全身型幼年特发性关节炎合并肺间质病变概述

全身型幼年特发性关节炎是儿童常见的风湿性疾病之一,肺间质病变是指以肺间质和远端气隙重构为特征,进而导致异常气体交换的疾病.近年来,国内外对全身型幼年特发性关节炎合并肺间质病变的报道有增多趋势.该文总结了近年来报道中全身型幼年特发性关节炎儿童合并肺间质病变的情况,并对儿童肺间质病变的临床表现,影像学表现,诊断和治疗进行综...     

儿童系统性红斑狼疮伴肺间质病变的临床特征

目的了解儿童系统性红斑狼疮伴肺间质病变（SLE-ILD）的发生情况、特征。方法系统性红斑狼疮（SLE）患儿52例进行胸部X线和肺高分辨率CT（HRCT）检查,由2名以上放射科医师阅片,描述影像表现性质、程度。同时结合临床表现、血清学检查进行相关因素分析。结果SLE患儿出现ILD占26.9%,肺HRCT在发现SLE-ILD病变时优于普通胸片;根据患儿的临床表现、胸片、肺HRCT等,可将SLE-ILD患儿分为临床前期型12例（85.7%）,急性肺泡炎型2例（14.3%）,未发现有慢性ILD病变型病例;SLE-ILD患儿并浆膜炎发生率明显升高,而并肾损害发生率却明显降低（P〈0.05）;SLE-ILD患儿抗SSA抗体的阳性率明显高于无ILD组（P〈0.05）。结论儿童SLE-ILD的发生在儿童中并不少见,SLE-ILD更易出现在并浆膜炎、无肾损害及抗SSA抗体阳性患儿。肺HRCT对发现早期肺间质病变有诊断意义,对SLE-ILD患儿进行临床分型对预后判断和指导治疗有重要的价值。     

儿童混合性结缔组织病9例临床分析

目的总结分析儿童混合性结缔组织病(MCTD)的临床及转归特点。方法对2001—2009年间确诊为MCTD的9例患儿的临床表现、实验室检查、诊治及随访情况进行总结分析。结果 9例患儿均有雷诺现象,其次常见的症状包括关节肿痛、手指肿胀、硬指、发热、乏力、贫血、活动后气短等。血液系统受累4例,其中3例轻度贫血,1例血小板减少。1例肾活检提示符合狼疮肾炎IIA型。实验室指标中红细胞沉降率增快8例,IgG升高7例,C4降低3例,CH50升高5例,CK升高4例(该4例患儿均行肌电图检查,3例未见明显肌源性损害,1例为可疑肌源性损害)。自身抗体ANA阳性9例,抗U1-RNP抗体阳性9例,抗SSA抗体低滴度阳性3例。6例行肺功能检查,4例存在弥散功能障碍。6例行肺部高分辨CT检查,2例示肺间质病变。超声心动图检查示肺动脉高压3例,合并右心增大、主动脉增宽2例,轻度肺动脉高压1例,合并少量心包积液1例。3例行食管造影检查,未见明显食管蠕动障碍。多数患儿入院前曾诊断为系统性红斑狼疮、幼年特发性关节炎、雷诺现象、结缔组织病、发热原因待查等,不规律接受激素或免疫抑制剂治疗者4例。9例患儿经确诊后予规范激素和(或)免疫抑制剂治疗,8例病情好转,1例病情控制无明显进展,无死亡病例。结论儿童MCTD为多系统受累,早期以雷诺现象、关节症状、发热多见,在尚未出现肺部症状时可存在肺功能、肺部高分辨CT异常,可伴有肺动脉高压,误诊率较高。早期完善相关免疫指标、超声心动图、肺功能、肺部高分辨CT、食管造影等检查可帮助诊断。规范的激素及免疫抑制剂治疗对病情转归及预后极为重要,需长期对患儿进行随访观察。     

儿童风湿性疾病合并巨噬细胞活化综合征六例临床分析

目的巨噬细胞活化综合征（MAS）的诊断和治疗尚未得到统一,总结分析我院6例MAS患儿的临床资料,为临床救治工作提供参考。方法回顾性分析我院1998-2004年风湿性疾病合并MAS患儿的临床资料。结果共有6例（女4例,男2例）患儿符合MAS的诊断条件。发生MAS前,4例诊断幼年特发性关节炎全身型（SOJIA）,2例诊断系统性红斑狼疮和狼疮性肾炎。所有病例确诊MAS时疾病状态处于低活动期,主要的临床特征是：峰形热（5／6）或中高热（1／6）,肝脾肿大（6／6）,淋巴结病（6／6）,外周血3系减少（6／6）,明显的肝损伤（6／6）,弥漫性血管内凝血象（2／6）和中枢神经系统功能障碍（3／6）。低纤维蛋白原血症、肝酶升高和高甘油三酯血症均持续存在。3例骨髓涂片可见吞噬血细胞现象和浆细胞岛,其他3例未行此检查。3例患儿被证实存在感染,2例单纯疱疹病毒（HSV）和1例甲型肝炎病毒（HAV）。未发现药物相关因素。分别进行个体化治疗,重点在早期发现和联合抗病毒治疗,适度地联合免疫化疗（HLH-94或环孢素A）可以获得及时的缓解。1例SOJIA患儿严重肝损伤经连续性血液滤过／肾脏替代治疗联合免疫化疗获得迅速缓解。2例SOJIA患儿死于严重的内脏出血。结论MAS不仅见于SOJIA,还可以发生在儿科其他风湿性疾病的任何阶段,其发生率可能要高于以往的认识。感染可能是主要的触发因素。适时的免疫净化联合免疫化疗有益于严重肝损伤的恢复。     

