PDF(Pubmed)
Abstract：
BACKGROUND: Interstitial lung disease (ILD) is a frequently observed comorbidity in autoimmune diseases such as dermatomyositis/polymyositis (DM/PM), and it is significantly associated with specific autoantibody types. One unique antibody type is the anti-transcription intermediate factor-1γ antibody (anti-TIF-1γ Ab), which has a positive rate of only 7%. It is often found in combination with malignancy and rarely with ILD, particularly rapidly progressive ILD (RPILD). In some cases, the presence of ILD in individuals with DM may indicate a paraneoplastic syndrome. Pneumocystis jiroveci pneumonia (PJP) typically occurs due to intensive immunosuppressive therapy, human immunodeficiency virus (HIV) infection, or malignancy, and rarely as an isolated condition.
METHODS: A 52-year-old man with a history of rapid weight loss but non-HIV infected and not immunosuppressed who presented with fever, cough, dyspnea, weakness of the extremities, characteristic rash and mechanic\'s hand. Pathogenic tests suggested PJP, laboratory tests suggested a single anti-TIF-1γ Ab positive DM, imaging suggested ILD, and pathology revealed no malignancy. RPILD and acute respiratory distress syndrome (ARDS) developed after anti-infection and steroid hormone therapy. After mechanical support therapy such as Extracorporeal Membrane Oxygenation (ECMO), the patient developed late-onset cytomegalovirus pneumonia (CMVP), complicated bacterial infection, and ultimately death. Additionally, we discuss the potential causes of rapid weight loss, the mechanisms by which anti-TIF-1γ Ab may lead to ILD, and the possible connection between anti-TIF-1γ Ab positivity, rapid weight loss, immune abnormalities, and opportunistic infections.
CONCLUSIONS: This case emphasizes the importance of early recognition of malignant tumors and pulmonary lesions, assessment of the body\'s immune status, prompt initiation of immunosuppressive treatment, and prevention of opportunistic infections in individuals with single anti-TIF-1γ Ab positive DM presenting with rapid weight loss.
METHODS: A 52-year-old man with a history of rapid weight loss but non-HIV infected and not immunosuppressed who presented with fever, cough, dyspnea, weakness of the extremities, characteristic rash and mechanic\'s hand. Pathogenic tests suggested PJP, laboratory tests suggested a single anti-TIF-1γ Ab positive DM, imaging suggested ILD, and pathology revealed no malignancy. RPILD and acute respiratory distress syndrome (ARDS) developed after anti-infection and steroid hormone therapy. After mechanical support therapy such as Extracorporeal Membrane Oxygenation (ECMO), the patient developed late-onset cytomegalovirus pneumonia (CMVP), complicated bacterial infection, and ultimately death. Additionally, we discuss the potential causes of rapid weight loss, the mechanisms by which anti-TIF-1γ Ab may lead to ILD, and the possible connection between anti-TIF-1γ Ab positivity, rapid weight loss, immune abnormalities, and opportunistic infections.
CONCLUSIONS: This case emphasizes the importance of early recognition of malignant tumors and pulmonary lesions, assessment of the body\'s immune status, prompt initiation of immunosuppressive treatment, and prevention of opportunistic infections in individuals with single anti-TIF-1γ Ab positive DM presenting with rapid weight loss.
摘要：
背景：间质性肺病（ILD）是在自身免疫性疾病如皮肌炎/多发性肌炎（DM/PM）中经常观察到的合并症，它与特定的自身抗体类型显著相关。一种独特的抗体类型是抗转录中间因子-1γ抗体（抗TIF-1γAb），阳性率仅为7%。它通常与恶性肿瘤合并，很少与ILD合并。特别是快速进行性ILD（RPILD）。在某些情况下,DM患者中ILD的存在可能提示副肿瘤综合征.肺孢子虫肺炎（PJP）通常是由于强化免疫抑制治疗而发生的。人类免疫缺陷病毒（HIV）感染，或者恶性肿瘤，很少是孤立的。
方法：一名52岁男子，有快速减肥史，但未感染HIV且未受到免疫抑制，出现发热，咳嗽,呼吸困难,四肢的弱点，特征性皮疹和机械师的手。致病试验建议PJP，实验室测试表明单一抗TIF-1γAb阳性DM，成像建议ILD，病理显示无恶性肿瘤。抗感染和类固醇激素治疗后出现RPILD和急性呼吸窘迫综合征（ARDS）。在体外膜氧合（ECMO）等机械支持治疗后，患者出现迟发性巨细胞病毒肺炎（CMVP），复杂的细菌感染，最终死亡。此外,我们讨论了快速减肥的潜在原因，抗TIF-1γAb可能导致ILD的机制，以及抗TIF-1γAb阳性之间的可能联系，快速减肥，免疫异常，和机会性感染。
结论：本案例强调了早期识别恶性肿瘤和肺部病变的重要性，评估身体的免疫状态，立即开始免疫抑制治疗，和预防单一抗TIF-1γAb阳性DM个体的机会性感染，表现为快速体重减轻。
方法：一名52岁男子，有快速减肥史，但未感染HIV且未受到免疫抑制，出现发热，咳嗽,呼吸困难,四肢的弱点，特征性皮疹和机械师的手。致病试验建议PJP，实验室测试表明单一抗TIF-1γAb阳性DM，成像建议ILD，病理显示无恶性肿瘤。抗感染和类固醇激素治疗后出现RPILD和急性呼吸窘迫综合征（ARDS）。在体外膜氧合（ECMO）等机械支持治疗后，患者出现迟发性巨细胞病毒肺炎（CMVP），复杂的细菌感染，最终死亡。此外,我们讨论了快速减肥的潜在原因，抗TIF-1γAb可能导致ILD的机制，以及抗TIF-1γAb阳性之间的可能联系，快速减肥，免疫异常，和机会性感染。
结论：本案例强调了早期识别恶性肿瘤和肺部病变的重要性，评估身体的免疫状态，立即开始免疫抑制治疗，和预防单一抗TIF-1γAb阳性DM个体的机会性感染，表现为快速体重减轻。
