Rapidly Progressive Interstitial Lung Disease Associated with Dermatomyositis Treated with Combination of Immunosuppressive Therapy, Direct Hemoperfusion with a Polymyxin B Immobilized Fiber Column and Intravenous Immunoglobulin

Abstract

Rapidly progressive interstitial lung disease (ILD) is associated with dermatomyositis (DM) and has a high mortality rate even with immunosuppressive agents. For such cases, there is no evidence on the combined effect of direct hemoperfusion with a Polymyxin B immobilized fiber column and intravenous immunoglobulin. We herein report a case of 61-year-old woman who presented with respiratory failure. She showed ILD associated with DM which did not improve with immunosuppressive agents, but was improved with the addition of both direct hemoperfusion with a Polymyxin B immobilized fiber column and intravenous immunoglobulin.

Direct hemoperfusion with polymyxin B‐immobilized fibre treatment for acute exacerbation of interstitial pneumonia

“…Several case reports similarly documented improvement in patients treated for rapid progressive interstitial pneumonia with dermatomyositis. 10,11,29,30 In the 12 patients with dermatomyositis in our study (5 with and 7 without PMX-DHP), the survival proportion was better when PMX-DHP treatment was administered. Teruya et al reported a successful response to PMX-DHP in a patient with amyopathic dermatomyositis who developed RP-ILD characterized by elevated anti-MDA5 autoantibodies.…”

Intravenous immunoglobulin treatment stabilizing a patient with Anti-PL7 antisynthetase syndrome with interstitial lung disease and eosinophilic inflammation

“…[ 11 ] Case reports Anti-Jo-1 AS-ILD 3 1–5x30–40 g up to 9 courses CS, CYC Improvement of dyspnea and chest CT (in 2 patients) Takai et al. [ 12 ] Case report Anti-Jo-1 AS-ILD 1 20 g for 5 days CS, MPT, CYC, CYa, Polymyxin B Recovered acute phase, improvement of dyspnea and interstitial changes Suzuki et al. [ 14 ] Case report Anti-Jo-1 AS-ILD.…”

“…IVIG increased FVC, DLCO and decreased the mean prednisolone dose over time. Three other AS-ILD case reports [ [10] , [11] , [12] ] describe clinical improvement after treatment with IVIG. However, Hervier et al.…”

Polymyxin-B Hemoperfusion as a Novel Treatment for Rapidly Progressive Interstitial Lung Disease in a Pediatric Patient Diagnosed With Anti-MDA5 Juvenile Dermatomyositis

“…Teruya et al 11 demonstrated serial reductions in circulating immune complexes after the use of PMX-DHP, suggesting that this treatment may work to directly remove anti-MDA5 complexes as well. Based on these hypotheses, PMX-DHP has been used for treatment of adult patients with anti-MDA5 dermatomyositis-associated RP-ILD 8,11,13 …”

“…As the patient continued to worsen on traditional aggressive therapy, at the family's request, a literature review was conducted to investigate possible novel treatments in patients with RP-ILD. PMX-DHP was considered based on recent evidence, suggesting improved outcomes for adult patients with acute respiratory distress syndrome 10 and RP-ILD 11,13 . After discussion with the ChILD program, as well as with the manufacturer (Toray Medical Inc, Tokyo, Japan.…”