发病时呼吸道病变发展的全身性幼年关节炎的表现特点。单中心回顾性队列研究和文献综述的结果

Q4 Medicine

Pediatriya - Zhurnal im G.N. Speranskogo Pub Date : 2023-10-13 DOI:10.24110/0031-403x-2023-102-5-44-53

K.E. Belozerov, N.M. Solomatina, N.N. Abramova, E.A. Isupova, E.V. Gaidar, M.A. Kaneva, T.L. Kornishina, V.V. Masalova, O.V. Kalashnikova, I.A. Chikova, V.G. Chasnyk, A.A. Kuznetsova, M.M. Kostik, D.O. Ivanov

{"title":"发病时呼吸道病变发展的全身性幼年关节炎的表现特点。单中心回顾性队列研究和文献综述的结果","authors":"K.E. Belozerov, N.M. Solomatina, N.N. Abramova, E.A. Isupova, E.V. Gaidar, M.A. Kaneva, T.L. Kornishina, V.V. Masalova, O.V. Kalashnikova, I.A. Chikova, V.G. Chasnyk, A.A. Kuznetsova, M.M. Kostik, D.O. Ivanov","doi":"10.24110/0031-403x-2023-102-5-44-53","DOIUrl":null,"url":null,"abstract":"Juvenile arthritis with systemic onset (sJIA) is one of the most difficult tasks of differential diagnosis and selection of therapy for a rheumatologic practitioner as yet. The presence of a systemic hyperinflammatory response, the development of macrophage activation syndrome (MAS) and sepsis determine the severity of sJIA. Pulmonary involvement coupled with the development of interstitial lung disease and/or pulmonary arterial hypertension is a relatively new, potentially life-threatening complication of sJIA. The purpose of this research was to characterize patients with sJIA who had respiratory lesions (RL) at the onset of the disease. Methods used: a retrospective cohort study included the data from the case histories of 85 pediatric patients with sJIA who were divided into 2 groups: G1 with RL (dyspnea, pleurisy, interstitial lung disease, acute respiratory distress syndrome); and G2 of those who’ve had no such complaints. Results: 18 (21.1%) had RL associated with female gender, heart lesions (OR=6.4 (2.1; 19.7), p=0.0006), macrophage activation syndrome (72.2%; OR=6.6 (2.1; 21.0), p=0.0006), hemorrhagic syndrome, CNS disorders (OR=6.1 (1.2; 30.3), p=0.015), decreased levels of hemoglobin, platelets, prothrombin, fibrinogen, albumin, total protein and sodium as well as the higher levels of ferritin, lactate dehydrogenase, aspartate aminotransferase, triglycerides. Patients with lung involvement had tended to have higher C-reactive protein levels and were more likely to have transient proteinuria in the active phase of the disease. Multivariate analysis identified two criteria associated with the respiratory damage: heart damage (β=0.18, p=0.039) and hypoalbuminemia <26 g/l (β=0.73, p=0.0000001). Conclusion: the presence of macrophage activation syndrome, damage to the cardiovascular system requires targeted monitoring of respiratory damage in patients with sJIA.","PeriodicalId":39654,"journal":{"name":"Pediatriya - Zhurnal im G.N. Speranskogo","volume":"34 1","pages":"0"},"PeriodicalIF":0.0000,"publicationDate":"2023-10-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"MANIFESTATIONS PECULIARITIES OF JUVENILE ARTHRITIS WITH SYSTEMIC ONSET IN PATIENTS WITH THE RESPIRATORY LESIONS’ DEVELOPMENT AT THE ONSET OF THE DISEASE. RESULTS OF A SINGLE-CENTER RETROSPECTIVE COHORT STUDY AND A BIBLIOGRAPHICAL REVIEW\",\"authors\":\"K.E. Belozerov, N.M. Solomatina, N.N. Abramova, E.A. Isupova, E.V. Gaidar, M.A. Kaneva, T.L. Kornishina, V.V. Masalova, O.V. Kalashnikova, I.A. Chikova, V.G. Chasnyk, A.A. Kuznetsova, M.M. Kostik, D.O. Ivanov\",\"doi\":\"10.24110/0031-403x-2023-102-5-44-53\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Juvenile arthritis with systemic onset (sJIA) is one of the most difficult tasks of differential diagnosis and selection of therapy for a rheumatologic practitioner as yet. The presence of a systemic hyperinflammatory response, the development of macrophage activation syndrome (MAS) and sepsis determine the severity of sJIA. Pulmonary involvement coupled with the development of interstitial lung disease and/or pulmonary arterial hypertension is a relatively new, potentially life-threatening complication of sJIA. The purpose of this research was to characterize patients with sJIA who had respiratory lesions (RL) at the onset of the disease. Methods used: a retrospective cohort study included the data from the case histories of 85 pediatric patients with sJIA who were divided into 2 groups: G1 with RL (dyspnea, pleurisy, interstitial lung disease, acute respiratory distress syndrome); and G2 of those who’ve had no such complaints. Results: 18 (21.1%) had RL associated with female gender, heart lesions (OR=6.4 (2.1; 19.7), p=0.0006), macrophage activation syndrome (72.2%; OR=6.6 (2.1; 21.0), p=0.0006), hemorrhagic syndrome, CNS disorders (OR=6.1 (1.2; 30.3), p=0.015), decreased levels of hemoglobin, platelets, prothrombin, fibrinogen, albumin, total protein and sodium as well as the higher levels of ferritin, lactate dehydrogenase, aspartate aminotransferase, triglycerides. Patients with lung involvement had tended to have higher C-reactive protein levels and were more likely to have transient proteinuria in the active phase of the disease. Multivariate analysis identified two criteria associated with the respiratory damage: heart damage (β=0.18, p=0.039) and hypoalbuminemia <26 g/l (β=0.73, p=0.0000001). Conclusion: the presence of macrophage activation syndrome, damage to the cardiovascular system requires targeted monitoring of respiratory damage in patients with sJIA.\",\"PeriodicalId\":39654,\"journal\":{\"name\":\"Pediatriya - Zhurnal im G.N. Speranskogo\",\"volume\":\"34 1\",\"pages\":\"0\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2023-10-13\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Pediatriya - Zhurnal im G.N. Speranskogo\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.24110/0031-403x-2023-102-5-44-53\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"Medicine\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Pediatriya - Zhurnal im G.N. Speranskogo","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.24110/0031-403x-2023-102-5-44-53","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"Medicine","Score":null,"Total":0}

