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

{"title":"Impact of Health Care Financing Models on Biological Agent Accessibility and Clinical Outcomes in Juvenile Idiopathic Arthritis: A Comparative Study From Türkiye and India.","authors":"Batuhan Küçükali, Çisem Yıldız, Jitendra S Oswal, Shyam Mohan, Nuran Belder, Merve Kutlar, Büşra Acun, Khadija Ahmadova, Nihal Karaçayır, Övgü Özenli Yağcı, Deniz Gezgin Yıldırım, Sevcan A Bakkaloğlu","doi":"10.1097/RHU.0000000000002353","DOIUrl":"10.1097/RHU.0000000000002353","url":null,"abstract":"<p><strong>Objective: </strong>Biological treatments have transformed juvenile idiopathic arthritis (JIA) outcomes, yet their availability and cost remain challenging. We investigated the impact of 2 different health care systems on the JIA disease course and outcomes.</p><p><strong>Materials and methods: </strong>JIA patients presenting to 2 tertiary centers in Türkiye and India between January 2014 and January 2025, with ≥1 year of follow-up, were enrolled. Systemic JIA (sJIA) patients were analyzed separately due to differing management and prognosis. Data at 6 months, 1, 2, 3, and 5 years if follow-up occurred, were obtained retrospectively.</p><p><strong>Results: </strong>A total of 207 patients were enrolled: 15 sJIA and 129 non-sJIA from Türkiye, and 12 sJIA and 51 non-sJIA from India. Biological disease-modifying antirheumatic drugs (bDMARDs) were prescribed significantly more often in the Turkish cohort (46% vs. 23%, p =0.001). In contrast, systemic corticosteroid use was significantly higher in the Indian cohort (26% vs. 69%, p =0.001). Initial remission was achieved more rapidly in the Turkish cohort (median month 2 vs. 4, p <0.001), and the number of patients with active joints and elevated acute-phase reactants was consistently higher in the Indian cohort throughout all follow-up periods.</p><p><strong>Conclusions: </strong>Universal health care coverage was associated with early initiation and sustained use of bDMARDs, leading to more rapid control of disease activity. In contrast, when bDMARDs were unavailable, physicians were forced to rely more heavily on systemic corticosteroids. Interestingly, JIA-related complications such as chronic joint involvement and uveitis did not significantly differ between the Turkish and Indian cohorts, possibly due to differences in ethnic and disease characteristics, survivor bias, and the limited number of patients with such complications.</p>","PeriodicalId":520664,"journal":{"name":"Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases","volume":" ","pages":"e230-e238"},"PeriodicalIF":1.8,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147849256","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":"Identifying Comorbid Fibromyalgia in Inflammatory Arthritis: The Role of Disease Activity in Fibromyalgia Survey Questionnaire Performance.","authors":"Milena Mimica, Josefina Durán, Miguel Gutiérrez, Oslando Padilla, Alejandra Segovia, Sebastián Ibáñez, Oscar Neira, Joanny Rey, María José Monsalves, Jocelyn Muñoz, Loreto Massardo","doi":"10.1097/RHU.0000000000002367","DOIUrl":"10.1097/RHU.0000000000002367","url":null,"abstract":"<p><strong>Objective: </strong>To evaluate the utility of the Fibromyalgia Survey Questionnaire (FSQ) as a screening tool for fibromyalgia (FM) in patients with inflammatory arthritis (IA) and to determine optimal cutoff points according to arthritis disease activity.</p><p><strong>Methods: </strong>Patients with rheumatoid arthritis (RA), axial spondyloarthritis (SpA), and psoriatic arthritis (PsA), with and without comorbid fibromyalgia (FM) at a 1:1 ratio were invited to participate in this cross-sectional study. FM status was defined by an expert physician's diagnosis. Disease activity indexes were recorded. Participants completed the FSQ through a structured online interview. ROC analysis and the area under the curve (AUC) were calculated to identify the optimal FSQ cutoff values.