Unilateral acute exacerbation of pulmonary fibrosis in association with Sjögren's syndrome.

A 70-year-old female had an abnormal chest roentgenogram. Infiltrative shadows were recognized in the right lung, and an open lung biopsy (OLB) specimen revealed usual interstitial pneumonia (UIP). Xerostomia, keratoconjunctivitis sicca, and lymphocyte infiltration in salivary glands were consistent with Sjögren's syndrome; she was diagnosed as having pulmonary fibrosis in association with Sjögren's syndrome (SjS-IP). Acute exacerbation occurred and she was successfully treated with corticosteroids. Unilateral exacerbation and UIP in SjS-IP are rare. OLB is useful for diagnosis and to select a pertinent therapy if lung involvement is unilateral in Sjögren's syndrome.