{"title":"Abstracts of the 35th Meeting of the German Society for Pediatric and Adolescents Rheumatology (Gesellschaft für Kinder- und Jugendrheumatology (GKJR).","authors":"","doi":"10.1186/s12969-025-01055-w","DOIUrl":"10.1186/s12969-025-01055-w","url":null,"abstract":"","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 Suppl 1","pages":"28"},"PeriodicalIF":2.8,"publicationDate":"2025-03-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11912620/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143651911","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"T-ing up the storm: pathogenic cycling lymphocytes in the biology of macrophage activation syndrome.","authors":"Michael T Lam, Connie L Jiang, Pui Y Lee","doi":"10.1186/s12969-025-01081-8","DOIUrl":"10.1186/s12969-025-01081-8","url":null,"abstract":"<p><strong>Background: </strong>Hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) are potentially fatal cytokine storm syndromes with clinical features including fever, pancytopenia, hepatosplenomegaly, coagulopathy, and progressive multiorgan system dysfunction. Mechanistically, HLH / MAS are driven by persistent activation of lymphoid and myeloid cells, but our understanding of the pathogenic cell populations remains incomplete.</p><p><strong>Main body: </strong>In this Perspectives article, we provide an overview of the biology of HLH / MAS and the critical role of interferon-g in disease pathogenesis. We discuss the recent discovery of cycling lymphocytes in HLH / MAS marked by expression of CD38 and HLA-DR, which are primary producers of IFN-γ. The expansion of cycling lymphocytes correlates with disease activity and helps to distinguish HLH / MAS from clinical mimics. We demonstrate an approach to quantify CD38<sup>+</sup>HLA-DR<sup>+</sup> cycling lymphocytes and evaluate their utility as a diagnostic biomarker for HLH / MAS. Lastly, we discuss the treatment of MAS, including potential therapeutic options to target these pathogenic lymphocytes.</p><p><strong>Conclusion: </strong>Understanding of biology of cycling lymphocytes in HLH / MAS will facilitate the development of novel therapeutic approaches to overcome these fatal hyperinflammatory disorders.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"29"},"PeriodicalIF":2.8,"publicationDate":"2025-03-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11912701/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143651909","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Mohammed Hassan Abu-Zaid, Angela Nyangore Migowa, Hanna Lishan Kassa, Wassila Messadi, Yassmine Taha, Yaninga Halwani Fuseini, Madeleine Ngandeu, Yasser El Miedany, Michael Hofer, Wafa Hamdi, Temesgen Teferi Libe, Ali Sobh, Waleed Hassan, Yasmine Makhlouf, Ayodele Faleye, Soad Hashed, Samah Ismail Nasef, Chafia Dahou Makhloufi, Elisa Palalane, Hanene Lassoued Ferjani, Ahmed Seri, Doaa Mosad Mosa, Ourida Gacem, Francis Fredrick Furia, Samy Slimani, Christiaan Scott, Djohra Hadef
{"title":"African guidelines for diagnosis and management of polyarticular juvenile idiopathic arthritis: PAFLAR initiative.","authors":"Mohammed Hassan Abu-Zaid, Angela Nyangore Migowa, Hanna Lishan Kassa, Wassila Messadi, Yassmine Taha, Yaninga Halwani Fuseini, Madeleine Ngandeu, Yasser El Miedany, Michael Hofer, Wafa Hamdi, Temesgen Teferi Libe, Ali Sobh, Waleed Hassan, Yasmine Makhlouf, Ayodele Faleye, Soad Hashed, Samah Ismail Nasef, Chafia Dahou Makhloufi, Elisa Palalane, Hanene Lassoued Ferjani, Ahmed Seri, Doaa Mosad Mosa, Ourida Gacem, Francis Fredrick Furia, Samy Slimani, Christiaan Scott, Djohra Hadef","doi":"10.1186/s12969-025-01076-5","DOIUrl":"10.1186/s12969-025-01076-5","url":null,"abstract":"<p><strong>Background: </strong>Juvenile idiopathic arthritis (JIA) is the most common rheumatologic disease of childhood. The Existing guidelines for polyarticular JIA are typically based on data from non-African populations and may not fully address the unique challenges faced in African settings. We aimed to produce updated African guidelines for the diagnosis and treatment of children and adolescents with polyarticular juvenile idiopathic arthritis (poly-JIA).