</p><p><strong>Results: </strong>A total of 152 IA patients were included (72% RA, 11% SpA, and 17% PsA), 51% of whom had comorbid FM. The ability of the FSQ to differentiate comorbid FM among patients in remission or with low disease activity (n=80) was high (AUC=0.896), showing 88% sensitivity and 88% specificity at a cutoff >12. In contrast, the FSQ performance was lower in patients with moderate to very high disease activity (n=72, AUC=0.695), with 83% sensitivity and 56% specificity at a cutoff >15.</p><p><strong>Conclusions: </strong>The FSQ is a useful screening tool for identifying comorbid FM in IA patients with low disease activity or in remission, with performance comparable to the general population (cutoff value >12). Results should be interpreted cautiously in those with active disease. These findings may facilitate the recognition and management of comorbid FM in IA patients.</p>","PeriodicalId":520664,"journal":{"name":"Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases","volume":" ","pages":"e254-e260"},"PeriodicalIF":1.8,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148166543","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":"In-Hospital Mortality After Coronary Artery Bypass Graft Surgery With and Without Systemic Lupus Erythematosus.","authors":"Jay Manadan, Burak Celik, Ehizogie Edigin","doi":"10.1097/RHU.0000000000002392","DOIUrl":"10.1097/RHU.0000000000002392","url":null,"abstract":"<p><strong>Background/objective: </strong>To evaluate in-hospital mortality after coronary artery bypass graft (CABG) surgery in patients with and without systemic lupus erythematosus (SLE) using a large contemporary nationwide inpatient database.</p><p><strong>Methods: </strong>Using the 2016 to 2022 National Inpatient Sample database, we identified adults undergoing CABG surgery and compared those with and without SLE. Demographics, comorbidities, socioeconomic factors, and perioperative outcomes were analyzed. Multivariable logistic regression was used to assess factors associated with in-hospital mortality.</p><p><strong>Results: </strong>Among 1,342,510 CABG hospitalizations, 4260 (0.3%) involved patients with SLE. Compared with non-SLE patients, those with SLE were younger (median 63 vs. 67; P <0.001), predominantly female (72.9% vs. 24.1%; P <0.001), more likely to be African American (20.8% vs. 6.7%; P <0.001), had higher comorbidity scores (median Charlson Comorbidity Index: 4 vs. 2; P <0.001), longer median hospital LOS (9 vs. 8 d; P <0.001), and higher median total hospital charges ($206,441 vs. $186,257; P <0.001). SLE itself was not independently associated with in-hospital death (adjusted OR: 0.92; 95% CI: 0.635-1.340). Factors independently associated with in-hospital mortality included older age, female sex, concurrent valve surgery, and greater comorbidity burden. Higher-income quartiles and private insurance were protective. Rates of major perioperative complications were generally similar between the groups, although respiratory failure occurred more frequently in SLE patients.</p><p><strong>Conclusions: </strong>After adjusting for demographic and clinical factors, SLE was not independently associated with mortality during CABG surgery hospitalization. These findings suggest that SLE alone may not independently increase in-hospital mortality risk after CABG surgery.</p>","PeriodicalId":520664,"journal":{"name":"Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases","volume":" ","pages":"217-221"},"PeriodicalIF":1.8,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148611996","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":"Clinical Features Distinguishing Tumors From Tumor-Like Lesions in Patients With Rheumatoid Arthritis: An Observational Study.","authors":"Toshifumi Fujiwara, Akira Nabeshima, Makoto Endo, Nobuhiko Yokoyama, Ichiro Kurakazu, Ryosuke Yamaguchi, Yukio Akasaki, Goro Motomura, Yoshinao Oda, Nakashima Yasuharu","doi":"10.1097/RHU.0000000000002347","DOIUrl":"10.1097/RHU.0000000000002347","url":null,"abstract":"<p><strong>Background: </strong>Rheumatoid arthritis (RA)-related lesions, including synovitis and rheumatoid nodules, may mimic musculoskeletal tumors and cause diagnostic uncertainty. This study aimed to characterize clinical features that distinguish true tumors from tumor-like lesions in RA patients referred for suspected bone or soft tissue tumors.