Abstract
BackgroundRapidly progressive interstitial pneumonias (RPIPs) associated with clinically amyopathic dermatomyositis (CADM) are highly resistant to therapy and have a poor prognosis. Multimodal therapies, including direct hemoperfusion using a polymyxin B-immobilized fiber column (PMX-DHP), have a protective effect on RPIPs. We evaluated the effects of PMX-DHP on CADM-associated RPIPs.MethodsWe retrospectively enrolled 14 patients with CADM-associated RPIPs and acute respiratory failure treated with PMX-DHP, corticosteroids, and immunosuppressive agents. Clinical manifestations were compared between survivors and non-survivors at 90 days after PMX-DHP.ResultsThe survival rate at 90 days after PMX-DHP was 35.7% (5/14). Before PMX-DHP, the survivor group exhibited a significantly higher PaO2/FiO2 (P/F) ratio and serum surfactant protein-D (SP-D) levels and significantly lower lactate dehydrogenase (LDH) and ferritin levels than the non-survivor group. Platelet counts were significantly decreased after PMX-DHP therapy in both groups, but remained higher in the survivor group than the non-survivor group over the course of treatment. Anti-melanoma differentiation-associated gene 5 (MDA-5) antibody positive patients demonstrated a poor 90-day survival rate, lower platelet counts and P/F ratio, and higher LDH levels than anti-MDA-5 antibody negative patients.ConclusionsCADM-associated RPIPs with anti-MDA-5 antibody is associated with a very poor prognosis. A higher P/F ratio and SP-D level, lower LDH and ferritin levels, higher platelet counts, and anti-MDA-5 antibody negativity are important prognostic markers in patients with CADM-associated RPIPs treated with PMX-DHP.
Highlights
Progressive interstitial pneumonias (RPIPs) associated with clinically amyopathic dermatomyositis (CADM) are highly resistant to therapy and have a poor prognosis
CADMassociated Rapidly progressive interstitial pneumonia (RPIP) are resistant to combination therapy with high-dose corticosteroids and immunosuppressive agents, and have a poor prognosis
The antiMDA-5 antibody is mutually exclusive to antiaminoacyl-tRNA synthetase (ARS) antibodies, a group of representative antibodies detected in DM and polymyositis (PM)
Summary
Progressive interstitial pneumonias (RPIPs) associated with clinically amyopathic dermatomyositis (CADM) are highly resistant to therapy and have a poor prognosis. Multimodal therapies, including direct hemoperfusion using a polymyxin B-immobilized fiber column (PMX-DHP), have a protective effect on RPIPs. We evaluated the effects of PMX-DHP on CADM-associated RPIPs. Dermatomyositis (DM) is an autoimmune disorder affecting the muscles, skin, joints, heart, and lungs. Interstitial pneumonia arises in approximately 40%– 50% of patients with DM. The clinical course of interstitial pneumonia with DM varies. Progressive interstitial pneumonias (RPIPs) are a serious complication of interstitial pneumonia with DM and a leading cause of death among patients with the condition [1]. CADMassociated RPIPs are resistant to combination therapy with high-dose corticosteroids and immunosuppressive agents, and have a poor prognosis. The antiMDA-5 antibody is mutually exclusive to antiaminoacyl-tRNA synthetase (ARS) antibodies, a group of representative antibodies detected in DM and polymyositis (PM)
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