title: Rapid progressive interstitial lung disease as initial manifestation of primary Sjögren's syndrome: a case report.
abstract: 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.
authors: Department of Respiratory Medicine, West China Hospital of Sichuan University China ; Department of Respiratory Medicine, The First Affiliated Hospital of Xiamen University China.
authors: Yihua
authors: Y
authors: Lin
authors: Department of Respiratory Medicine, West China Hospital of Sichuan University China.
authors: Qun
authors: Q
authors: Yi
authors: Department of Respiratory Medicine, West China Hospital of Sichuan University China.
authors: Deyun
authors: D
authors: Cheng
keywords: Primary Sjögren’s syndrome; lymphocytic interstitial pneumonia; nonspecific interstitial pneumonia; rapid progressive interstitial lung disease