</p><p><strong>Methods: </strong>This study was conducted with the aim of reaching a consensus among African experts on the diagnosis and treatment of poly-JIA using the Delphi technique. The first scientific committee identified a total of 15 key clinical questions according to the PICO (Patient/Population, Intervention, Comparison, Outcome) approach. A systematic review of the evidence-based literature was conducted for this work. The core steering group identified researchers and clinicians with expertise in pediatric rheumatology. A Delphi process was used to reach consensus.</p><p><strong>Results: </strong>An online questionnaire was sent to the expert panel that participated in the survey (100% response rate). A total of 15 recommendation points were identified, divided into two parts: five recommendations for diagnosis and ten recommendations for management. The percentage of those who agreed with the recommendations (fourth and fifth place) ranged from 80 to 100%. All 15 clinical recommendation statements that the scientific committee had identified had been agreed upon in wording (i.e., 75% of respondents agreed or strongly agreed).</p><p><strong>Conclusions: </strong>We successfully developed guidelines for children with polyarticular JIA, taking into consideration the African specific nature of limited resources and low income, also on the same time incorporating newly released data and using a treat to target approach.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"27"},"PeriodicalIF":2.8,"publicationDate":"2025-03-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11909873/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143634965","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ahmed E Abdulgalil, Noha H Elnagdy, Nehal M Ramadan, Eman Hamza, Ayman Hammad, Mai S Korkor, Atef Elmougy, Ali Sobh, Marwa H Elnagdy
{"title":"Mycophenolic acid trough level assessment in patients with lupus nephritis; does it make a difference?","authors":"Ahmed E Abdulgalil, Noha H Elnagdy, Nehal M Ramadan, Eman Hamza, Ayman Hammad, Mai S Korkor, Atef Elmougy, Ali Sobh, Marwa H Elnagdy","doi":"10.1186/s12969-025-01074-7","DOIUrl":"10.1186/s12969-025-01074-7","url":null,"abstract":"<p><strong>Introduction: </strong>Mycophenolate Mofetil (MMF) has become one of the cornerstone treatments of lupus nephritis (LN). It is converted into mycophenolic acid (MPA), an active metabolite, that displays high inter- and intra-individual pharmacokinetic variability. However, the routine monitoring of MPA trough level is still debatable.</p><p><strong>Objectives: </strong>The present study aims to evaluate the relationship between MPA trough levels and both clinical outcomes and drug-related adverse effects during the maintenance phase of LN in Egyptian patients.</p><p><strong>Methods: </strong>We included thirty-five adults and twenty-nine children with biopsy-proven class III and IV LN, who had been maintained on steroid and MMF as maintenance therapy for more than six months. Clinical and laboratory markers of lupus activity as well as MMF adverse events were reported. MPA trough levels were measured by High-Performance Liquid Chromatography (HPLC).</p><p><strong>Results: </strong>There was a significant association between low MPA trough levels and both flares and SLEDAI scores in the adult group (P = 0.027 and 0.019, respectively). Moreover, high MPA trough levels were associated with higher risk of gastritis in the same age group (P = 0.007). There was no significant association with any of the parameters studied in the pediatric group. Gastritis was the most frequent side effect in both age groups.</p><p><strong>Conclusion: </strong>MPA trough levels correlated with disease activity and gastritis in adult LN patients, and this may help to optimize MMF dosage in these patients. However, MPA concentration-effect relationships were not observed in pediatric patients.