of a screening strategy for early axial spondyloarthritis (axSpA) detection using acute anterior uveitis (AAU) and chronic back pain (CBP) as referral criteria. This follow-up study assessed changes in diagnostic categories (definite, suspected, and no axSpA) over 2 years and identified baseline factors predicting axSpA diagnosis at 24 months.</p><p><strong>Methods: </strong>Patients with AAU and CBP were categorized into 3 groups: definite axSpA, suspected of axSpA, and no axSpA, based on clinical and radiographic data within 6 months after baseline. Suspected cases were monitored for 24 months, with the possibility of reclassification. A competing risk analysis was used to estimate the probability of transitioning from \"suspected of axSpA\" to \"definite axSpA\" or \"no axSpA,\" and logistic regression analysis was employed to determine if baseline factors could predict definite axSpA at 24 months.</p><p><strong>Results: </strong>Among 81 patients, 26 were classified as no axSpA, 36 as suspected of axSpA, and 19 as definite axSpA. At 24 months, suspected patients had an 18% probability to transition to definite axSpA (4 cases) and a 60% to no axSpA (15 cases). Significant predictors of axSpA diagnosis included the following: HLA-B27 positivity, good response to nonsteroidal anti-inflammatory drugs, inflammatory back pain, increasing C-reactive protein levels, buttock pain, and higher Bath Ankylosing Spondylitis Metrology Index scores.</p><p><strong>Conclusions: </strong>Our screening strategy identified approximately one third of previously undiagnosed axSpA cases among patients with AAU and CBP, mostly at baseline, with few additional cases at follow-up. The predictors revealed in this study could aid physicians in estimating axSpA disease probability.</p>","PeriodicalId":14745,"journal":{"name":"JCR: Journal of Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2025-02-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143414206","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":"Clinical Experience with TNF Inhibition and Longitudinal Image Monitoring in Osseous Sarcoidosis.","authors":"Seth J VanDerVeer, Erica M Hill","doi":"10.1097/RHU.0000000000002190","DOIUrl":"https://doi.org/10.1097/RHU.0000000000002190","url":null,"abstract":"<p><strong>Background: </strong>In this case series, we present longitudinal imaging surveillance of 6 cases of osseous sarcoidosis, each of which was effectively treated with tumor necrosis factor (TNF) inhibition.</p><p><strong>Methods: </strong>We identified 6 patients from Brooke Army Medical Center with osseous sarcoidosis, who were treated with TNF inhibition and followed with longitudinal imaging studies. Cases of osseous sarcoidosis were defined as having pathologic evidence of noncaseating granulomas on bone biopsy and evidence of osseous lesions on imaging attributable to sarcoidosis by the radiologist, treating clinician, and reviewer. Clinical data were obtained through review of the military electronic medical record.</p><p><strong>Results: </strong>Longitudinal imaging with positron emission tomography/computed tomography, magnetic resonance imaging, and bone scintigraphy assisted in the identification of active disease and clinical remission. Imaging progression of asymptomatic lesions was associated with the eventual development of bone pain 1 to 3 years later. Clinical remission was achieved in all six cases of osseous sarcoidosis and effective doses for TNF inhibition were adalimumab 40 mg subcutaneously every 1 to 2 weeks and infliximab 5 mg/kg every 6 to 8 weeks. Time to complete imaging response ranged from 3 to 8 months.</p><p><strong>Conclusions: </strong>Longitudinal imaging with bone scintigraphy, positron emission tomography/computed tomography, and magnetic resonance imaging demonstrated several benefits including evaluation for occult disease, surveillance of asymptomatic lesions, and evaluation of treatment response. TNF inhibition with adalimumab or infliximab was successful in all cases, and complete resolution of osseous lesions was demonstrated in 5 of 6 patients. Discontinuation of TNF inhibition led to disease recurrence in 2 cases, which prompted the use of long-term immunosuppressive therapy in all treated patients.</p>","PeriodicalId":14745,"journal":{"name":"JCR: Journal of Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2025-01-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143005580","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}
Lilian Otalora Rojas, Karishma Ramsubeik, Luis Sanchez-Ramos, Shastri Motilal, Jasvinder A Singh, Gurjit S Kaeley
{"title":"Rituximab Treatment in Adult Patients With Idiopathic Inflammatory Myositis: A Systematic Review and Meta-analysis.","authors":"Lilian Otalora Rojas, Karishma Ramsubeik, Luis Sanchez-Ramos, Shastri Motilal, Jasvinder A Singh, Gurjit S Kaeley","doi":"10.1097/RHU.0000000000002151","DOIUrl":"10.1097/RHU.0000000000002151","url":null,"abstract":"<p><strong>Objective: </strong>This systematic review and meta-analysis assess the efficacy and safety of rituximab (RTX) in treating idiopathic inflammatory myositis (IIM).</p><p><strong>Methods: </strong>PubMed and Embase were systematically searched for trials and observational studies involving RTX use in IIM. Data were analyzed using a random-effects model to generate pooled estimates for overall response, complete remission, partial response, and adverse events, with subgroup analyses by myositis type and RTX dosage (PROSPERO registered number CRD42022353740). Risk of bias assessments were done using the Newcastle-Ottawa Scale for observational studies and risk of bias 1 tool for trials.</p><p><strong>Results: </strong>Seventeen studies (1 randomized controlled trial and 16 observational studies), encompassing 362 patients, were included. The overall pooled response rate was 70% (95% confidence interval [CI]: 57%-82%; I2 = 74%, p < 0.001). Complete remission occurred in 13% (95% CI: 3%-25%; I2 = 79%, p < 0.001) and partial response in 48% (95% CI: 30%-67%; I2 = 87%, p < 0.001), both with significant heterogeneity. Subgroup analysis revealed high response rates across all myositis types: polymyositis 69%, dermatomyositis 67%, antisynthetase syndrome 70%, juvenile dermatomyositis 60%, and immune-mediated necrotizing myopathy 86%. Response rates were similar between RTX induction doses of 1 g IV on days 0 and 14 (68%) and 375 mg/m 2 weekly for 4 weeks (71%). Reported adverse events totaled 120, including infusion reactions (18.5%) and infections (12.4%).</p><p><strong>Conclusions: </strong>RTX shows a favorable clinical response in IIM treatment, though response rates vary. There was a significant heterogeneity in treatment effect estimates that are based on a small number of patients. The incidence of infusion reactions and infections highlights the need for careful monitoring. Further controlled trials are essential to refine treatment protocols and evaluate long-term outcomes for RTX's role in IIM.</p>","PeriodicalId":14745,"journal":{"name":"JCR: Journal of Clinical Rheumatology","volume":" ","pages":"33-39"},"PeriodicalIF":2.4,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142620688","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}