引用次数: 0

摘要

全身性幼年关节炎(sJIA)是鉴别诊断和选择治疗风湿病从业者最困难的任务之一。系统性高炎症反应、巨噬细胞激活综合征(MAS)和脓毒症的出现决定了sJIA的严重程度。肺受累合并间质性肺疾病和/或肺动脉高压是sJIA的一个相对较新的、可能危及生命的并发症。本研究的目的是表征sJIA发病时有呼吸道病变(RL)的患者。方法:回顾性队列研究纳入85例小儿sJIA患者的病史资料，分为2组:G1组为RL(呼吸困难、胸膜炎、间质性肺疾病、急性呼吸窘迫综合征);没有这种抱怨的人占G2。结果:女性RL患者18例(21.1%)，心脏病变(OR=6.4;19.7)， p=0.0006)，巨噬细胞激活综合征(72.2%;= 6.6 (2.1;21.0)， p=0.0006)，出血性综合征，中枢神经系统疾病(OR=6.1 (1.2;30.3)， p=0.015)，血红蛋白、血小板、凝血酶原、纤维蛋白原、白蛋白、总蛋白和钠水平降低，铁蛋白、乳酸脱氢酶、天冬氨酸转氨酶、甘油三酯水平升高。累及肺部的患者往往有较高的c反应蛋白水平，并且更有可能在疾病的活动期出现短暂性蛋白尿。多变量分析确定了与呼吸损伤相关的两个标准:心脏损伤(β=0.18, p=0.039)和低白蛋白血症(β=0.73, p=0.0000001)。结论:sJIA患者存在巨噬细胞激活综合征，对心血管系统的损害需要有针对性地监测呼吸损伤。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

