Fukui Saeko, Kobayashi Kazuma, Fujita Yuya, Fukui Shoichi, Iwamoto Naoki, Adachi Tomohiko, Hidaka Masaaki, Takatsuki Mitsuhisa, Abe Kuniko, Kuwana Masataka, Kawakami Atsushi, Eguchi Susumu
Department of Surgery, Nagasaki University Graduate School of Biomedical Science, Japan.
Department of Immunology and Rheumatology, Nagasaki University Hospital, Japan.
Intern Med. 2020 Aug 15;59(16):2071-2076. doi: 10.2169/internalmedicine.3923-19. Epub 2020 May 23.
A 74-year-old man with interstitial lung disease (ILD) underwent surgical excision of a growing retroperitoneal tumor and was diagnosed with spindle cell sarcoma. Just after the surgery, skin eruption and muscle weakness emerged. Based on his symptoms and examination findings, we diagnosed him with anti-synthetase syndrome (ASS) with positive anti-glycyl-transfer ribonucleic acid synthetase antibody (anti-EJ) as paraneoplastic syndrome. Immunosuppressive treatments kept his progressing ILD stable for 21 months, although an expanding lung metastatic lesion from primary sarcoma was detected. Measurements of myositis-specific antibodies may enable the prediction of the efficacy of immunosuppressive treatments for paraneoplastic syndrome, even if the primary disease becomes progressive.
一名患有间质性肺病（ILD）的74岁男性接受了生长性腹膜后肿瘤的手术切除，被诊断为梭形细胞肉瘤。术后不久，出现了皮疹和肌肉无力。根据他的症状和检查结果，我们将他诊断为伴有抗甘氨酰转移核糖核酸合成酶抗体（抗EJ）阳性的抗合成酶综合征（ASS），作为副肿瘤综合征。免疫抑制治疗使他进展性的ILD稳定了21个月，尽管检测到原发性肉瘤出现了肺部转移灶扩大。即使原发性疾病进展，检测肌炎特异性抗体可能有助于预测免疫抑制治疗对副肿瘤综合征的疗效。
