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1、结缔组织病肺间质改变的诊治进展,北京协和医院风湿免疫科 张 烜,间质性肺病（interstitial lung disease，ILD）,CTD的常见疾病 可以是CTD的首发症状 是预后不良的原因之一 是肺动脉高压（pulmonary hypertension，PAH）的原因之一,影响肺功能,respiratory muscle dysfunction recurrent aspiration (esophageal dysmotility) thromboembolic events Inflammation of the cricoarytenoid joints or developme
2、nt of bronchiectasis.,ILD的发生率,15%的CTD合并ILD 70% SSc合并肺部病变，2年内可发展为ILD，组织病理77.5%SSc伴ILD 10%DM/PM合并ILD 胸片显示3.7%RA合并ILD SLE，SS，MCTD,Clin Chest Med 2004;25:549 559 Am J Respir Crit Care Med 2002;165:1581 6 Ann Rheum Dis 2003 ; 62 : 897 900,CTD中的ILD发生率、严重度及病死率的比较1）,CTD中ILD的特点,注：1）指5年之内因呼吸衰竭死亡2）国外无对比数据3）均为D
3、M患者,张烜,董怡,张奉春.中华风湿病学杂志, 1999;3:247,分型的目的,自然病程、对激素反应、预后 CTD-UIP是否等同特发UIP?,ILD病理和影像学特点,Curr Opin Rheumtol 2004;16:186 191,HRCT - non-specific interstitial pneumonia,Systemic sclerosis DM/PM,HRCT - usual interstitial pneumonia,Systemic sclerosis RA DM/PM,UIP-HRCT特点,patchy pattern of peripheral “honeyco
4、mbing” more prominent in the bases of the lungs, traction bronchiectasis absence of prominent ground-glass opacity.,HRCT - chronic lymphocytic interstitial pneumonia,Sjogrens syndrome RA Drug induced,HRCT obliterative bronchiolitis,RA SLE Scleroderma PM/DM,HRCT organizing pneumonia,Gold SSZ MTX Sjog
5、rens syndrom RA,HRCT a patient with RA,33% of with RA associated parenchymal lung disease 31IPF Radiography: 2-6% 29 HRCT: 10% - 47% 35-8HRCT: 50% with broncioectases and bronchiolectasis,SLE - Chronic interstitial pneumonia,Radiographic - 624%HRCT 24/34 abnormal11/34 CIP Fenlon HM, Doran M, Sant SM
6、, et al. Am J Roentgenol 1996;166:3017. Estes D, Christian CL. Medicine (Baltimore) 1971;50:8595.Raynauds phenomenon, swollen fingers, sclerodactyly, telangiectasia, dyspnoea, nailfold capillary abnormalitiesMay be efficacious: Corticosteroids Immunosuppressive agents,与ILD的相关因素,与病种有关 在RA中与RF的滴度有关 DM
7、/PM与抗Jo-1抗体有关 抗RNP抗体,Clin Exp Allergy 2003;33:226 232 Arthritis Rheum 2002;47:614 622,预后,病理分型还是CTD,治疗,ILD治疗的中心问题是GC和免疫抑制的指征GC是最常用药，众多病人无反应预后取决于分型GC+CTX疗效好于单用GC,Am J Respir Crit Care Med 1996;154:400 Arthritis Rheum 1994;37:1290 Semin Arthritis Rheum 2003;32:273,治疗,ILD治疗的中心问题是GC和免疫抑制的指征GC是最常用药，众多病人无反
8、应预后取决于分型GC+CTX疗效好于单用GC,Am J Respir Crit Care Med 1996;154:400 Arthritis Rheum 1994;37:1290 Semin Arthritis Rheum 2003;32:273,治疗RA-ILD,CTX,cyclosporine ,azathioprine, hydroxychloroquine have all been reported to be useful in the management of RA-associated ILD refractory to corticosteroids.,治疗RA-ILD
9、TNF？,In a preliminary study, infliximab also led to stabilization or improvement in symptoms, lung function, and radiology scores in five RA-ILD . RCT trial needed 非UIP-recommend corticosteroidsUIP-recommend TNFscreen for secondary PH. Bosentan?,治疗SSc-ILD,prognosis better -77.5% respond to IShistopa
10、thologic subsetseverity of disease at presentationincreased level of plasma homocysteinePHBAL eosinophil and neutrophil levels？TGFbeta,MMP,治疗SSc-ILD-ACR2006报道,美国Denver Fischer A对27例肺活检的SSc患者（14例NSIP，8例UIP）分析表明：尽管都予同样的激素和免疫抑制剂治疗，SSc-NSIP中位生存时间为15.3年，而UIP为3年。,the first positive results of a PCT,治疗SSc-
11、ILD-NEJM报道,DBRPCT 美国13个中心，158例患者 口服CTX或安慰剂年，随访年 PEP-FVC,治疗SSc-ILD-NEJM报道,Of 158 patients, 145 completed at least six months of treatment and were included in the analysis. The mean absolute difference in adjusted 12-month FVC% predicted between the CTX and placebo groups was 2.53% (95%CI 0.28 to 4.7
12、9%), favoring CTX (P8 ws) should be considered for P. carinii prophylaxis?,治疗ILD排除感染,CMV感染,治疗ILDMTX?,acute hypersensitivity pneumonitis and pulmonary fibrosis May predispose to P. carinii independently of corticosteroid use prefer to avoid using MTX in patients with ILDrecent studies suggest that lo
13、w-dose MTX is associated with a very low risk of pulmonary toxicity, suggesting that patients should not be automatically discontinued from the MTX, provided that the ILD does not progress,治疗IPF启示,治疗IPF启示,The synthesis of glutathione can be accelerated by the administration of NAC, which crosses cell membranes easily and can be converted to l-cysteine. Uptake of l-cysteine is an important rate-limiting step for the synthesis of glutathione. NAC increases the pool of other antioxidant thiols that also protect cells from injury.,