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"26"},"PeriodicalIF":2.8,"publicationDate":"2025-03-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11905544/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143617795","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Cyclophosphamide treatment with a comparison in both pediatric rheumatology and pediatric nephrology practices.","authors":"Deniz Gezgin Yildirim, Emine Yılmaz Orulluoglu, Cisem Yildiz, Ceyhun Acari, Hatice Adiguzel Dundar, Okan Akaci, Nurver Akinci, Emil Aliyev, Bedriye Nuray Alpman, Ozge Altug Gucenmez, Elif Arslanoglu Aydin, Bahriye Atmis, Pinar Ozge Avar Aydin, Fatma Aydin, Ozge Baba, Esra Baglan, Ilknur Bagrul, Kenan Barut, Ozge Basaran, Umut Selda Bayrakci, Nuran Belder, Burcu Bozkaya Yucel, Bahar Buyukkaragoz, Sengul Caglayan, Mustafa Cakan, Elif Celikel, Ferhat Demir, Selcan Demir, Yasemin Demir Yigit, Fatma Gul Demirkan, Nida Dincel, Seyda Dogantan, Zahide Ekici Tekin, Esra Genc, Fatih Haslak, Rana Isguder, Aslihan Kara, Muserref Kasap Cuceoglu, Ummusen Kaya Akca, Hakan Kisaoglu, Rabia Miray Kisla Ekinci, Zehra Kızıldag, Tuba Kurt, Batuhan Kucukali, Emre Leventoglu, Hulya Nalcacioglu, Gulcin Otar Yener, Semanur Ozdel, Yesim Ozdemir Atikel, Sumeyra Ozdemir Cicek, Sule Pektas Leblebiciler, Erkin Serdaroglu, Hafize Emine Sonmez, Emine Nur Sunar Yayla, Serra Surmeli Doven, Sezgin Sahin, Seher Sener, Ayse Tanatar, Merve Tanidir, Sema Nur Taskin, Betul Tiryaki, Serife Tuncez, Serkan Turkucar, Bahriye Uzun Kenan, Nurdan Yildiz, Kenan Yilmaz, Yilmaz Tabel, Ismail Dursun, Nur Canpolat, Sevgi Mir, Harun Peru, Rezan Topaloglu, Metin Kaya Gurgoze, Ayse Balat, Yelda Bilginer, Banu Celikel Acar, Betul Sozeri, Erbil Unsal, Ozgür Kasapcopur, Sevcan A Bakkaloglu","doi":"10.1186/s12969-025-01080-9","DOIUrl":"10.1186/s12969-025-01080-9","url":null,"abstract":"<p><strong>Background: </strong>Cyclophosphamide (CYC) is an inactive alkylating agent that transforms the alkyl radicals into other molecules and is used in combination with systemic corticosteroids in the treatment of many childhood rheumatic diseases, such as systemic lupus erythematosus (SLE), and ANCA-associated vasculitis (AAV). In recent years, rituximab (RTX), a B-cell-targeting anti-CD20 monoclonal antibody, has emerged as a new alternative treatment modality over CYC for induction therapy of childhood-onset rheumatic diseases. Clinicians adopt different practices for using CYC particularly in relation to indications, posology, pre-treatment laboratory work-up, post-treatment follow-up, and screening pre- and post-treatment vaccination status. This study aimed to evaluate the principles and approaches of administering CYC therapy in pediatric rheumatology and pediatric nephrology practices and to compare the clinician preferences for CYC and RTX in induction therapy of childhood-onset rheumatic diseases.</p><p><strong>Methods: </strong>This study includes a web-based questionnaire executed on 87 participants (56 pediatric rheumatologists (PRs) and 31 pediatric nephrologists (PNs)). Both pediatric subspecialties evaluated and compared the most common indications for CYC treatment, pre-treatment consent protocols, pre-and post-treatment laboratory tests, dosing strategies, and side effects.</p><p><strong>Results: </strong>Childhood-onset SLE (95%) and AAV (69%) were the most common diseases for which CYC treatment is used. All clinicians, except 2 PNs prescribed CYC via intravenous route. 61% of the PRs and 71% of PNs reported using a monthly dose of 500 mg/m² CYC for 6 months in accordance with the National Institutes of Health (NIH) protocol. All clinicians conducted pre-CYC treatment assessments of complete blood count and kidney function tests. Hepatitis B (82%), chickenpox (76%), and mumps-measles-rubella (72%) were the most frequently assessed vaccines. Adverse effects associated with CYC include cytopenia (86%), nausea (52%), liver toxicity (20%), hair loss (31%), hemorrhagic cystitis (37%), allergic reactions (16%), dyspnea (5%), and infertility (2%). 9 clinicians stated that they performed gonad-sparing interventions before CYC, which clarifies why CYC was more commonly preferred in the induction therapy of SLE and AAV over RTX by both PRs and PNs.</p><p><strong>Conclusions: </strong>Clinicians still tend to choose CYC over RTX in induction therapy of SLE and AAV and mostly prefer the high-dose CYC treatment regimen suggested by the NIH.