Favorable outcome with hemoperfusion of polymyxin B-immobilized fiber column for rapidly progressive interstitial pneumonia associated with clinically amyopathic dermatomyositis: report of three cases

Abstract

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.

Recent Treatment of Interstitial Lung Disease with Idiopathic Inflammatory Myopathies

“… 71 Ichiyasu et al reported three cases of RP-ILD with CADM, in which the PaO 2 /FiO 2 ratio and CT findings improved, and all patients survived after treatment with PMX-DHP. 67 The mechanism of the efficacy of PMX-DHP in RP-ILD is not sufficiently known, although PMX-DHP likely inhibits monocyte activation. 72 …”

Rapidly progressive interstitial lung disease due to anti-MDA-5 antibody-positive clinically amyopathic dermatomyositis complicated with cervical cancer: Successful treatment with direct hemoperfusion using polymyxin B-immobilized fiber column therapy

“…demonstrated that the disease activities of CADM-ILD are related to the serum level of interleukin-8 and the mRNA level of CD11b in circulating neutrophils [15]. Several case reports and retrospective studies have demonstrated that applying PMX-DHP in addition to conventional therapies was effective for the treatment of rapidly progressive interstitial pneumonias with respiratory failure, including CADM [4], [12], [14]. However, the efficacy of PMX-DHP therapy in combination with conventional therapies remains unclear.…”

“…Rapidly progressive ILD associated with CADM is refractory to intensive therapies such as the systemic administration of high-dose corticosteroids and immunosuppressive agents, leading to a poor prognosis [2], [3]. We have previously reported that direct hemoperfusion using polymyxin B-immobilized fiber column (PMX-DHP) therapy in combination with conventional therapy can be effective in the management of rapidly progressive ILD in patients with CADM [4]. However, the efficacy of adding PMX-DHP therapy when rapidly progressive ILD is associated with CADM and a malignancy remains unclear.…”

Reversible platypnea‐orthodeoxia syndrome induced by rapidly progressive interstitial pneumonia in a patient with polymyositis

“…Therefore, although we did not investigate the lung tissue pathologically in our patient, we thought that her respiratory failure showed the presence of a diffuse alveolar damage pattern. Recent reports have suggested that PMX‐DHP might improve oxygenation in patients with acute lung injury/acute respiratory distress syndrome associated with fulminant interstitial pneumonia or rapidly progressive interstitial pneumonia with polymyositis/dermatomyositis [2]. Moreover, early intervention with aggressive combination therapy including IVCY and cyclosporine might be life saving as a treatment for rapidly progressive interstitial pneumonia polymyositis/dermatomyositis [3].…”