Abstract

Lymphocytic interstitial pneumonia is a rare lymphoproliferative disease involving the lung. It commonly occurs in rheumatological conditions. Among them, primary Sjögren syndrome is the most common association. We report a rare case of lymphocytic interstitial pneumonia in a patient with primary Sjögren syndrome. A 65 years old previously healthy female initially presented to the respiratory clinic with a history of progressive exertional dyspnea without other respiratory symptoms, anemic symptoms or features of heart failure, diagnosed as a lymphocytic interstitial pneumonia and then one year later she develop dry eyes and dry mouth without evidence of other autoimmune disorder and found to have primary Sjögren syndrome. Initially she was treated with prednisolone for lymphocytic interstitial pneumonia then treated with hydroxychloroquine, artificial tears and chewing gum for Sjögren syndrome. Her respiratory symptoms and lung functions were improved with prednisolone. Her symptoms of dryness also improved. Although rare, lymphocytic interstitial pneumonia is a well-known association with primary Sjögren syndrome. It can be developed before the diagnosis of Sjögren syndrome as in this patient; therefore, screening for Sjögren syndrome and other rheumatological disorders is warranted in a patient with lymphocytic interstitial pneumonia. Steroid is the mainstay of treatment of lymphocytic interstitial pneumonia.