</p><p><strong>Methods: </strong>We retrospectively reviewed 70 RA patients evaluated between September 2009 and February 2025 for suspected musculoskeletal tumors. Patients were categorized as (1) newly diagnosed RA with tumor-like lesions (n=27), (2) established RA with tumor-like lesions (n=20), and (3) established RA with true tumors (n=23). Clinical characteristics, MRI findings, and histologic results were assessed. Logistic regression was used to identify factors associated with tumor diagnosis.</p><p><strong>Results: </strong>Patients with true tumors were older (median 69 vs. 55 y, p <0.01) and more likely to have extra-articular lesions (96% vs. 28%, p <0.01). Synovitis-like MRI patterns-low-to-intermediate T1 and heterogeneous high T2 signals-were seen in 83% of tumor-like lesions but in only 43% of true tumors. Histologic confirmation was performed in 56% of all cases (74% of tumors vs. 47% of tumor-like lesions, p =0.03). Multivariate analysis suggested that older age (OR: 1.13, 95% CI: 1.04-1.23) and extra-articular location (OR: 434.86, 95% CI: 9.44-20,035.22) were associated with tumor diagnosis.</p><p><strong>Conclusions: </strong>Among RA patients evaluated for suspected musculoskeletal tumors, older age and extra-articular lesion location were associated with true tumor diagnosis, although the estimates showed wide confidence intervals. MRI features alone were insufficient for reliable differentiation, underscoring the continued need for histologic confirmation to ensure accurate and timely diagnosis.</p>","PeriodicalId":520664,"journal":{"name":"Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases","volume":" ","pages":"e239-e246"},"PeriodicalIF":1.8,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147826061","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":"Incidence of Herpes Zoster in Patients With Rheumatoid Arthritis: Comparison With Controls and Risk According to Different Treatments.","authors":"Erika S Palacios Santillán, María V Chiarvetto, Enrique R Soriano, Javier E Rosa, Marina Scolnik","doi":"10.1097/RHU.0000000000002363","DOIUrl":"10.1097/RHU.0000000000002363","url":null,"abstract":"<p><strong>Objective: </strong>To compare the incidence of herpes zoster (HZ) between patients with rheumatoid arthritis (RA) and matched controls from a health plan in Buenos Aires, and to assess incidence under different immunosuppressive treatments.</p><p><strong>Methods: </strong>Patients with newly diagnosed RA between 2000 and 2023 were matched 1:2 by age and sex with controls. Incidence densities per 100 patient-years (PY) were calculated. Cox regression and Kaplan-Meier analyses were performed to identify factors associated with HZ.</p><p><strong>Results: </strong>A total of 460 RA patients and 920 controls (81.3% women, mean age: 62.2 y) were included, with a median follow-up of 9 years. The RA cohort contributed 4184 PY and controls 9783 PY. Forty-six HZ events occurred in RA and 29 in controls. HZ incidence was 1.08 (95% CI: 0.80-1.47) per 100 PY in RA versus 0.29 (95% CI: 0.19-0.44) in controls [incidence rate ratio (IRR) 3.63; 95% CI: 2.28-5.77; p <0.001]. Compared with conventional DMARDs, biologic therapy increased risk (IRR: 2.48; p =0.023), and JAK inhibitors further elevated it (IRR: 4.47; p =0.001). In multivariate analysis, older age (HR: 1.04; p =0.005) and biologic use (HR: 2.39; p =0.006) were independent predictors. Most HZ cases involved one dermatome; 9.3% were disseminated, and 6.8% required hospitalization. Postherpetic neuralgia was more frequent in RA (42.2% vs. 7.7%; p =0.002).</p><p><strong>Conclusion: </strong>HZ incidence was over 3-fold higher in RA patients, particularly among those treated with biologics and JAK inhibitors.