Systemic juvenile idiopathic arthritis–associated lung disease: A retrospective cohort study  

作　　者：Konstantin E Belozerov Natalia M Solomatina Eugenia A Isupova Alla A Kuznetsova Mikhail M Kostik 

机构地区：[1]Department of Pediatric,Saint-Petersburg State Pediatric Medical University,Saint-Petersburg 194100,Russia [2]St.Petersburg State Budgetary Institution of Health Care,Children's City Polyclinic No.29 of the Kalininsky District of St.Petersburg,St.Petersburg 195274,Russia [3]Research Laboratory of Autoimmune and Autoinflammatory Diseases,World-Class Research Centre for Personalized Medicine,Almazov National Medical Research Centre,St.Petersburg 197341,Russia

出　　处：《World Journal of Clinical Pediatrics》2024年第1期73-83,共11页世界临床儿科杂志

基　　金：Supported by the Ministry of Science and Higher Education of the Russian Federation,No.075-15-2022-301.

摘　　要：BACKGROUND Lung damage in systemic juvenile arthritis(sJIA)is one of the contemporary topics in pediatric rheumatology.Several previous studies showed the severe course and fatal outcomes in some patients.The information about interstitial lung disease(ILD)in the sJIA is scarce and limited to a total of 100 cases.AIM To describe the features of sJIA patients with ILD in detail.METHODS In the present retrospective cohort study,information about 5 patients less than 18-years-old with sJIA and ILD were included.The diagnosis of sJIA was made according to the current 2004 and new provisional International League of Associations for Rheumatology criteria 2019.ILD was diagnosed with chest computed tomography with the exclusion of other possible reasons for concurrent lung involvement.Macrophage activation syndrome(MAS)was diagnosed with HLH-2004 and 2016 EULAR/ACR/PRINTO Classification Criteria and hScores were calculated during the lung involvement.RESULTS The onset age of sJIA ranged from 1 year to 10 years.The time interval before ILD ranged from 1 mo to 3 years.The disease course was characterized by the prevalence of the systemic features above articular involvement,intensive rash(100%),persistent and very active MAS(hScore range:194-220)with transaminitis(100%),and respiratory symptoms(100%).Only 3 patients(60%)developed a clubbing phenomenon.All patients(100%)had pleural effusion and 4 patients(80%)had pericardial effusion at the disease onset.Two patients(40%)developed pulmonary arterial hypertension.Infusion-related reactions to tocilizumab were observed in 3(60%)of the patients.One patient with trisomy 21 had a fatal disease course.Half of the remaining patients had sJIA remission and 2 patients had improvement.Lung disease improved in 3 patients(75%),but 1 of them had initial deterioration of lung involvement.One patient who has not achieved the sJIA remission had the progressed course of ILD.No cases of hyper-eosinophilia were noted.Four patients(80%)received canakinumab and one(20%)tocilizumab at the last

关 键 词：Systemic juvenile arthritis Interstitial lung disease CANAKINUMAB TOCILIZUMAB INTERLEUKIN-6 INTERLEUKIN-1 

分 类 号：R725.7[医药卫生—儿科] R725.9[医药卫生—临床医学]