查看原文本刊更多论文

MANIFESTATIONS PECULIARITIES OF JUVENILE ARTHRITIS WITH SYSTEMIC ONSET IN PATIENTS WITH THE RESPIRATORY LESIONS’ DEVELOPMENT AT THE ONSET OF THE DISEASE. RESULTS OF A SINGLE-CENTER RETROSPECTIVE COHORT STUDY AND A BIBLIOGRAPHICAL REVIEW

Juvenile arthritis with systemic onset (sJIA) is one of the most difficult tasks of differential diagnosis and selection of therapy for a rheumatologic practitioner as yet. The presence of a systemic hyperinflammatory response, the development of macrophage activation syndrome (MAS) and sepsis determine the severity of sJIA. Pulmonary involvement coupled with the development of interstitial lung disease and/or pulmonary arterial hypertension is a relatively new, potentially life-threatening complication of sJIA. The purpose of this research was to characterize patients with sJIA who had respiratory lesions (RL) at the onset of the disease. Methods used: a retrospective cohort study included the data from the case histories of 85 pediatric patients with sJIA who were divided into 2 groups: G1 with RL (dyspnea, pleurisy, interstitial lung disease, acute respiratory distress syndrome); and G2 of those who’ve had no such complaints. Results: 18 (21.1%) had RL associated with female gender, heart lesions (OR=6.4 (2.1; 19.7), p=0.0006), macrophage activation syndrome (72.2%; OR=6.6 (2.1; 21.0), p=0.0006), hemorrhagic syndrome, CNS disorders (OR=6.1 (1.2; 30.3), p=0.015), decreased levels of hemoglobin, platelets, prothrombin, fibrinogen, albumin, total protein and sodium as well as the higher levels of ferritin, lactate dehydrogenase, aspartate aminotransferase, triglycerides. Patients with lung involvement had tended to have higher C-reactive protein levels and were more likely to have transient proteinuria in the active phase of the disease. Multivariate analysis identified two criteria associated with the respiratory damage: heart damage (β=0.18, p=0.039) and hypoalbuminemia <26 g/l (β=0.73, p=0.0000001). Conclusion: the presence of macrophage activation syndrome, damage to the cardiovascular system requires targeted monitoring of respiratory damage in patients with sJIA.

求助全文

通过发布文献求助，成功后即可免费获取论文全文。去求助

来源期刊

Pediatriya - Zhurnal im G.N. Speranskogo Medicine-Pediatrics, Perinatology and Child Health

CiteScore

0.60

自引率

0.00%

发文量

150

期刊介绍： Journal “Pediatria” named after G.N. Speransky (the official short names of the Journal are “Journal «Pediatria»,” “Pediatria,” and “«Pediatria,» the Journal”) is the oldest Soviet-and-Russian (in the Russian Federation, the CIS and former Soviet Union) scientific and practical medical periodical assigned for pediatricians that is published continuously since May, 1922, and distributed worldwide. Our mission statement specifies that we aim to the ‘raising the level of skills and education of pediatricians, organizers of children’s health protection services, medicine scientists, lecturers and students of medical institutes for higher education, universities and colleges worldwide with an emphasis on Russian-speaking audience and specific, topical problems of children’s healthcare in Russia, the CIS, Baltic States and former Soviet Union Countries and their determination with the use of the World’s best practices in pediatrics.’ As part of this objective, the Editorial of the Journal «Pediatria» named after G.N. Speransky itself adopts a neutral position on issues treated within the Journal. The Journal serves to further academic discussions of topics, irrespective of their nature - whether religious, racial-, gender-based, environmental, ethical, political or other potentially or topically contentious subjects. The Journal is registered with the ISSN, - the international identifier for serials and other continuing resources, in the electronic and print world: ISSN 0031-403X (Print), and ISSN 1990-2182 (Online). The Journal was founded by the Academician, Dr. Georgiy Nestorovich SPERANSKY, in May, 1922. Now (since 1973) the Journal bears his honorary name.