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"24"},"PeriodicalIF":2.8,"publicationDate":"2025-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11895381/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143607097","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Minor salivary gland biopsy in the diagnosis of definite ocular sarcoidosis in paediatric granulomatous uveitis.","authors":"Leire Etchandy, Marie-Noelle Meaux, Olivier Richer, Chloé Bianchi, Skander Korbi, Claire Castain, Guillaume Chotard, Marie-Bénédicte Rougier, Emmanuel Ribeiro, Johanna Clet, Pascal Pillet, Jérôme Granel","doi":"10.1186/s12969-025-01078-3","DOIUrl":"10.1186/s12969-025-01078-3","url":null,"abstract":"<p><strong>Background: </strong>Non-infectious paediatric granulomatous uveitis (PGU) is a rare disease that is idiopathic in more than half of affected children. The diagnosis of definite ocular sarcoidosis (OS) must be supported by the presence of non-caseating granulomas detected in biopsy, and is therefore a challenge in children with PGU. This study investigated the utility of minor salivary gland biopsy (MSGB) in the diagnosis of definite OS in PGU.</p><p><strong>Methods: </strong>Twenty-six consecutive children with PGU diagnosed between 2018 and 2023 and with a systematically performed MSGB within 3 months of the diagnosis were enrolled.</p><p><strong>Results: </strong>The median age at PGU diagnosis was 11.6 (4.2-16.5) years, and 54% of the children were boys. PGU consisted mainly of bilateral (92%) pan-uveitis (96%). MSGB detected non-caseating granulomas (MSGB+) in 12/26 (46%) children. In all, 13 of the 26 (50%) children were diagnosed with definite OS, and 8 (31%) had idiopathic uveitis. MSGB had a sensitivity of 92%, and a NPV of 93% in the diagnosis of definite OS in children with PGU. Compared to MSGB- children, those who were MSGB + were more frequently older than 10 years of age at diagnosis (p = 0.02), had a higher rate of general signs (p = 0.003), extra-ocular organ involvement (p = 0.005) and polyclonal hypergammaglobulinaemia (p = 0.03). The most frequent extra-ocular organ involvements at OS diagnosis were renal (46%) and thoracic (46%). First-line therapy was systemic corticosteroids in 88% of the children. During a median follow-up time of 3.1 (0.6-6.3) years after PGU diagnosis, 88% of the children needed methotrexate and/or anti-tumour necrosis factor-alpha therapy to achieve inactive uveitis.</p><p><strong>Conclusions: </strong>MSGB is useful to improve the diagnosis of OS and to reduce the incidence of uveitis considered idiopathic in PGU. MSGB could be considered in PGU patients, particularly those > 10 years of age with general signs and/or hypergammaglobulinaemia.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"23"},"PeriodicalIF":2.8,"publicationDate":"2025-03-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11892283/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143598177","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Clinical remission rate and drug withdrawal status in articular juvenile idiopathic arthritis.","authors":"Akira Oshima, Takasuke Ebato, Masanori Kaneko, Yoshiaki Shikama, Tomoyuki Imagawa","doi":"10.1186/s12969-025-01075-6","DOIUrl":"10.1186/s12969-025-01075-6","url":null,"abstract":"<p><strong>Background: </strong>The clinical remission rate of articular juvenile idiopathic arthritis (JIA) differs according to the disease categories. At present, there is no consensus regarding drug withdrawal after remission is achieved.</p><p><strong>Objectives: </strong>To clarify the clinical remission rate and drug withdrawal status of patients with juvenile idiopathic arthritis (JIA).</p><p><strong>Methods: </strong>We conducted a retrospective observational study in patients who developed articular JIA by 2017 and were followed up (2013-2022). The Wallace criteria were used as remission criteria.