A Case of Sjogren's Syndrome with Interstitial Lung Disease and Multiple Cystic Lung Disease

Jang, Dai Yong; Shin, Byung Chul; Jung, Ki Young; Kim, Jong O; Yang, Jong Tae; Joo, Yoo Chul; Lee, Seung Il

Abstract

A 47-years-old woman presented with a 2-month history of a dry mouth and dry cough. The patient had been taking medication for Sjogren's syndrome for approximately 7 years. The chest radiography showed multiple cystic lesions and a hazy density in both lower lung fields. The HRCT showed a diffuse ground glass like appearance and multiple variable sized cystic lesions in both lung fields. After medication, the symptoms were aggravated. Bronchoscopy was preformed with a transbronchial lung biopsy. The biopsies showed an infiltration of lymphocytes, neutrophils, monocytes and histiocytes through the interstitial space of the alveola and a widening of the alveolar septa. However, the histological findings of the cysts were not obtained. Sjogren's syndrome is a slowly progressive inflammatory autoimmune disease, which is characterized by lymphocyte mediated destruction of the exocrine glands, with pulmonary involvement in approximately 19-65%, High-resolution CT is a sensitive technique for assessing the pulmonary involvement in patients with Sjogren's syndrome. Although a lung biopsy is not always necessary for establishing a diagnosis of an interstitial lung disease in Sjogren's syndrome. A lung biopsy may reveal a wide spectrum of changes ranging from a mild inflammatory response to end stage fibrosis with honeycombing. Because of the predominantly peribronchiolar inflammatory infiltration and inspissated secretions the cysts were suspected to have been formed by the ballvalve phenomenon. However, no definite evidence was obtained.