Survival from acute exacerbation of idiopathic pulmonary fibrosis with or without direct hemoperfusion with polymyxin B-immobilized fiber column: A retrospective analysis

Abstract

Objective Acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF) are fatal episodes of acute respiratory worsening of unknown etiology. Previous studies on acute respiratory distress syndrome have shown that direct hemoperfusion with a polymyxin B-immobilized fiber column (PMX-DHP) can have a beneficial effect on the respiratory status. This retrospective study investigated the prognosis and survival outcome of patients with AE-IPF who underwent PMX-DHP. Methods We examined the records of 50 patients with AE-IPF treated in our hospital. All patients received corticosteroid pulse therapy. We compared the disease outcome between 27 patients who underwent PMX-DHP (PMX group) and 23 patients who did not (non-PMX group). The independent predictors of survival were determined using Cox proportional hazards analyses. Results A multivariate analysis of all patients revealed that PMX-DHP therapy was a significant predictor of survival (HR=0.442, 95% CI 0.223-0.873; p=0.019). The 12-month survival rate was significantly higher in the PMX group than in the non-PMX group (41.7% vs. 9.8%; p=0.040). According to a subanalysis of the PMX group, the time from AE-IPF onset to PMX-DHP was a significant predictor of survival (HR=1.080, 95% CI 1.001-1.166; p=0.049). Conclusion PMX-DHP improved the prognosis of AE-IPF. The time from AE-IPF onset to PMX-DHP may therefore be informative for predicting the patient outcome.

Prognostic significance of Glasgow prognostic score in patients with acute exacerbation of idiopathic pulmonary fibrosis

“…In previous clinical studies, CRP levels were a significant prognostic factor of IPF-AE, suggesting that inflammation may be involved in the pathogenesis of AE. 9,10,38 Lower levels of serum albumin, a negative acute-phase protein and marker of inflammation, were strongly and independently associated with higher mortality in patients with IPF on transplant waiting lists. 8 Factors known to be associated with poor prognosis in IPF-AE are combined diffuse and multifocal patterns rather than peripheral patterns on high- DLco, diffusing capacity of the lung for carbon monoxide; FEV 1 , forced expiratory volume in 1 s ; FVC, forced vital capacity; GPS, Glasgow prognostic score; LDH, lactate dehydrogenase; P/F, partial pressure of oxygen/fraction of inspired oxygen; PFT, pulmonary function test; Pro-BNP, pro-brain type natriuretic peptide; RV, residual volume; TLC, total lung capacity; WBC, white blood cell.…”

The Role of Infection in Interstitial Lung Diseases

“…106 A more recent retrospective Japanese study suggests that survival in those patients who received PMX may have been improved. 107 PMX is not a currently accepted treatment for AE-ILD, but such research may support the role of infections as antigenic stimulus in the setting of AE.…”

Direct hemoperfusion with polymyxin B-immobilized fiber column in a patient with acute exacerbation of idiopathic pulmonary fibrosis

“…Data on the effective use time and frequency of PMX-DHP of AE-IPF are lacking; additionally, no randomized clinical trials have studied this approach. However, one study reported that PMX-DHP was more effective when applied within 1 week of acute symptom exacerbation onset when the underlying IPF was more severe [1,2]. Here, we reported two cases of AE-IPF that were treated by PMX-DHP.…”

“…AE-IPF is typically associated with an extremely poor prognosis, with the reported 90-day mortality rate being approximately 60%-80% [8]. Steroids and immune-suppressants have been used to treat AE-IPF, but there are currently no effective treatments [1]. PMX-DHP was developed to remove plasma endotoxins from patients with sepsis.…”

“…The prognosis for idiopathic pulmonary fibrosis (IPF) patients with acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is devastating. Currently, no well-validated effective treatment modalities are available and the mean survival time from the onset of acute exacerbation is only 1.5 months [1].…”