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Abstract：
BACKGROUND: IgG4-related disease is a systemic fibroinflammatory disease that is mainly seen in older men, and involves multiple organs, such as the pancreas and lungs. However, 75% of patients with IgG4-related lung disease are asymptomatic (if they are symptomatic, they mainly complain of nasal congestion, rhinorrhoea, chest pain, and cough) and are incidentally diagnosed through chest computed tomograph. Although, nodules in the airway and bronchial wall thickening are criteria for diagnosis, it is important that nodules have been reported in peripheral airways in several cases and rarely in the central airway.
METHODS: A 74-year-old woman previously diagnosed with Mikulicz\'s disease presented with swelling of the eyelid margin on both sides and visual disturbances. Computed tomography revealed extensive multiple nodules and mucosal oedema of the trachea and both bronchi. On flexible bronchoscopy under local anaesthesia, extensive lesions were observed from the middle of the trachea to the carina, extending into both segmental bronchi. The nodules were continuous with the normal respiratory tract mucosa, and the surfaces were smooth with minimal neovascularisation. Due to the solid nature of the lesion, obtaining an adequate amount of specimen was challenging. Therefore, we used a 1.9 mm cryoprobe under intubation, resulting in minimal bleeding. Subsequently, the patient was diagnosed with IgG4-related lung disease.
CONCLUSIONS: The present case is very rare because of the presence of multiple nodules, severe mucosal edema of the central airway and the absence of mediastinal lymphadenopathy, ground glass nodules, and lung masses. Therefore, it is important to consider differential diagnoses. Thus, we emphasise the importance of endobronchial cryobiopsy for obtaining an adequate number of tissue specimens in such cases to establish a definitive pathological diagnosis.
METHODS: A 74-year-old woman previously diagnosed with Mikulicz\'s disease presented with swelling of the eyelid margin on both sides and visual disturbances. Computed tomography revealed extensive multiple nodules and mucosal oedema of the trachea and both bronchi. On flexible bronchoscopy under local anaesthesia, extensive lesions were observed from the middle of the trachea to the carina, extending into both segmental bronchi. The nodules were continuous with the normal respiratory tract mucosa, and the surfaces were smooth with minimal neovascularisation. Due to the solid nature of the lesion, obtaining an adequate amount of specimen was challenging. Therefore, we used a 1.9 mm cryoprobe under intubation, resulting in minimal bleeding. Subsequently, the patient was diagnosed with IgG4-related lung disease.
CONCLUSIONS: The present case is very rare because of the presence of multiple nodules, severe mucosal edema of the central airway and the absence of mediastinal lymphadenopathy, ground glass nodules, and lung masses. Therefore, it is important to consider differential diagnoses. Thus, we emphasise the importance of endobronchial cryobiopsy for obtaining an adequate number of tissue specimens in such cases to establish a definitive pathological diagnosis.
摘要：
背景：IgG4相关疾病是一种系统性纤维炎症性疾病，主要见于老年男性，涉及多个器官，例如胰腺和肺。然而,75%的IgG4相关性肺病患者无症状（如果有症状，他们主要抱怨鼻塞，鼻漏,胸痛,和咳嗽），并通过胸部计算机断层扫描偶然诊断。虽然,气道结节和支气管壁增厚是诊断标准,重要的是，在一些病例中，在中央气道中很少有结节的报道。
方法：一名74岁女性，先前被诊断患有Mikulicz病，表现为两侧眼睑边缘肿胀和视力障碍。计算机断层扫描显示气管和支气管广泛多发结节和粘膜水肿。在局部麻醉下的柔性支气管镜检查中，从气管中部到隆突观察到广泛的病变，延伸到两段支气管。结节与正常呼吸道粘膜连续，表面光滑，新生血管形成最少。由于病变的实质性，获得足够的样本是具有挑战性的。因此,我们在插管下使用了1.9毫米的冷冻探头，导致最小的出血。随后，患者被诊断为IgG4相关肺部疾病.
结论：由于存在多个结节，本病例非常罕见，中央气道严重的粘膜水肿和纵隔淋巴结病的缺失，磨玻璃结节,和肺部肿块。因此,重要的是要考虑鉴别诊断.因此，我们强调支气管内冷冻活检对于在这种情况下获得足够数量的组织标本以建立明确的病理诊断的重要性。
方法：一名74岁女性，先前被诊断患有Mikulicz病，表现为两侧眼睑边缘肿胀和视力障碍。计算机断层扫描显示气管和支气管广泛多发结节和粘膜水肿。在局部麻醉下的柔性支气管镜检查中，从气管中部到隆突观察到广泛的病变，延伸到两段支气管。结节与正常呼吸道粘膜连续，表面光滑，新生血管形成最少。由于病变的实质性，获得足够的样本是具有挑战性的。因此,我们在插管下使用了1.9毫米的冷冻探头，导致最小的出血。随后，患者被诊断为IgG4相关肺部疾病.
结论：由于存在多个结节，本病例非常罕见，中央气道严重的粘膜水肿和纵隔淋巴结病的缺失，磨玻璃结节,和肺部肿块。因此,重要的是要考虑鉴别诊断.因此，我们强调支气管内冷冻活检对于在这种情况下获得足够数量的组织标本以建立明确的病理诊断的重要性。
