Efficiency of Therapeutic Plasma-Exchange in Acute Interstitial Lung Disease, Associated With Polymyositis/Dermatomyositis Resistant to Glucocorticoids and Immunosuppressive Drugs: A Retrospective Study.

Yaogui Ning,Guixiu Shi,Yuan Liu,Yuechi Sun,Guomei Yang,Shiju Chen

doi:10.3389/fmed.2019.00239

Yaogui Ning, Guixiu Shi + Show 4 more

Open Access

https://doi.org/10.3389/fmed.2019.00239

Copy DOI

Abstract

Interstitial lung disease (ILD) is a life-threating complication, commonly associated with polymyositis (PM), and dermatomyositis (DM). A subset of acute ILD associated with PM/DM patients are refractory to conventional treatment, and leads to a high rate of mortality. The efficacy of therapeutic plasma-exchange (TPE) as a PM/DM treatment to improve muscle involvement is controversial due to a lack of evidence. However, in recent reports, TPE has been effective in improving lung involvement. To evaluate the efficacy of this therapy, we retrospectively studied TPE treatment outcomes for in 18 acute PM/DM-ILD patients who were resistant to conventional therapies. Five patients were diagnosed with DM (27.8%), 11 with CADM (61.1%), and two with PM (11.1%). Among 18 patients, 11 (61.1%) achieved satisfactory improvement after four or more rounds of TPE, whereas seven died due to respiratory failure. We also analyzed risk factors to predict unresponsiveness to TPE in these patients. Notably, the prevalence of subcutaneous/mediastinal emphysema was significantly higher in the non-responsive group (6/7, 85.7%) than in the responsive group (2/11, 18.2%; P = 0.013); moreover, patients with this complication were mainly in the CADM subgroup (6/8, 75%). Subcutaneous/mediastinal emphysema and increased serum ferritin levels were shown to be poor prognostic factors, predictive of unresponsiveness to TPE, in PM/DM patients. No autoantibodies were found to be associated with TPE outcome, although we only investigated anti-Jo-1 and anti-Ro antibodies; the clinical significance of other myositis-specific autoantibodies, especially anti-melanoma differentiation-associated gene 5 (MDA5) antibody, is not known. Our results indicate that TPE might be an alternative treatment for acute PM/DM-ILD patients resistant to conventional therapies, except for those with subcutaneous/mediastinal emphysema and high serum ferritin levels.

Highlights

Idiopathic inflammatory myopathy (IIM) is a group of heterogeneous inflammatory muscle disorders, which includes subacute, chronic, and acute IIM
The results of logistic regression analyses showed that subcutaneous/mediastinal emphysema and serum ferritin levels were significantly associated with this in acute PM/DM-Interstitial lung disease (ILD) patients who were resistant to conventional therapies
A large proportion of patients with acute PM/DM-ILD show no response to conventional therapies including glucocorticoids and immunosuppressive agents, leading to uncontrolled and aggressive lung involvement and death due to respiratory failure

Summary

Introduction

Idiopathic inflammatory myopathy (IIM) is a group of heterogeneous inflammatory muscle disorders, which includes subacute, chronic, and acute IIM. DM and PM are the most common forms of IIM and are life-threating in rheumatic diseases. Interstitial lung disease (ILD) is one of the most common and life-threating complications of PM/DM, with a prevalence up to 86% [2,3,4,5,6]. The survival rate of patients with PM/DM-ILD is 56.7% during the first year, and even lower in those with acute ILD [7]. Patients with rapidly progressive ILD are often resistant to high-dose glucocorticoids and immunosuppressive agents [8], thereby resulting in acute fatal respiratory failure with a 6months survival rate of 40.8–45.0% [9, 10]. The lack of effective treatment strategies for PM/DM-ILD patients who are resistant to glucocorticoids and immunosuppressive agents is the main contributor to high mortality rates

Methods

Results

Conclusion