</p><p><strong>Results: </strong>Forty-nine patients were included, i.e., 16 (33%) with polyarticular JIA (PJIA) and 33 (67%) with oligoarticular JIA (OJIA). Rheumatoid factor-positive (RF +) PJIA had significantly higher biological disease-modifying antirheumatic drug (bDMARD) introduction rates (86%, p < 0.01). The rate of clinical remission off medication was significantly higher in OJIA (67%). Numerous cases of RF + PJIA (50%), RF-negative (RF -) PJIA (25%), and OJIA (30%) flared within 2 years after conventional synthetic disease-modifying antirheumatic drug withdrawal. Patients with RF - PJIA and OJIA (two cases each) discontinued bDMARDs. Both RF - PJIA cases (100%) and half of OJIA cases (50%) flared within 2 years after bDMARD withdrawal. In one case of OJIA, remission was maintained after withdrawal of all drugs.</p><p><strong>Conclusions: </strong>OJIA had the highest rate of clinical remission off medication (67%) versus others. In OJIA, it was possible to discontinue all drugs in some patients with OJIA receiving bDMARDs. In PJIA requiring bDMARDs, withdrawal of bDMARDs was difficult all two cases.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"21"},"PeriodicalIF":2.8,"publicationDate":"2025-02-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11853753/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143494842","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Mortality in children and adolescents with autoimmune inflammatory rheumatic diseases admitted to the pediatric intensive care unit.","authors":"Tinnapat Buranapattama, Suwannee Phumeetham, Nuntawan Piyaphanee, Maynart Sukharomana, Sirirat Charuvanij","doi":"10.1186/s12969-025-01068-5","DOIUrl":"10.1186/s12969-025-01068-5","url":null,"abstract":"<p><strong>Background: </strong>This study aimed to describe the characteristics and outcomes of children and adolescents with autoimmune inflammatory rheumatic diseases (AIIRD) who were admitted to the pediatric intensive care unit (PICU). The accuracy of the Pediatric Risk of Mortality (PRISM) III and Pediatric Index of Mortality (PIM) 3 scores to predict the mortality were investigated.</p><p><strong>Methods: </strong>This was a retrospective cohort study. Children and adolescents with AIIRD aged ≤ 18 years who were admitted to the PICU at the largest university-based referral center in Thailand during July 2011 to June 2021 were included.</p><p><strong>Results: </strong>There were 122 PICU admissions from 74 patients; mean age of 12.0 ± 4.3 years, 74.3% female. Majority of AIIRD were systemic lupus erythematosus (SLE) (83.8%), followed by systemic juvenile idiopathic arthritis (5.4%), juvenile dermatomyositis (JDM) (2.7%) and microscopic polyangiitis (2.7%). The main cause of admission was combined infection and disease flare (29.5%). Pneumonia was the main site of infection. Acinetobacter baumanii was the most common causative agent. Macrophage activation syndrome occurred in 8 (6.5%) admissions. The mortality rate of PICU admissions was 14.8% from 18 deaths; 17 with SLE and 1 with JDM. Mechanical ventilation (aOR 24.07, 95%CI:1.33-434.91, P= 0.031), pneumothorax (aOR 24.08, 95%CI:1.76-328.86, P = 0.017 and thrombocytopenia (aOR 8.34, 95%CI:1.31-53.73, P = 0.025) were associated with mortality. The risk of mortality rate as predicted by the PRISM III score increased with a score ≥ 9. For the PIM 3 score, the risk of mortality increased if the score ≥ 3. The area under the ROC curve for the PRISM III and PIM 3 scores was 0.741 (95%CI: 0.633-0.849), P = 0.001 and 0.804 (95%CI: 0.685-0.924), P < 0.001, respectively. The model calibration using the Hosmer-Lemeshow goodness of fit test demonstrated a chi-square of 4.335, P = 0.826 for PRISM III and 7.987, P = 0.435 for PIM 3.</p><p><strong>Conclusion: </strong>SLE was the main AIIRD that required admission to the PICU. Mechanical ventilation, pneumothorax and thrombocytopenia were associated with mortality in pediatric patients with AIIRD. The PRISM III and PIM 3 scores demonstrated good calibration, while the PIM 3 score provided better discrimination ability in the prediction of mortality for pediatric AIIRD.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"23 1","pages":"20"},"PeriodicalIF":2.8,"publicationDate":"2025-02-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11843957/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143470227","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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