title: Rapidly progressive pulmonary fibrosis following the onset of diffuse alveolar hemorrhage in Sjögren's syndrome: an autopsy case report.
abstract: We report an autopsy case of a patient with Sjögren's syndrome (SjS) who presented with rapid progressive pulmonary fibrosis following the onset of diffuse alveolar hemorrhage (DAH) without cryoglobulinemia. Despite early and aggressive immunosuppressive therapy, pulmonary fibrosis progressed and the patient succumbed to his illness. An autopsy was performed and revealed DAH and interstitial pneumonia with a fibrotic nonspecific interstitial pneumonia pattern. We could not find any previously-reported underlying causes of DAH. The findings from this case suggest that DAH can occur as a pulmonary manifestation of SjS as well as other connective tissue diseases or vasculitis.
authors.affiliation: Department of Respiratory Medicine, Kumamoto City Hospital, Japan. y-tomita@space.ocn.ne.jp
authors.forename: Yusuke
authors.initials: Y
authors.lastname: Tomita
authors.forename: Shunsuke
authors.initials: S
authors.lastname: Mori
authors.forename: Nobuyuki
authors.initials: N
authors.lastname: Arima
authors.forename: Koichiro
authors.initials: K
authors.lastname: Fukuda
authors.forename: Hirotsugu
authors.initials: H
authors.lastname: Kohrogi
mesh_terms: D000368:Aged; D001344:Autopsy; D018450:Disease Progression; D006470:Hemorrhage / Q000150:complications / Q000473:pathology*; D006801:Humans; D008297:Male; D011650:Pulmonary Alveoli / Q000473:pathology*; D011658:Pulmonary Fibrosis / Q000209:etiology / Q000473:pathology*; D012859:Sjogren's Syndrome / Q000150:complications / Q000473:pathology*; D013997:Time Factors