</p>","PeriodicalId":520664,"journal":{"name":"Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases","volume":" ","pages":"e247-e253"},"PeriodicalIF":1.8,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148160310","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":"Nationwide Analysis of Eosinophilic Granulomatosis With Polyangiitis Hospitalizations.","authors":"Anuya Natu, Isadora Small, Augustine M Manadan","doi":"10.1097/RHU.0000000000002358","DOIUrl":"10.1097/RHU.0000000000002358","url":null,"abstract":"<p><strong>Objective: </strong>To characterize adult hospitalizations for eosinophilic granulomatosis with polyangiitis (EGPA) in the United States and identify clinical and sociodemographic factors independently associated with in‑hospital mortality.</p><p><strong>Methods: </strong>We conducted a retrospective analysis of the National Inpatient Sample Database (2016-2022) to identify adult EGPA hospitalizations. Demographics, comorbidities, complications, insurance type, income quartile, length of stay, and total charges were compared between survivors and nonsurvivors. Variables with p ≤ 0.2 in univariable analysis were entered into multivariable logistic regression to determine independent predictors of in‑hospital death.</p><p><strong>Results: </strong>Among 12,900 EGPA hospitalizations, 355 patients died (2.75%). Nonsurvivors were older, had longer hospitalizations, and incurred higher total hospital charges. In multivariable analysis, cardiac disease (OR: 1.94; 95% CI: 1.157-3.237), central nervous system (CNS) involvement (OR: 2.91; 95% CI: 1.004-8.453), gastrointestinal (GI) disease (OR: 3.05; 95% CI: 1.159-8.042), infection (OR: 3.87; 95% CI: 2.123-7.046), interstitial lung disease (OR: 2.62; 95% CI: 1.227-5.598), and renal disease (OR: 5.20; 95% CI: 2.784-9.708) were independently associated with in‑hospital mortality. Demographic and socioeconomic variables, including sex, race/ethnicity, insurance type, and income quartile, were not independent predictors of in-hospital death.</p><p><strong>Conclusions: </strong>In this nationally representative cohort, in‑hospital mortality for EGPA was 2.75%. Renal, infectious, cardiac, CNS, GI, and pulmonary complications were strongly associated with death. These findings underscore the need for early recognition and aggressive management of organ‑threatening disease and infection in hospitalized EGPA patients.</p>","PeriodicalId":520664,"journal":{"name":"Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases","volume":" ","pages":"207-211"},"PeriodicalIF":1.8,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147849222","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":"When Rheumatoid Nodules Break the Rules.","authors":"Despoina Mylona, Styliani Partalidou","doi":"10.1097/RHU.0000000000002379","DOIUrl":"10.1097/RHU.0000000000002379","url":null,"abstract":"","PeriodicalId":520664,"journal":{"name":"Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases","volume":" ","pages":"e278"},"PeriodicalIF":1.8,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148261257","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":"Physical, Psychological, Social Support, Adherence, and Educational Factors Associated With Self-Efficacy in Rheumatoid Arthritis: Scoping Review.","authors":"Carla Moreira Lorentz Higa, Aline Moraes da Silva, Jhoniffer Lucas das Neves Matricardi, Andréia Insabralde de Queiroz Cardoso, Paula Felippe Martinez","doi":"10.1097/RHU.0000000000002377","DOIUrl":"10.1097/RHU.0000000000002377","url":null,"abstract":"<p><strong>Background: </strong>Rheumatoid arthritis is a disease whose treatment requires patient commitment to control disease activity, which is directly related to self-efficacy. The objective of this study was to map physical, psychological, social support, treatment adherence, and educational program variables associated with self-efficacy, as measured by validated RA-specific instruments.</p><p><strong>Methods: </strong>This scoping review followed a comprehensive search of major biomedical databases and gray literature. Inclusion criteria were full-text articles, theses, and dissertations published from 1977 onwards, in English, Spanish, or Portuguese. The search identified 4737 records, of which 43 studies met the criteria and were included in the final sample.