Rapidly Progressive Pulmonary Fibrosis Following the Onset of Diffuse Alveolar Hemorrhage in Sjoegren's Syndrome: An Autopsy Case Report

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概要

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• 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.

著者

• Mori Shunsuke

  Clinical Research Center for Rheumatic Disease and Department of Rheumatology, Kumamoto Saishunsou National Hospital, Japan

• Kohrogi Hirotsugu

  Department of Respiratory Medicine, Graduate School of Medical Sciences, Kumamoto University, Japan

関連論文

• Rapidly Progressive Pulmonary Fibrosis Following the Onset of Diffuse Alveolar Hemorrhage in Sjoegren's Syndrome: An Autopsy Case Report

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