Anti-MDA5 antibody, ferritin and IL-18 are useful for the evaluation of response to treatment in interstitial lung disease with anti-MDA5 antibody-positive dermatomyositis
Anti-MDA5 antibody, ferritin and IL-18 are useful for the evaluation of response to treatment in interstitial lung disease with anti-MDA5 antibody-positive dermatomyositis
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DOI：
10.1093/rheumatology/kes102
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发表时间：
2012-09-01
期刊：
影响因子：
5.5
通讯作者：
Yamanaka, Hisashi
中科院分区：
文献类型：
--
作者：
Gono, Takahisa;Sato, Shinji;Yamanaka, Hisashi
Objective.本研究的目的是调查确切的临床特征，并分析抗MDA 5抗体（抗MDA 5ab）滴度与抗MDA 5ab阳性DM患者疾病状态之间的相关性。入组了27例抗MDA 5ab阳性的DM患者。分析其临床表现与抗MDA 5ab等临床指标的相关性。20例（74%）患者发生快速进展性间质性肺疾病（RP-ILD）并发症。致死性结局、复发和恶性肿瘤的发生率分别为33%、4%和4%。值得注意的是，在前6个月内发生了致命的结局。与6例非RP-ILD患者相比，20例抗-MDA 5ab RP-ILD患者的发病年龄较大，肺功能严重受损，血清铁蛋白水平较高。入院时肺泡-动脉血氧分压差（AaDO（2））≥ 32 mmHg和铁蛋白≥ 828 ng/ml是抗-MDA 5ab阳性DM的RP-ILD患者的不良预后因素。入院时抗MDA 5ab滴度的中位数在后来死亡的患者中高于存活的患者。通过抗-MDA 5ab、铁蛋白和IL-18浓度指示治疗的功效。治疗后，死亡患者亚组的抗MDA 5ab滴度下降指数低于存活患者亚组。死亡患者血清抗MDA 5ab、铁蛋白和IL-18水平持续升高。抗MDA 5ab滴度和铁蛋白及IL-18浓度可用于评价抗MDA 5ab阳性DM患者的治疗应答和ILD状态。
Objective. The aim of this study was to investigate the precise clinical characteristics and to analyse the association between the anti-MDA5 antibody (anti-MDA5ab) titre and disease status in patients with anti-MDA5ab-positive DM.Methods. Twenty-seven patients who presented with DM and were positive for the anti-MDA5ab were enrolled. The association between the clinical manifestations and the clinical parameters, including the anti-MDA5ab, was analysed.Results. The complication of rapidly progressive interstitial lung disease (RP-ILD) occurred in 20 (74%) patients. The frequencies of fatal outcome, relapse and malignancy were 33, 4 and 4%, respectively. Remarkably, a fatal outcome occurred within the first 6 months. Compared with six non-RP-ILD patients, elderly age at onset, severely involved pulmonary function and high levels of serum ferritin were present in 20 RP-ILD patients with anti-MDA5ab. Alveolar-arterial oxygen difference (AaDO(2)) epsilon 32 mmHg and ferritin epsilon 828 ng/ml at admission were poor prognostic factors in RP-ILD patients with anti-MDA5ab-positive DM. The median value of the anti-MDA5ab titre on admission was higher in patients who later died than in those who survived. The efficacy of treatment was indicated by the anti-MDA5ab, ferritin and IL-18 concentrations. The decline index of the anti-MDA5ab titre after treatment was lower in the subset of patients who died than in the subset of patients who lived. Sustained high levels of anti-MDA5ab, ferritin and IL-18 were present in the patients who died.Conclusion. Anti-MDA5ab titre and ferritin and IL-18 concentrations are useful for the evaluation of the response to treatment and the status of ILD in patients with anti-MAD5ab-positive DM.