Usual interstitial pneumonia associated with primary Sjögren's syndrome.

Abstract

We describe the first case, to our knowledge, of usual interstitial pneumonia (UIP) as the pulmonary manifestation in primary Sjögren's syndrome (SjS). A 45-year-old woman was admitted to our hospital because of a dry cough and an interstitial shadow on a chest roentgenogram. Labial biopsy and sialogram confirmed a diagnosis of SjS. BAL fluid analysis revealed lymphocytosis with a decreased CD4/CD8 ratio compatible with bronchiolitis obliterans organizing pneumonia or lymphoid interstitial pneumonia. Open-lung biopsy specimen, however, showed evidence of UIP. Open-lung biopsy was a useful and necessary examination to determine the nature of the pulmonary complication in primary SjS. Conservative treatment without corticosteroids maintained a stable condition for a follow-up period of 3 years.