儿童川崎病与关节炎关系的研究进展

儿童川崎病(Kawasaki disease,KD)或不典型川崎病(incomplete Kawasaki disease,IKD)是一种以全身血管炎病变为主要病理特征的疾病,临床主要表现为无明显诱因的发热,伴有皮肤黏膜及淋巴结的损害.由于本病常伴有严重的心血管并发症如冠状动脉瘤,故引起人们的重视.但由于约三分之一患儿可伴有关节炎或关节痛,早期不易与幼年特发性关节炎区别,尤其是与全身型幼年特发性关节炎(sys-temic-onset juvenile idiopathic arthritis,SoJIA)的早期区分困难.KD和SoJIA是对于儿童长期发热、皮疹、淋巴结肿大的不同诊断,诊疗程序也截然不同,但在早期临床诊断上完全区分两者是很困难的,尤其是IKD和早期SoJIA.故该文对KD与关节炎的关系作一综述,旨在提醒临床医生重视KD与IKD的诊断,以免延误治疗.     

儿童混合性结缔组织病诊治新进展

混合性结缔组织病(mixed connective tissue disease,MCTD)是一种以多脏器损害为主的自身免疫性疾病,该病的临床特征有类似系统性红斑狼疮(SLE)、系统性硬化症(PSS)、多发性肌炎/皮肌炎(PM/DM)和类风湿性关节炎(RA)的混合表现,并伴有血清学上极高滴度的斑点型抗核抗体和高滴度U1RNP抗体。该病病因及发病机制尚不明确,是一种免疫功能紊乱的疾病,B细胞过度活化产生的自身抗体、Th1/Th2细胞的平衡偏离导致的Th1/Th2细胞因子网络的改变在MCTD的发病机制中可能存在着一定的作用[1]。儿童MCTD的临床表现多种多样,症状多不典型,极易…     

儿童风湿病临床研究进展

1 幼年特发性关节炎 目前,幼年特发性关节炎(JIA)发病机制仍然不明确,已知中性粒细胞在类风湿关节炎关节中大量存在,推测是中性粒细胞分泌酶类和氧化剂损害了组织.Hallett等[1]通过基因敲除动物模型证实了中性粒细胞移动至关节,在类风湿关节炎发病机制中起重要作用.该研究利用反义RNA敲除鼠细胞磷脂酶A-Mpha的表达,使中性粒细胞移动至关节过程受到影响,从而减少了模型动物组织炎症水肿和损伤,此研究为利用中性粒细胞抑制剂治疗类风湿关节炎奠定了基础.     

儿童风湿病相关巨噬细胞活化综合征单中心临床研究

目的 总结儿童风湿病相关巨噬细胞活化综合征(MAS)临床和实验室特征、治疗及转归.方法 回顾性分析2008年1月至2019年11月重庆医科大学附属儿童医院75例MAS患儿的临床和实验室特征、治疗及转归.结果 MAS的基础疾病包括全身型幼年特发性关节炎(SJIA) 32例、系统性红斑狼疮(SLE) 22例、川崎病(KD)...     

儿童风湿病相关性肺间质病变的诊断和治疗进展

儿童风湿病是一组自身免疫性疾病,主要累及结缔组织和血管.由于肺脏具有丰富的结缔组织和血管,风湿病常伴有肺间质病变,预后极差.儿童风湿病更易发生肺间质病变,而且以亚临床型多见,但能通过影像学等检查早期发现,及时治疗可改善预后.现主要介绍儿童风湿病相关性肺间质病变的流行病学、临床表现、诊断、影像学表现和病理基础、治疗及预后.     

Ӥ�׶������֯������Էμ��ʼ���

??Abstract??Interstitial lung disease ??ILD?? is a group of widely heterogeneous etiology disease. Infant and early childhood special type of ILD belongs to a more complex etiology category. Connective tissue disease ??CTD?? is a group of autoimmune disease involves multi-systems and organs in the whole body?? lung is the organ most commonly involved. Connective tissue disease-associated interstitial lung disease ??CTD-ILD?? and ILD have similar clinical symptoms?? with their own characteristics?? but different treatment options and prognosis. Deeply understanding the infant CTD-ILD and ILD?? learning their clinical analysis ideas?? will become very important in carrying out extensive clinical research for prognosis and therapy in the future.     

