结缔组织病肺间质改变的诊治进展.pptVIP

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 development 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 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 HRCT - non-specific interstitial pneumonia HRCT - usual interstitial pneumonia UIP-HRCT特点 patchy pattern of peripheral “honeycombing” more prominent in the bases of the lungs, traction bronchiectasis absence of　prominent ground-glass opacity. HRCT - chronic lymphocytic interstitial pneumonia HRCT obliterative bronchiolitis HRCT organizing pneumonia HRCT a patient with RA SLE - Chronic interstitial pneumonia 与ILD的相关因素 与病种有关 在RA中与RF的滴度有关 DM/PM与抗Jo-1抗体有关 抗RNP抗体 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 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 治疗RA-ILD 治疗RA-ILD　　TNFａ？ 治疗SSc-ILD 治疗SSc-ILD-ACR2006报道 治疗SSc-ILD-NEJM报道 治疗SSc-ILD-NEJM报道 治疗SSc-ILD-NEJM报道 治疗SSc-ILD-NEJM报道 治疗SSc-ILD-NEJM结论 治疗SSc-ILD-NEJM问题 治疗ILD－排除感染 mimic ILD or develop as a complication of immunosuppressive　therapy. Pneumocystis carinii pneumonia. 　　SLE, myositis,WG—chronic corticosteroids, MTX, inhibitors of TNFa all patients with CTD on chronic immunosuppression (8 ws) should be considered for P. carinii prophylaxis? 治疗ILD－排除感染 CMV感染 治疗ILD－MTX? acute hypersensitivity pneumonitis and pulmonary fibrosis May predispose to P. carinii independently of corticosteroid use prefer to avoid using MTX in patients with ILD recent studies suggest that low-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