Ono Ryohei, Kumagae Tomohiro, Igasaki Mari, Murata Takaaki, Yoshizawa Masaki, Kitagawa Izumi
Department of General Internal Medicine, Shonan Kamakura General Hospital, 1370-1 Okamoto, Kamakura, Kanagawa, 247-8533, Japan.
Department of Surgery, Shonan Kamakura General Hospital, 1370-1 Okamoto, Kamakura, Kanagawa, 247-8533, Japan.
J Med Case Rep. 2021 Mar 22;15(1):142. doi: 10.1186/s13256-021-02664-1.
Anti-transcriptional intermediary factor 1 gamma (TIF1γ) antibody is a marker for predicting cancer association in patients with dermatomyositis (DM). The overall survival rate in DM patients with cancer was reported to be considerably worse than that in DM patients without cancer. However, the treatment for cancer-associated DM remains controversial, because the treatment priority between surgical resection for the tumor and internal treatments, including glucocorticoids, immunosuppressive agents, and intravenous immune globulin, has not been established.
We report the case of a 57-year-old Japanese man diagnosed with anti-TIF1γ antibody-positive DM associated with ascending colon cancer. His clinical symptoms included facial and brachial edema, muscle weakness, dysphagia, myalgia, and rash. Physical examination revealed periorbital edema and Gottron's papules over his knuckles with brachial edema, and tenderness and weakness of the proximal limb muscles. The findings of hyperintense muscles in T2-weighted sequences of brachial contrast-enhanced magnetic resonance imaging and the infiltration of lymphocytic cells and CD4-positive lymphocytes from muscle biopsy were compatible with the diagnostic criteria for dermatomyositis. Anti-TIF1γ antibody was positive by immunoprecipitation assay. He first started internal treatment including intravenous immunoglobulin, steroid pulse, prednisolone, and azathioprine, followed by surgical resection for the tumor because of the elevation of creatine kinase and progression of dysphagia. However, clinical symptoms did not improve, and the patient died 6 months later.
We faced difficulties in determining the treatment priority between surgical resection and internal treatment for our case; therefore, this case would be educational for readers. We searched PubMed to identify English-language case reports of anti-TIF1γ antibody-positive dermatomyositis with malignancy and found 21 reported cases. We herein review and summarize previously reported cases of anti-TIF1γ antibody-positive DM with malignancy. Cancer screening is essential in patients with anti-TIF1γ antibody-positive dermatomyositis because it is associated with a high prevalence of malignancies. Our review revealed that initial surgical treatment should be recommended for better prognosis if the general condition allows.
抗转录中介因子1γ(TIF1γ)抗体是预测皮肌炎(DM)患者癌症相关性的标志物。据报道,患有癌症的DM患者的总生存率远低于无癌症的DM患者。然而,癌症相关性DM的治疗仍存在争议,因为肿瘤手术切除与包括糖皮质激素、免疫抑制剂和静脉注射免疫球蛋白在内的内科治疗之间的治疗优先级尚未确定。
我们报告了一例57岁日本男性,诊断为抗TIF1γ抗体阳性的DM合并升结肠癌。他的临床症状包括面部和臂部水肿、肌肉无力、吞咽困难、肌痛和皮疹。体格检查发现眶周水肿、指关节处Gottron丘疹伴臂部水肿,以及近端肢体肌肉压痛和无力。肱部增强磁共振成像T2加权序列中肌肉高信号的表现以及肌肉活检中淋巴细胞和CD4阳性淋巴细胞的浸润符合皮肌炎的诊断标准。免疫沉淀试验显示抗TIF1γ抗体呈阳性。他首先开始内科治疗,包括静脉注射免疫球蛋白、类固醇冲击治疗、泼尼松龙和硫唑嘌呤,随后由于肌酸激酶升高和吞咽困难进展而进行肿瘤手术切除。然而,临床症状并未改善,患者6个月后死亡。
对于我们的病例,我们在确定手术切除和内科治疗的治疗优先级方面面临困难;因此,该病例对读者具有教育意义。我们检索了PubMed以确定抗TIF1γ抗体阳性皮肌炎合并恶性肿瘤的英文病例报告,共发现21例报告病例。我们在此回顾并总结先前报道的抗TIF1γ抗体阳性DM合并恶性肿瘤的病例。抗TIF1γ抗体阳性的皮肌炎患者进行癌症筛查至关重要,因为其与高恶性肿瘤患病率相关。我们的综述表明,如果患者一般状况允许,建议进行初始手术治疗以获得更好的预后。