Department of Respiratory Medicine and Allergology, Sapporo Medical University School of Medicine, minami1-nishi16Chuo-Ku, Sapporo, Hokkaido, 060-8543, Japan.
Department of Respiratory Medicine, Steel Memorial Muroran Hospital, 1-45 Chiribetsucho, Muroran, Hokkaido, 050-0076, Japan.
J Med Case Rep. 2024 Sep 28;18(1):471. doi: 10.1186/s13256-024-04793-9.
Clinical amyopathic dermatomyositis is characterized by cutaneous symptoms but lacks muscle symptoms. Anti-melanoma differentiation-associated gene 5 antibodies are frequently found in Japanese patients with clinical amyopathic dermatomyositis. Patients with rapidly progressive interstitial lung disease with positive anti-melanoma differentiation-associated gene 5 antibodies have poor prognoses, and majority of them are treated with combination immunosuppressive therapy; however, the best treatment is yet to be determined.
A 52-year-old Asian male patient presented with a chief complaint of dyspnea on exertion. He had a typical skin rash and rapidly progressive interstitial pneumonia. Additionally, anti-melanoma differentiation-associated gene 5 antibodies were detected; therefore, he was diagnosed with dermatomyositis-associated interstitial pneumonia. Respiratory failure worsened despite administering steroid pulse therapy, tacrolimus, and cyclophosphamide. Consequently, plasma exchange was performed on day 13 of admission. After a slight improvement, the patient's respiratory failure worsened. Thus, cyclophosphamide was replaced by tofacitinib on day 28. Although respiratory failure improved and the progression of interstitial pneumonia seemed under control, βD-glucan level increased and Aspergillus antigen was detected on day 49. Micafungin and voriconazole were administered, but the patient succumbed to worsening respiratory failure on day 61. The pathological autopsy revealed multiple nodular lesions with cavity formation in both lungs and the presence of Aspergillus with severe neutrophilic infiltration and necrosis, which supported the diagnosis of invasive pulmonary aspergillosis.
The patient with anti-melanoma differentiation-associated gene 5 antibody-related rapidly progressive interstitial lung disease, whose disease was difficult to control after the administration of triple immunosuppressive therapy (steroids, tacrolimus, and cyclophosphamide), showed good response with tofacitinib. Unfortunately, the patient died of invasive pulmonary aspergillosis owing to severe immunosuppression; thus, the signs of complications should be promptly detected.
临床无肌病性皮肌炎的特征是皮肤症状，但缺乏肌肉症状。抗黑色素瘤分化相关基因 5 抗体在日本临床无肌病性皮肌炎患者中经常发现。具有阳性抗黑色素瘤分化相关基因 5 抗体的快速进展性间质性肺病患者预后较差，大多数患者接受联合免疫抑制治疗；然而，最佳治疗方法仍有待确定。
一名 52 岁亚洲男性患者以活动后呼吸困难为主诉就诊。他有典型的皮疹和快速进展性间质性肺炎。此外，检测到抗黑色素瘤分化相关基因 5 抗体；因此，他被诊断为皮肌炎相关间质性肺炎。尽管给予了类固醇脉冲治疗、他克莫司和环磷酰胺，但呼吸衰竭仍在恶化。因此，在入院第 13 天进行了血浆置换。在稍有改善后，患者的呼吸衰竭恶化。因此，在入院第 28 天将环磷酰胺替换为托法替尼。尽管呼吸衰竭得到改善，间质性肺炎的进展似乎得到控制，但β-D-葡聚糖水平升高，第 49 天检测到曲霉菌抗原。给予米卡芬净和伏立康唑，但患者在第 61 天因呼吸衰竭恶化而死亡。病理尸检显示双肺多个结节状病变伴空洞形成，存在曲霉菌，严重中性粒细胞浸润和坏死，支持侵袭性肺曲霉病的诊断。
该患者具有抗黑色素瘤分化相关基因 5 抗体相关的快速进展性间质性肺病，在接受三联免疫抑制治疗（类固醇、他克莫司和环磷酰胺）后疾病难以控制，使用托法替尼后反应良好。不幸的是，由于严重的免疫抑制，患者死于侵袭性肺曲霉病；因此，应及时发现并发症的迹象。
