1 / 59

结缔组织病肺间质改变的诊治进展

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

xandy
Download Presentation

结缔组织病肺间质改变的诊治进展

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  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

  2. 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

  3. 影响肺功能 • respiratory muscle dysfunction • recurrent aspiration (esophagealdysmotility) • thromboembolic events • Inflammationof the cricoarytenoid joints or • development ofbronchiectasis.

  4. 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

  5. CTD中ILD的特点 CTD中的ILD发生率、严重度及病死率的比较1） 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DM患者 张烜,董怡,张奉春.中华风湿病学杂志, 1999;3:247

  6. 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?

  7. ILD病理和影像学特点

  8. Curr Opin Rheumtol 2004;16:186 ~ 191

  9. HRCT - non-specific interstitial pneumonia Systemic sclerosis DM/PM

  10. HRCT - usual interstitial pneumonia Systemic sclerosis RA DM/PM

  11. UIP-HRCT特点 • patchy pattern of peripheral “honeycombing” • more prominent in the bases of the lungs, • traction bronchiectasis • absence ofprominent ground-glass opacity.

  12. HRCT - chronic lymphocytic interstitial pneumonia Sjogren’s syndrome RA Drug induced

  13. HRCT obliterative bronchiolitis RA SLE Scleroderma PM/DM

  14. HRCT organizing pneumonia Gold SSZ MTX Sjogren’s syndrom RA

  15. HRCT a patient with RA 33% of with RA associated parenchymal lung disease 31 IPF Radiography: 2-6% 29 HRCT: 10% - 47% 35-8 HRCT: 50% with broncioectases and bronchiolectasis

  16. SLE - Chronic interstitial pneumonia • Radiographic - 6–24% • HRCT – 24/34 abnormal • 11/34 CIP • Fenlon HM, Doran M, Sant SM, et al. Am J Roentgenol 1996;166:301–7. • Estes D, Christian CL. Medicine • (Baltimore) 1971;50:85–95. • Raynaud’s phenomenon, swollen fingers, sclerodactyly, telangiectasia, dyspnoea, nailfold capillary abnormalities • May be efficacious: • Corticosteroids • Immunosuppressive agents

  17. 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 Clin Exp Allergy 2003;33:226 ~ 232 Arthritis Rheum 2002;47:614 ~ 622

  18. 预后 • 病理分型还是CTD

  19. 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

  20. 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

  21. 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.

  22. 治疗RA-ILDTNFａ？ • In a preliminary study, infliximab also led to stabilization or improvement in symptoms, lung function, and radiologyscores in five RA-ILD . RCT trial needed • 非UIP--recommend corticosteroids UIP--recommend TNFａ screen for secondary PH. • Bosentan?

  23. 治疗SSc-ILD prognosis better --77.5% respond to IS histopathologic subset severity of disease at presentation increased level of plasma homocysteine PH BAL eosinophil and neutrophil levels？ TGFbeta,MMP

  24. 治疗SSc-ILD-ACR2006报道 美国DenverFischer 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。

  25. the first positive results of a PCT

  26. 治疗SSc-ILD-NEJM报道 DBRPCT 美国13个中心，158例患者 口服CTX或安慰剂１年，随访２年 PEP--FVC

  27. 治疗SSc-ILD-NEJM报道 • Of 158 patients, 145 completed at least six months of treatment and were includedin the analysis. • The mean absolute difference in adjusted 12-month FVC% predictedbetween the CTX and placebo groups was 2.53% (95%CI 0.28 to 4.79%), favoring CTX (P<0.03).

  28. 治疗SSc-ILD-NEJM报道 • There were also treatment-related differences in physiological and symptom outcomes,and the difference in FVC was maintained at 24 months. • There was a greaterfrequency of adverse events in the CTX group, but the difference not significant.

  29. 治疗SSc-ILD-NEJM报道 在治疗１年时，HRCT纤维化严重病人在安慰剂组FVC下降明显，而在CTX组纤维化对FVC影响不明显 (P = 0.009) 对SSc中已有纤维化说明存在相对早期活动性肺泡炎，如不治疗，病情容易进展。

  30. 治疗SSc-ILD-NEJM结论 • One year of oral CTX in symptomatic SSc-ILD had a significant but modest beneficial effect onlung function, dyspnea, thickening of the skin, and the health-related quality of life. • The effects on lung function weremaintained through the 24 ms of the study.

  31. 治疗SSc-ILD-NEJM问题 no significant treatment-related difference was noted in the DLco

  32. 治疗ILD－排除感染 • mimic ILD or develop as a complication of immunosuppressivetherapy. • 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?

  33. 治疗ILD－排除感染 CMV感染

  34. 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 does not progress

More Related