Reduction in serum high mobility group box-1 level by polymyxin B-immobilized fiber column in patients with idiopathic pulmonary fibrosis with acute exacerbation.

BACKGROUND/AIM: Recent reports suggest that polymyxin B (PMX)-immobilized fiber may have beneficial effects in idiopathic pulmonary fibrosis (IPF) with acute exacerbation (AE). High mobility group box-1 (HMGB-1) is an important pro-inflammatory mediator that contributes to acute lung inflammation. This study was aimed to investigate whether PMX treatment affects serum HMGB-1 levels and oxygenation in IPF patients with AE. MATERIALS AND METHODS: Twenty IPF patients with AE were treated by PMX. PMX treatment was carried out once daily for 2 successive days. Serum HMGB-1 levels were measured before and after PMX treatment. We also monitored arterial oxygen tension (PaO(2))/inspiratory oxygen fraction (FiO(2)) (P/F) ratio. PMX fiber columns were analyzed to examine whether HMGB-1 was absorbed by PMX. RESULTS: PMX treatment significantly improved both the serum HMGB-1 level and P/F ratio. HMGB-1 was detected in washing medium from the PMX column. CONCLUSION: PMX treatment may reduce serum HMGB-1 and improve oxygenation in patients with IPF with AE.

Neutrophil adsorption by polymyxin B-immobilized fiber column for acute exacerbation in patients with interstitial pneumonia: a pilot study.

BACKGROUND/AIMS: Polymyxin B-immobilized fiber (PMX) treatment has beneficial effects in patients with acute lung injury/acute respiratory distress syndrome or acute exacerbation of idiopathic pulmonary fibrosis. This study was aimed to clarify the mechanism of PMX treatment for acute exacerbation of interstitial pneumonia (IP). MATERIALS AND METHODS: Sixteen consecutive IP patients with acute exacerbation were included. The patients were treated with PMX once daily for 2 successive days at a flow rate of 80-100 ml/min for 6 h. Cells adsorbed by PMX were analyzed morphologically by electron microscopy. Surface markers of these cells were determined by flow cytometry. Serum matrix metalloproteinase (MMP)-9 was measured before and after PMX treatment. RESULTS: Cells adsorbed by PMX were neutrophils and highly expressed HLA-DR, CD14, CD62L and CD114. Serum MMP-9 levels were significantly decreased after PMX treatment. CONCLUSION: This pilot study demonstrated neutrophil adsorption by PMX and its possible clinical application for acute exacerbation of IP.

[A case of suspected idiopathic pulmonary upper lobe fibrosis (Amitani disease) with acute exacerbation].

An 82-year old man was admitted to our hospital for evaluation of progressive general malaise. He had previously been in good health. His chest roentgenogram showed reticular shadows and we suspected interstitial lung disease. On admission, his roentgenographic images showed deterioration compared with previous images. Acute lung injury was diagnosed by transbronchial lung biopsy, and steroid administration was started. He initially responded to treatment, but bilateral spontaneous pneumothorax occurred. Despite treatment, he died of respiratory failure. Amitani disease (idiopathic pulmonary upper lobe fibrosis) was suspected based on postmortem pathology, but his lung parenchyma was poor due to the presence of changes producing diffuse alveolar damage. We report and discuss this case because there are apparently no previous similar cases.

Beneficial effect of polymyxin B-immobilized fiber column (PMX) hemoperfusion treatment on acute exacerbation of idiopathic pulmonary fibrosis.

OBJECTIVE: This study was aimed to investigate the effect of polymyxin B-immobilized fiber column (PMX) hemoperfusion treatment on the acute exacerbation of idiopathic pulmonary fibrosis (IPF). PATIENTS AND METHODS: Six patients with a clinical diagnosis of idiopathic pulmonary fibrosis (IPF) who developed acute exacerbation were included in this study. Although five of six patients were treated with high-dose corticosteroid therapy, mechanical ventilation was necessary for all six patients due to severe respiratory failure. Blood endotoxin levels were undetectable in all patients. PMX treatment was performed on these six patients. RESULTS: In four of six patients, alveolar-arterial difference of oxygen (AaDO(2)), serum KL-6 and lactate dehydrogenase (LDH) were improved after PMX treatment. These four patients were successfully weaned from mechanical ventilation and survived more than 30 days after the initial PMX treatment. CONCLUSION: These data suggest a potential beneficial effect of PMX treatment on acute exacerbation of IPF.

[A case of polymyositis with concomitant diffuse alveolar damage following interstitial pneumonia].

A 68-year-old woman had been given a diagnosis of interstitial pneumonia (NSIP pattern) and followed up at our hospital for 3 years. She was admitted to our hospital because of dyspnea, lower limb edema and myalgia. On admission, serum CPK and CRP levels were elevated and an electromyogram suggested inflammatory myopathy. We diagnosed polymyositis (PM) with progressive interstitial pneumonia (IP). Although methylprednisolone pulse therapy and immunosuppressive agents were administered, pulmonary lesions became aggravated. The patient died due to respiratory failure as a result of the progress of IP. The autopsy lung revealed diffuse alveolar damage (DAD) at the both acute and fibrotic phases, suggesting that DAD could coincide with PM. We report here a rare case of polymyositis with diffuse alveolar damage (DAD).