• Title/Summary/Keyword: Ball-valve phenomenon

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A Case of Sj$\ddot{o}$gren's Syndrome with Interstitial Lung Disease and Multiple Cystic Lung Disease (간질성 및 다낭성 폐질환이 동반된 쇼그렌 증후군 1례)

  • Jang, Dai-Yong;Shin, Byung-Chul;Jung, Ki-Young;Kim, Jong-O;Yang, Jong-Tae;Joo, Yoo-Chul;Lee, Seung-Il
    • Tuberculosis and Respiratory Diseases
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    • v.51 no.6
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    • pp.597-602
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    • 2001
  • 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 Sj$\ddot{o}$gren'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. Sj$\ddot{o}$gren'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 Sj$\ddot{o}$gren's syndrome. Although a lung biopsy is not always necessary for establishing a diagnosis of an interstitial lung disease in Sj$\ddot{o}$gren'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 ball-valve phenomen. However, no definite evidence was obtained.

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