Abstract
BackgroundThe pathophysiologic mechanisms underlying acute exacerbation of idiopathic pulmonary fibrosis (IPF) are not fully understood. Few studies have examined autopsy findings in patients who have died from an acute exacerbation of IPF. The pathologic findings in systemic organs have not been described.MethodsWe retrospectively reviewed the autopsy findings in 12 patients who had died from an acute exacerbation of IPF and two of connective tissue disease- associated interstitial lung disease between 2005 and 2015. We recorded demographic and clinical characteristics, autopsy findings and cytologic findings in peripheral blood.ResultsThe median age at autopsy was 68 years (range 45–87 years); 11 subjects (78.5 %) were men. High-dose corticosteroid, cyclophosphamide and oxygen therapy had been administered to all patients. Underlying lesions had the usual interstitial pneumonia pattern; diffuse alveolar damage and contraction band necrosis were observed in all cases. Large cells expressing scavenger receptor A (SRA+) had been observed in the systemic circulation of 11 of the 14 cases (78.6 %) before acute exacerbation, and cells expressing tumor necrosis factor-α (TNF-α+) were detected after its diagnosis in nine (64.3 %). Both were detected in all cases at autopsy. There was neutrophil and platelet accumulation predominantly in capillaries, and extensive capillary endothelial cells injury.ConclusionsOur findings suggest that acute exacerbation of IPF has systemic consequences with multiple organ injury, with SRA+ and TNF-α+ cells in the systemic circulation playing central roles in multiple organ injury.
Highlights
The pathophysiologic mechanisms underlying acute exacerbation of idiopathic pulmonary fibrosis (IPF) are not fully understood
We have previously reported that cells expressing scavenger receptor A (SRA) and tumor necrosis factor-α (TNF-α) can be identified at autopsy in the systemic circulation of patients who had died with multiple organ dysfunction syndrome
Large SRA+ cells were observed only in patients who had died of multiple organ dysfunction syndrome, while small SRA+ cells were observed in all healthy control. These findings suggest that large SRA+ and Tumor necrosis factor (TNF)-α+ cells play important roles in the development of multiple organ injury [15]
Summary
The pathophysiologic mechanisms underlying acute exacerbation of idiopathic pulmonary fibrosis (IPF) are not fully understood. Many patients with idiopathic pulmonary fibrosis (IPF) may experience sudden worsening of respiratory symptoms [1] This frequently occurs without an identifiable cause, and is termed acute exacerbation of IPF [2]. Histopathologic evidence of diffuse alveolar damage (DAD) is frequently observed in patients who have died from an acute exacerbation of IPF [5, 8, 9]. This finding is shared with the acute respiratory distress syndrome (ARDS) [10] which is considered to be a systemic multiorgan disease [11,12,13]. The existing pathologic evidence base in IPF and CTD-associated ILD do not include an assessment of the peripheral blood or
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