Department of Rheumatology, Tohoku University Hospital Sendai, 1-1 Seiryo-Machi, Aoba-ku, Sendai, Miyagi, 980-8574, Japan.
Rheumatol Int. 2024 Sep;44(9):1781-1788. doi: 10.1007/s00296-023-05442-y. Epub 2023 Sep 8.
Dermatomyositis (DM) is associated with interstitial lung disease (ILD) and malignancy. However, the coexistence of ILD and malignancy (DM-ILD-malignancy) is rare, and limited information exists regarding its management. Herein, we report the case of a 70-year-old man who developed DM with rapidly progressive ILD and advanced gastric cancer and provide a literature review of managing DM-ILD-malignancy. The patient presented with typical DM skin rashes and shortness of breath, which worsened within 1 month, without muscular symptoms. Additionally, the patient tested negative for myositis-specific autoantibodies (MSAs). Computed tomography revealed ILD and advanced gastric cancer, which was confirmed on endoscopic examination to be a poorly differentiated adenocarcinoma. Although the patient's ILD progressed rapidly, surgical treatment of the cancer was prioritized. Prednisolone (PSL) 0.5 mg/kg was initiated 3 days before surgery and increased to 1 mg/kg at 7 days postoperative. Remarkable improvement in the skin rash and ILD was observed, and the PSL dose was tapered without immunosuppressants. A literature review revealed that anti-melanoma differentiation-associated gene 5 and anti-aminoacyl transfer RNA synthetase antibodies are the predominant MSAs in DM-ILD-malignancy, and the optimal treatment should be determined based on several factors, including ILD patterns, and malignancy type and stage. In particular, lung cancer may be a risk factor for the acute exacerbation of ILD, and preceding immunosuppression would be useful. Furthermore, prioritizing surgery for gastric cancer is effective because of its paraneoplastic nature.
皮肌炎(DM)与间质性肺病(ILD)和恶性肿瘤相关。然而,ILD 和恶性肿瘤同时存在(DM-ILD-恶性肿瘤)的情况较为罕见,关于其管理的信息有限。在此,我们报告一例 70 岁男性,患有快速进展性ILD 和晚期胃癌的 DM,并对 DM-ILD-恶性肿瘤的治疗进行了文献复习。患者表现为典型的 DM 皮疹和呼吸困难,在 1 个月内病情加重,无肌肉症状。此外,患者肌炎特异性自身抗体(MSAs)检测为阴性。计算机断层扫描显示ILD 和晚期胃癌,内镜检查证实为低分化腺癌。尽管患者的ILD 迅速进展,但优先考虑癌症的手术治疗。在手术前 3 天开始给予泼尼松龙(PSL)0.5mg/kg,并在术后 7 天增加至 1mg/kg。皮疹和ILD 显著改善,未使用免疫抑制剂逐渐减少 PSL 剂量。文献复习显示,抗黑色素瘤分化相关基因 5 和抗氨酰基转移 RNA 合成酶抗体是 DM-ILD-恶性肿瘤中主要的 MSAs,最佳治疗应根据ILD 模式和恶性肿瘤类型和分期等多种因素确定。特别是肺癌可能是ILD 急性加重的危险因素,先前的免疫抑制可能会有所帮助。此外,由于胃癌具有副瘤性质,优先进行手术治疗是有效的。