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课件-伴肺部损害的自身免疫病-王国春.ppt

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SSc-ILD Azathioprine appears to be less efficacious in SSc-ILD than oral daily CTX. A retrospective series of 27 pts with SSc-ILD found improvements in pulmonary function tests after a combination of 6 months of monthly CTX IV followed by 18 months of AZA Nadashkevich O,et al.Clin. Rheumatol. 25, 205–212 (2006). Bérezné A,et al. J. Rheumatol. 35, 1064–1072 (2008). Treatment: AZA SSc-ILD Two retrospective studies have demonstrated clinical benefits, including an improved skin score, less new organ involvement and improved survival. A prospective double-blind study revealed that there was no difference in the extent of skin involvement or survival with d-penicillamine use. Clements PJ,et al.Arthritis Rheum. 42, 1194–1203 (1999). de Clerck LS,et al.Arthritis Rheum.30,643–650(1987). Steen VD,et al.Arthritis Rheum.28,882–888(1985). Treatment: D-penicillamine SSc-ILD Colchicine used for treating SSc-ILD demonstrated no improvement in lung function. Guttadauria M et al.. J. Rheumatol. 4, 272–276 (1977). Treatment: Colchicine IIM-ILD的发病率 IIM-ILD的发病机制 IIM-ILD的临床特征 IIM-ILD的血清生物标记物 IIM-ILD的治疗进展及预后 IIM-ILD IIM-ILD的发病率 Shu X, G Wang, et al. BMC Neurology 2011, 11:143 IIM-ILD Emerging data highlight the importance of autoantigen expression and conformation in the target tissue (lung and muscle,in this case), as well as identifying relevant amplifying pathways (such as regeneration). G Wick, 2013 31, Annu Rev Immunol IIM-ILD的发病机制 Sigong Zhang, Guochun Wang, et al. Clin Exp Immunol. 2014 ;177(1):134-41 PM/DM患者Nets形成增加以及DNA酶I活力降低导致的Nets降解减少,参与PM/DM的发病机制,尤其是在诱发ILD中起重要作用。 Sigong Zhang, Guochun Wang, et al. Clin Exp Immunol. 2014 ;177(1):134-41 IIM-ILD的临床特征 ILD可以是PM/DM的首发症状. 约18-20%发生在肌炎之前. 大部分患者与肌病其他症状同时出现或之后出现. 咳嗽和呼吸困难是最常见的症状. IIM-ILD的临床特征 ILD occurring in one of three patterns based on symptoms at presentation: ?rapidly progressive form with acute onset symptoms, ?subacute form with slowly progressive symptoms, and ?asymptomatic or subclinical form with an abnormal ches

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