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The role of bDMARDs in idiopathic inflammatory myopathies: A systematic literature review
Affiliation:1. Rheumatology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, Siena, Italy;2. Rheumatology Section, Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties, University of Palermo, Palermo, Italy;3. Respiratory Diseases Unit, Department of Medical and Surgical Sciences and Neurosciences, University of Siena, Siena, Italy;1. Department of Epidemiology and Biostatistics, School of Public Health, Anhui Medical University, Hefei, Anhui, China;2. Inflammation and Immune Mediated Diseases Laboratory of Anhui Province, Hefei, Anhui, China;3. Department of Cardiovascular Surgery, the First Affiliated Hospital of USTC, Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei, Anhui, China;1. Department of Rheumatology, The Second Hospital of Shanxi Medical University, Taiyuan, Shanxi, China;2. Shanxi Key Laboratory of Immunomicroecology, Taiyuan, Shanxi, China;3. College of Basic Medicine, Shanxi Medical University, Taiyuan, Shanxi, China;4. Pathology, Joint Program in Transfusion Medicine, Brigham and Women’ Hospital/Children''s Hospital, Harvard Medical School, Boston, MA, USA;1. Department of Critical Care Medicine, Shengjing Hospital of China Medical University, Shenyang 110004, Liaoning Province, PR China;2. Department of Gastroenterology, Shengjing Hospital of China Medical University, Shenyang 110004, Liaoning Province, PR China;3. Department of Pediatrics, Central Michigan University, Detroit, MI 48201, USA;4. Division of Rheumatology/Allergy and Clinical Immunology, School of Medicine, University of California, Davis, CA 95616, USA;1. Laboratory of Pathophysiological and Nutritional Biochemistry, Université Libre de Bruxelles, Belgium;2. Université Libre de Bruxelles (ULB), Hôpital Universitaire de Bruxelles (H.U.B), CUB Hôpital Erasme, Rheumatology Department, Route de Lennik, 808 1070 Brussels, Belgium;1. via Ampere 126, 20131 Milan, Italy;2. Department of Biomedical Sciences, Humanitas University, Via Rita Levi Montalcini 4, 20072, Pieve Emanuele, Milan, Italy;3. Nephrology and Dialysis Division, IRCCS Humanitas Research Hospital, Via Manzoni 56, 20089, Rozzano, Milan, Italy
Abstract:Idiopathic inflammatory myopathies (IIM) are a group of different conditions typically affecting striate muscle, lung, joints, skin and gastrointestinal tract. Treatment typically relies on glucocorticoids and synthetic immunosuppressants, but the occurrence of refractory, difficult to treat, manifestations, may require more aggressive treatment, borrowed from other autoimmune diseases, including biologic disease modifying drugs (bDMARDs). In this regard, we conducted a systemic literature review in order to depict the current evidence about the use of bDMARDs in IIM. A total of 78 papers, published during the last 21 years, were retrieved. The majority of patients was treated with TNF-α inhibitors, whose effectiveness was assessed particularly in recalcitrant striate muscle, skin and joints involvement. Rituximab, whose evidence is supported by a large number of real-life studies and trials, seems to be an excellent option in case of ILD and anti-synthetase syndrome, while Tocilizumab, despite not meeting primary and secondary endpoints in a recently published clinical trial, proved its effectiveness in rapidly progressing ILD. Similarly, Abatacept, studied in a phase IIb clinical trial with conflicting evidence, was reported to be effective in some case reports of refractory dermatomyositis. Less data exist for anti-IL1 and anti-IL23 agents, which were employed particularly for inclusion body myositis and severe skin disease, respectively. This study provides an organ-focused assessment of bDMARDs in IIM, which display encouraging results in the treatment of refractory subsets of disease.
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