Department of Dermatology, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, 920-8641, Japan.
Medical and Biological Laboratories Co., Ltd, Nagoya, 460-0008, Japan.
Br J Dermatol. 2017 Feb;176(2):395-402. doi: 10.1111/bjd.14882. Epub 2017 Jan 19.
Antimelanoma differentiation-associated protein (anti-MDA)5 antibodies are associated with rapidly progressive interstitial lung disease (RP-ILD) in patients with clinically amyopathic dermatomyositis (CADM) or dermatomyositis (DM).
We aimed to evaluate the relevance of monitoring anti-MDA5 antibody levels for the management of RP-ILD in patients with CADM or DM.
Twelve patients with CADM (n = 10) or DM (n = 2) accompanied by RP-ILD were included. Baseline characteristics and outcomes were recorded. Serial measurements of anti-MDA5 antibody levels were measured. All patients were treated with corticosteroids, tacrolimus and intravenous cyclophosphamide.
All patients achieved RP-ILD remission after combined immunosuppressive therapy for a mean of 6·8 months, with significant decreases noted in the mean anti-MDA5 antibody levels at remission. Six (50%) patients became anti-MDA5 antibody negative after therapy. After a mean follow-up of 31 months, RP-ILD relapse was observed in four (33%) patients in both the anti-MDA5 antibody sustained positive group and the negative conversion group. However, relapsed patients in the sustained positive group relapsed earlier than those in the negative conversion group. Thus, a decrease in anti-MDA5 antibody levels during remission was associated with longer remission. Relapses were associated with a reincrease of anti-MDA5 antibody levels in four of four (100%) patients. In contrast, none of the patients without reincrease in anti-MDA5 antibody exhibited symptoms of relapse during follow-up. Therefore, reincrease in anti-MDA5 antibody levels was associated with relapse.
The anti-MDA5 antibody level is a novel parameter for monitoring and a good predictor of RP-ILD relapse in patients with CADM or DM.
抗黑色素瘤分化相关蛋白（anti-MDA）5 抗体与临床无肌病性皮肌炎（CADM）或皮肌炎（DM）患者快速进展性间质性肺病（RP-ILD）相关。
我们旨在评估监测 anti-MDA5 抗体水平对 CADM 或 DM 患者 RP-ILD 管理的相关性。
纳入 12 例伴有 RP-ILD 的 CADM（n=10）或 DM（n=2）患者。记录基线特征和结局。连续测量 anti-MDA5 抗体水平。所有患者均接受皮质类固醇、他克莫司和静脉注射环磷酰胺治疗。
所有患者经联合免疫抑制治疗平均 6.8 个月后均达到 RP-ILD 缓解，缓解时平均 anti-MDA5 抗体水平显著下降。治疗后 6（50%）例患者转为 anti-MDA5 抗体阴性。平均随访 31 个月后，anti-MDA5 抗体持续阳性组和阴性转换组各有 4（33%）例患者出现 RP-ILD 复发。然而，持续阳性组的复发患者比阴性转换组更早复发。因此，缓解期 anti-MDA5 抗体水平下降与缓解时间延长相关。4 例（100%）复发患者的 anti-MDA5 抗体水平再次升高。相比之下，在随访期间，没有任何 anti-MDA5 抗体水平未再次升高的患者出现复发症状。因此，anti-MDA5 抗体水平再次升高与复发相关。
anti-MDA5 抗体水平是监测 CADM 或 DM 患者 RP-ILD 复发的一个新参数，是 RP-ILD 复发的良好预测指标。
