CASE REPORTS
Rapidly Progressive Pulmonary Fibrosis Following the Onset of Diffuse Alveolar Hemorrhage in Sjögren's Syndrome: An Autopsy Case Report
2012 Volume 51 Issue 3 Pages 295-299
Details
2012 Volume 51 Issue 3 Pages 295-299
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.
