Lin Yihua, Yi Qun, Cheng Deyun
Department of Respiratory Medicine, West China Hospital of Sichuan University China ; Department of Respiratory Medicine, The First Affiliated Hospital of Xiamen University China.
Department of Respiratory Medicine, West China Hospital of Sichuan University China.
Int J Clin Exp Med. 2014 Dec 15;7(12):5904-8. eCollection 2014.
Primary Sjögren's syndrome is a chronic inflammatory disorder with many extraglandular organ systems involved, including the lungs. Diffuse interstitial lung disease is the most serious form of lung involvement. Parenchymal lung involvement in primary Sjögren's syndrome is usually manifested by cough and/or slowly progressive dyspnea and most of the cases present as chronic course. We describe here a case of primary Sjögren's syndrome who presented as rapid progressive interstitial lung disease. Improvement was obtained with treatment of corticosteroids and ventilatory support at early time. To the best of our knowledge, this is the first report documenting primary Sjögren's syndrome initially presenting as rapid progressive interstitial lung disease and it enriches our understanding of the clinical manifestations of primary Sjögren's syndrome.
原发性干燥综合征是一种慢性炎症性疾病，累及包括肺部在内的许多腺外器官系统。弥漫性间质性肺疾病是肺部受累最严重的形式。原发性干燥综合征的肺实质受累通常表现为咳嗽和/或缓慢进展的呼吸困难，大多数病例呈慢性病程。我们在此描述一例以快速进展性间质性肺疾病为表现的原发性干燥综合征病例。早期使用皮质类固醇治疗和通气支持后病情得到改善。据我们所知，这是第一例记录原发性干燥综合征最初表现为快速进展性间质性肺疾病的报告，它丰富了我们对原发性干燥综合征临床表现的认识。