</p><p><strong>Results: </strong>The studies originated from 5 continents and were published in English and Spanish, exhibiting heterogeneous characteristics. All used quantitative methods, with the majority being cross-sectional (44.19%, n=19), followed by randomized clinical trials (39.53%, n=17). Most participants were female (76.60%, n=5580), with a mean age of 55.90 years. The results were categorized into 3 areas: positive, negative, and nonsignificant associations. Treatment adherence, positive psychological factors, and social support were positively associated with self-efficacy, whereas physical factors and negative psychological factors showed negative associations. In the education and self-management group, 10 educational and self-management programs showed a positive association with self-efficacy. In contrast, 4 other programs showed no significant association.</p><p><strong>Conclusions: </strong>Although the results do not fully reflect causal relationships, they suggest the importance of addressing the psychological, physical, treatment adherence, and social support factors in individuals with rheumatoid arthritis, as well as promoting educational and self-management measures to increase self-efficacy.</p>","PeriodicalId":520664,"journal":{"name":"Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases","volume":" ","pages":"e271-e277"},"PeriodicalIF":1.8,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148335434","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":"Adherence to Biologics and Psycho-cognitive Factors in Axial Spondyloarthritis: A Multicentric Cross-sectional Study.","authors":"Maxime Vandersmissen, Charlotte Amory, Jessika Bernard, Jerome Despres, Thomas Barnetche, Justine Landrin, Marie-Elise Truchetet, Cédric Lukas, Cécile Gaujoux Viala, Adeline Ruyssen-Witrand, Anne Tournadre, Pascale Vergne-Salle","doi":"10.1097/RHU.0000000000002395","DOIUrl":"10.1097/RHU.0000000000002395","url":null,"abstract":"<p><strong>Background/objective: </strong>Adherence to treatment in axial spondyloarthritis (axSpA) remains poorly documented, with estimates ranging from 28.2% to 70.6%. Psychological factors may influence adherence. This study aimed to assess adherence to biological disease-modifying antirheumatic drugs (bDMARDs) and evaluate the impact of catastrophizing, fibromyalgia, anxiety, and depression on adherence.</p><p><strong>Methods: </strong>We conducted a 1-year multicenter cross-sectional study, including consecutive axSpA patients fulfilling ASAS criteria. Participants completed a self-administered questionnaire covering demographic data, disease characteristics, and activity scores. Adherence was assessed using the Girerd scale (GS); psychological assessments included the Pain Catastrophizing Scale (PCS), Fibromyalgia Rapid Screening Tool (FIRST), and Hospital Anxiety and Depression Scale (HADS).</p><p><strong>Results: </strong>Among 500 patients, 49.2% showed good adherence (GS=0). Prevalence of catastrophizing, fibromyalgia, anxiety, and depression was 17.2%, 21.4%, 25.6%, and 11.0%, respectively. Good adherence was significantly associated with older age (mean 53.3 vs. 45.9 y; p <0.001), retirement status (26.4% vs. 12.6%; p <0.001), and lower educational level (OR: 1.84; 95% CI: 1.23-2.75; p =0.003). Poor adherence was linked to anxiety (OR: 1.53; 95% CI: 1.02-2.30; p =0.041) and catastrophizing (OR: 1.61; 95% CI: 1.00-2.58; P =0.049). In multivariate analysis, younger age (OR: 0.96; 95% CI: 0.95-0.98; p <0.001) and anxiety (OR 1.72; 95% CI: 1.05-2.81; p =0.031) remained significantly associated with poor adherence.</p><p><strong>Conclusions: </strong>About half of axSpA patients had suboptimal adherence to bDMARDs. Younger age, anxiety, and catastrophizing were associated with poor adherence and should be considered in patient management.