Successful treatment of anti-MDA5 antibody-positive refractory interstitial lung disease with plasma exchange therapy
Successful treatment of anti-MDA5 antibody-positive refractory interstitial lung disease with plasma exchange therapy
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DOI：
10.1093/rheumatology/kez357
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发表时间：
2020-04-01
期刊：
影响因子：
5.5
通讯作者：
Tamura, Naoto
中科院分区：
文献类型：
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作者：
Abe, Yoshiyuki;Kusaoi, Makio;Tamura, Naoto
目的：我们研究了血浆交换（PE）治疗降低抗黑色素瘤分化相关基因5 （MDA5）抗体阳性患者快速进展性间质性肺病（RP-ILD）死亡率的有效性。方法：在我院2008 - 2019年新诊断为PM/DM或临床淀粉样病变DM的142例患者中，10例诊断为难治性RP-ILD，抗mda5抗体阳性。PE作为标准治疗的辅助，由新鲜冷冻血浆作为替代溶液。主要结局是非疾病特异性死亡率。结果：28例患者检测到抗mda5抗体，其中21例诊断为RP-ILD， 10例对强化免疫抑制治疗无效。6例患者接受PE治疗（PE组），4例未接受PE治疗（非PE组）。PE组1年生存率高于非PE组（分别为100%和25%，P = 0.033）。与PE相关的不良事件中，2例发生过敏性休克，1例因新鲜冷冻血浆过敏出现高热，1例发生导管感染。经过适当的治疗，所有不良事件都得到了解决。结论：我们评估了抗mda5抗体阳性的难治性RP-ILD患者1年生存率与PE之间的关系。强化免疫抑制治疗提高了抗mda5抗体的RP-ILD患者的生存率，但仍有20-30%的病例死亡。PE可用于通过强化免疫抑制治疗而免疫功能低下的活动性传染病患者。抗mda5抗体阳性的难治性RP-ILD患者可考虑PE。
Objectives: We examined the effectiveness of plasma exchange (PE) therapy to reduce the mortality of rapidly progressive interstitial lung disease (RP-ILD) in patients positive for anti-melanoma differentiation-associated gene 5 (MDA5) antibodies.Methods: Among 142 patients newly diagnosed with PM/DM or clinically amyopathic DM from 2008 to 2019 at our hospital, 10 were diagnosed with refractory RP-ILD and were positive for anti-MDA5 antibodies. PE was used as an adjunct to standard therapy and consisted of fresh frozen plasma as replacement solution. The primary outcome was non-disease-specific mortality.Results: Anti-MDA5 antibodies were detected in 28 patients, of whom 21 were diagnosed with RP-ILD and 10 were refractory to intensive immunosuppressive therapy. Six patients received PE (PE group) and four did not (non-PE group). The 1-year survival rate of the PE group was higher than that of the non-PE group (100% and 25%, respectively, P = 0.033). Regarding adverse events associated with PE, two patients had anaphylactic shock, one had high fever due to fresh frozen plasma allergy and one had a catheter infection. All adverse events resolved with appropriate treatment.Conclusion: We evaluated the association between 1-year survival rate and PE for refractory RP-ILD in patients positive for anti-MDA5 antibodies. Intensive immunosuppressive therapy improved the survival rate in RP-ILD patients with anti-MDA5 antibodies, but 20-30% of cases were still fatal. PE could be administered to patients with active infectious disease who were immunocompromised by intensive immunosuppressive therapy. PE may be considered in refractory RP-ILD patients positive for anti-MDA5 antibodies.