Abstract
Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is an acute respiratory worsening of unidentifiable cause that sometimes develops during the clinical course of IPF. Although the incidence of AE-IPF is not high, prognosis is poor. The pathogenesis of AE-IPF is not well understood; however, evidence suggests that coagulation abnormalities and inflammation are involved. Thrombomodulin is a transmembranous glycoprotein found on the cell surface of vascular endothelial cells. Thrombomodulin combines with thrombin, regulates coagulation/fibrinolysis balance, and has a pivotal role in suppressing excess inflammation through its inhibition of high-mobility group box 1 protein and the complement system. Thus, thrombomodulin might be effective in the treatment of AE-IPF, and we and other groups found that recombinant human soluble thrombomodulin improved survival in patients with AE-IPF. This review summarizes the existing evidence and considers the therapeutic role of thrombomodulin in AE-IPF.
Highlights
Idiopathic pulmonary fibrosis (IPF) is a representative subtype of idiopathic interstitial pneumonia.IPF is characterized histologically by a usual interstitial pneumonia (UIP) pattern and radiologically by the presence of typical honeycombing in high-resolution computed tomography images [1]
Maruyama and colleagues reported that itreceptor-3 specificallypathway binds High-mobility group box-1 (HMGB-1), thereby suppressing findings indicate the epidermal growth factor (EGF)-like domain of thrombomodulin exerts anticoagulant, inflammatory signal that by inhibiting
RhTM treatment significantly decreased lung inflammation and fibrosis induced by BLM instillation in mice in vivo. These results indicate that thrombomodulin has multiple effects on suppressing excess coagulation, inflammation, and lung fibrosis in vivo and may be a promising treatment for acute exacerbation of IPF (AE-IPF)
Summary
Idiopathic pulmonary fibrosis (IPF) is a representative subtype of idiopathic interstitial pneumonia. Plasma exchange and rituximab treatment improved gas exchange in 9 of 11 patients, and 2-month survival rate was comparable to that of a historical control group. Because these studies were retrospective and had small sample sizes, the effectiveness of these treatments remains unproven. Lung transplantation is another potential therapy for AE-IPF. IPF patients who transplanted lung during AE-IPF had significantly worse short-term and long-term survival compared to stable patients after lung transplantation It might be beneficial in some part of the patients, given the scarcity of available organs for transplantation, it appeared to be not suitable for standard treatment of AE-IPF. We discuss the pathogenesis of AE-IPF, thrombomodulin function, and the potential therapeutic role of thrombomodulin against AE-IPF
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