</p>","PeriodicalId":520664,"journal":{"name":"Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases","volume":" ","pages":"201-206"},"PeriodicalIF":1.8,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148715210","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":"Outcome of Children With Systemic Rheumatologic and Autoinflammatory Diseases Admitted to a Pediatric Intensive Care Unit: A Retrospective Cohort Study With Development of a Prognostic Risk-stratification Tool.","authors":"Ivonne Portaccio, Tony Christian Morena, Enzo Picconi, Federica Tosi, Donato Rigante, Elisabetta Cortis, Giulia Lais, Giorgia Spinazzola, Giorgio Attinà, Gabriella De Rosa, Daniele De Luca, Giorgio Conti, Marco Piastra","doi":"10.1097/RHU.0000000000002394","DOIUrl":"10.1097/RHU.0000000000002394","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Objective: &lt;/strong&gt;Children with systemic rheumatologic diseases (SRD) and autoinflammatory diseases can experience life-threatening deterioration requiring intensive care. We conducted a retrospective cohort study with 2 complementary components: (1) a descriptive analysis of clinical presentations and outcomes; and (2) an exploratory derivation of a bedside risk-stratification tool, intended as a candidate severity overlay on established pediatric intensive care unit (PICU) scoring systems rather than as a fully validated general-purpose prognostic model.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Design: &lt;/strong&gt;Retrospective observational cohort study with exploratory prognostic model development. Propensity-score matching was used as a secondary, descriptive analysis to contextualize outcomes against comparable populations and is not intended to support causal inference.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Setting: &lt;/strong&gt;Single academic pediatric intensive care unit (January 2005 to December 2015).&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Patients: &lt;/strong&gt;Forty children with confirmed SRD requiring PICU admission (first admission per patient) and propensity-matched controls (2:1 ratio).&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Measurements and main results: &lt;/strong&gt;Nearly two-thirds (62.2%) of patients experienced their first disease manifestation as a life-threatening crisis. Hemophagocytic lymphohistiocytosis/macrophage activation syndrome (HLH/MAS) was associated with the majority of deaths, accounting for 83.3% of fatalities despite representing only 20% of admissions (disease-specific mortality 62.5% vs. 6.3% for other diagnoses combined, p &lt;0.001). Backward stepwise logistic regression identified 3 predictors that were combined, with equal weighting, into the exploratory PHV Score (PRISM-HLH-Vasoactive): Pediatric Risk of Mortality III (PRISM-III) at 24 hours &gt;20, HLH/MAS diagnosis, and requirement for ≥2 vasoactive agents. In this derivation sample the score showed encouraging discrimination [apparent area under the curve (AUC) 0.91, 95% CI 0.83-0.99; optimism-corrected AUC 0.88]; however, given the very limited event count (n=6 deaths), wide CIs, and the fact that the PHV Score partially incorporates PRISM-III, head-to-head AUC comparisons with PRISM-III (AUC 0.72), PELOD-2 (Pediatric Logistic Organ Dysfunction-2; AUC 0.68), and pediatric SOFA (Sequential Organ Failure Assessment; AUC 0.64) should be interpreted with caution and regarded as hypothesis-generating. At the optimal cutoff of ≥2 (Youden index), sensitivity was 83.3% and specificity 91.2%; predictive values are prevalence-dependent and may not generalize to centers with different case mixes.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusions: &lt;/strong&gt;In this retrospective single-center cohort, HLH/MAS was associated with most PICU deaths among children with systemic rheumatologic and autoinflammatory diseases. The PHV Score is an exploratory, candidate bedside tool that appears to refine risk stratification in our cohort but requires prospective multicenter external validation.&lt;/p","PeriodicalId":520664,"journal":{"name":"Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases","volume":" ","pages":"e261-e270"},"PeriodicalIF":1.8,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148650449","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}