COVID-19诱发胸腺瘤相关多器官自身免疫的首例报告。

Rie Hyobu, Miho Mori, Tatsuo Maeda, Kazuki Fujimori, Yukako Shimai, Makiko Naito, Masayuki Masuda, Tatsuo Ohira, Norihiko Ikeda, Yukari Okubo, Kazutoshi Harada
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摘要

胸腺瘤相关多器官自身免疫(TAMA)表现为与移植物抗宿主病(GVHD)、肝功能障碍和肠炎相似的皮肤症状,但无造血干细胞或骨髓移植史。TAMA是一种与胸腺瘤相关的副肿瘤综合征。其病因尚不清楚,但被认为是免疫耐受破坏的结果。从组织病理学角度看,TAMA 的特征是表皮棘化,伴有角化不全、单个细胞角化、液化变性和 CD8 阳性淋巴细胞的表皮内浸润。一名 64 岁的女性患者曾患肌无力和胸腺瘤,在接受泼尼松龙(10 毫克/天)和环孢素(150 毫克/天)治疗后,躯干在冠状病毒病 2019(COVID-19)发病后出现红斑。怀疑是银屑病药物疹,停用了可能的致病药物,但皮疹未能改善。皮肤活检显示有类似GVHD的组织病理学结果。腹泻、腹痛和十二指肠穿孔同时出现,诊断为 TAMA。此后,患者继续使用与治疗 TAMA 相同剂量的泼尼松龙和环孢素,并增加了外用类固醇。在病程中,出现了念珠菌真菌血症和巨细胞病毒感染,导致患者死亡。TAMA被认为是由COVID-19诱导的炎性细胞因子释放、自反应性T细胞活化和调节性T细胞功能障碍引起的。
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First reported case of thymoma-associated multiorgan autoimmunity induced by COVID-19.

Thymoma-associated multiorgan autoimmunity (TAMA) presents with skin symptoms similar to those of graft-versus-host disease (GVHD), liver dysfunction, and enteritis, in the absence of a history of hematopoietic stem cell or bone marrow transplantation. TAMA is a type of paraneoplastic syndrome associated with thymoma. Its etiology is unclear but is thought to be a result of breakdown of immune tolerance. Histopathologically, TAMA is characterized by epidermal acanthosis with parakeratosis, individual cell keratinization, liquefaction degeneration, and intraepidermal infiltration of CD8-positive lymphocytes. A 64-year-old female patient with a history of myasthenia gravis and thymoma treated with prednisolone (10 mg/day) and cyclosporine (150 mg/day) experienced erythema on her trunk after coronavirus disease 2019 (COVID-19) onset. A psoriatic drug eruption was suspected and the possible causative drug was discontinued, but the skin rash failed to improve. A skin biopsy demonstrated GVHD-like histopathological findings. Diarrhea, abdominal pain, and duodenal perforation occurred concurrently, leading to the diagnosis of TAMA. Thereafter, the patient continued prednisolone and cyclosporine in the same doses as the TAMA treatment and added topical steroids. During the disease course, candida fungemia and cytomegalovirus infection developed, resulting in the patient's death. The TAMA was considered to have been caused by the release of inflammatory cytokines, autoreactive T cell activation, and regulatory T cell dysfunction induced by COVID-19.

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