Gal-9 attenuates ALI by expanding CD14(-)CD11b(+)Gr-1(+) pDC-like macrophages by preferentially suppressing macrophage functions to release proinflammatory cytokines through TLR4 and TLR2 down-regulation.
We present 3 cases of rapidly progressive interstitial pneumonia (RPIP) associated with clinically amyopathic dermatomyositis (C-ADM) that were treated with two courses of direct hemoperfusion with polymyxin B-immobilized fiber column (PMX-DHP). Despite initial treatment with high-dose corticosteroids, pulsed cyclophosphamide, and cyclosporine, the lung disease and hypoxemia deteriorated in all the patients. After PMX-DHP treatment, the PaO2/FiO2 ratio and serum LDH and KL-6 were improved, the abnormal shadows in chest high-resolution computed tomography (HRCT) scans gradually decreased, and, finally, all patients survived. These findings indicate that PMX-DHP treatment could be effective in the management of RPIP in patients with C-ADM in combination with conventional therapy.
Background:Direct hemoperfusion using polymyxin B-immobilized fiber column (PMX-DHP)
therapy has been approved for sepsis-associated acute respiratory distress
syndrome, but its efficacy for other rapidly progressive interstitial
pneumonias (RPIPs) is unclear. The purpose of this study was to examine the
efficacy of PMX-DHP therapy for acute respiratory failure in patients with
RPIPs, when compared with a historical control receiving conventional
treatment without PMX-DHP.Methods:This study comprised 77 patients with RPIPs in our institute between January
2002 and December 2015. The initial 36 patients between January 2002 and
March 2007 were treated without PMX-DHP (historical control group), and the
following 41 patients between April 2007 and December 2015 were treated with
PMX-DHP (PMX-DHP group) once daily for two successive days concurrently with
corticosteroids and/or immunosuppressive agents. The 90-day mortality and
clinical factors were compared between the groups. Cox proportional hazards
models were constructed to analyze 90-day mortality and identify
predictors.Results:The 90-day mortality rate was significantly lower in the PMX-DHP group than
in the controls (41.5% versus 66.7%, p =
0.019). PMX-DHP therapy was significantly associated with mortality (hazard
ratio 0.505; 95% confidence interval, 0.270–0.904; p =
0.032). There were significant differences in the serial changes in the
PaO2/FiO2 ratio, SOFA score, and blood neutrophil
counts from days 0–5 after PMX-DHP between the survivor and non-survivor
groups (p = 0.015, p < 0.001,
p = 0.035, respectively). The improved
PaO2/FiO2 ratio on day 3 significantly correlated
with the change in blood neutrophil counts (rs = −0.431,
p = 0.006).Conclusions:PMX-DHP therapy may be effective in RPIPs patients accompanied by acute
respiratory failure and is expected to reduce mortality rates.
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