警惕儿童结缔组织疾病合并结核感染

结缔组织病患儿本身存在多种免疫紊乱,大剂量皮质激素、免疫抑制剂的应用加重了细胞免疫缺陷,生物制剂进一步增加了结核感染的风险.结缔组织病合并结核感染临床表现复杂,有时很难鉴别是原发疾病活动还足结核感染.肺部影像学检查是临床诊断结核病的主要手段,QFT-G试验和T-SPOT.TB开辟了辅助诊断新途径.应详细询问结核接触史、卡介苗接种史,常规进行血清结核杆菌抗体检测及PPD皮试.临床高度提示结核感染者,应给予诊断性抗结核治疗.     

Soluble intercellular adhesion molecule-1 in paediatric connective tissue diseases

The intercellular adhesion molecule-1 (ICAM-1) is a cytokine-induced glycoprotein involved in the recruitment of cells into tissues undergoing inflammatory responses. The aim of this study was to compare the levels of soluble ICAM-1 (s-ICAM-1) in children with juvenile chronic arthritis (JCA) and systemic lupus erythematosus (SLE) and to evaluate the usefulness of this molecule as marker of disease activity. Levels of s-ICAM-1 were measured in sera using a monoclonal antibody sandwich enzyme-linked immunoassay. Serum levels (mean ± SD) of s-ICAM-1 in 37 children with JCA, 18 patients suffering from SLE and 25 healthy controls were 609 ± 184, 513 ± 139 and 210 ± 95 ng/ml, respectively. A significant difference could be demonstrated between the levels of s-ICAM-1 in sera from each disease, as a group, and those of healthy controls. Higher levels of s-ICAM-1 were recorded in JCA patients with systemic features and patients who had polyarthritis than in children who were pauciarticular. A positive correlation was observed between s-ICAM-1 levels and disease activity score in SLE patients. Moreover, s-ICAM-1 levels closely followed clinical conditions in five children with SLE during follow-up. The data show that s-ICAM-1 levels are increased in children suffering from connective tissue diseases and reflect disease status or activity, suggesting the usefulness of this molecule in the follow-up of these diseases.     

С���ط��Լ����Է���18���ٴ�����

??Abstract??Objective??In order to reduce misdiagnosis of idiopathic interstitial pneumonia ??IIP?? in children we summarized the etiology?? clinical characteristics?? treatment and follow-up results of 18 cases. Method??The clinical epidemic investigations were used in our present study. A retrospective analysis was made on the pediatric patient's clinical materials??who were diagnosed with IIP. Some of the serum indexes were compared with those of 35 children who came to our hospital for physical examination. Results??The etiology was unknown in these patients?? we just found some background factors. The main symptoms were progressive dyspnea and dry cough?? most of patients usually had no fever?? but had weight loss?? fatigue and poor appetite. Fine crackles could be heard on the base of bilateral lungs?? accompanied with cyanosis and clubbed fingers. Prior to being admitted to the hospital?? the patients were misdiagnosed with bronchopneumonia?? asthma or pulmonary tuberculosis etc. Blood test showed that WBC was elevated?? ESR was accelerated?? CRP and LDH??TNF-αand IL-8 levels were increased??but α-AT decreased. X ray film indicated diffused infiltrating change and HRCT characterized by reticulate?? honey comb and frosted change. Lung function test ??>8 years?? showed restrictive ventilation function impairment??blood gas analysis and monitoring showed persistent hypoxemia. The patients received systemic corticosteroid pulse therapy combined with intravenous gamma immunoglobulin?? azithromycin taken orally?? inhaled Fluticasone aerosol therapy and follow. Twelve cases clinically resolved?? 3 cases died?? there was no improvement in 3 case and they gave up treatment. Conclusion??The etiology is uncertain in this cohort?? and childhood IIP is easily misdiagnosed and under-diagnosed. The duration and degree of disease is prolonged. Most patients have persistency or recurrent symptoms. The key point of early diagnosis is clinical manifestations?? laboratory test results and imaging studies. Although the treatment effect is not ideal in this cohort?? combination therapy can improve symptoms in some patients. Pulmonary biopsy is very difficult to perform in the clinic.     

Quality of life and daily management of children with rheumatic disease

Appropriate management of the daily life of patients with rheumatic diseases is an important part of therapy for favorable results and a high quality of life. At home, gymnastic exercise, night splints and appropriate fun activities will help to preserve the function of affected joints of patients with juvenile rheumatoid arthritis (JRA). Improvement of home fixtures according to patient disability will provide a higher quality of life for the patients. The patient should be helped to perform as many school activities as would normally be possible. Instructional charts for management of school life for patients with JRA and systemic lupus erythematosus is shown in this article. Cooperation with school personnel is also crucial to support the patient emotionally and physiologically. For the best long-term treatment and a good relationship between patient and physician, comprehensive management is essential. Not only physicians, but also psychiatrists, occupational therapists, physical therapists and social workers should be involved in the treatment of children with rheumatic diseases.