Pulmonary manifestations of Sjögren's syndrome.
Pulmonary manifestations of Sjögren's syndrome.
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DOI：
10.1183/16000617.0011-2016
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发表时间：
2016-06
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--
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中科院分区：
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在9-20%的病例中，干燥综合征与各种呼吸道症状有关。最典型的表现是慢性间质性肺病（ILD）和气管支气管疾病。ILD最常见的表现是纤维化变异的非特异性间质性肺炎。其他类型的ILD，如机化性肺炎、普通型间质性肺炎和淋巴细胞性间质性肺炎较为罕见。其影像学表现不太明显，明确诊断可能需要经支气管或手术肺活检。皮质类固醇治疗是干燥综合征ILD治疗的主要方法，但其他免疫抑制药物的使用需要确定。ILD是干燥综合征的重要死因。气管支气管疾病在干燥综合征中很常见，以气道弥漫性淋巴细胞浸润为特征。它有时会导致严重的慢性咳嗽。它也可以以支气管高反应性、支气管扩张、细支气管炎或反复呼吸道感染的形式存在。这些表现的管理可能需要治疗气道干燥和/或炎症。气道疾病对呼吸功能的影响很小，很少是干燥综合征患者的死亡原因。干燥综合征患者中不应忽视罕见的呼吸系统并发症，如淀粉样变性、淋巴瘤或肺动脉高压。我们介绍了干燥综合征胸部表现的特点、诊断试验和治疗。http://ow.ly/10m8vd
In 9–20% of cases, Sjögren's syndrome is associated with various respiratory symptoms. The most typical manifestations are chronic interstitial lung disease (ILD) and tracheobronchial disease. The most common manifestation of ILD is nonspecific interstitial pneumonia in its fibrosing variant. Other types of ILD, such as organising pneumonia, usual interstitial pneumonia and lymphocytic interstitial pneumonitis, are rare. Their radiological presentation is less distinctive, and definitive diagnosis may require the use of transbronchial or surgical lung biopsy. Corticosteroid therapy is the mainstay of ILD treatment in Sjögren's syndrome, but the use of other immunosuppressive drugs needs to be determined. ILD is a significant cause of death in Sjögren's syndrome. Tracheobronchial disease is common in Sjögren's syndrome, characterised by diffuse lymphocytic infiltration of the airway. It is sometimes responsible for a crippling chronic cough. It can also present in the form of bronchial hyperresponsiveness, bronchiectasis, bronchiolitis or recurrent respiratory infections. The management of these manifestations may require treatment for dryness and/or inflammation of the airways. Airway disease has little effect on respiratory function and is rarely the cause of death in Sjögren's syndrome patients. Rare respiratory complications such as amyloidosis, lymphoma or pulmonary hypertension should not be disregarded in Sjögren's syndrome patients. We present the features, diagnostic tests and treatments of thoracic manifestations of Sjögren's syndrome http://ow.ly/10m8vd
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