title: Modern Therapies for Idiopathic Inflammatory Myopathies (IIMs): Role of Biologics.
abstract: Despite the lack of placebo-controlled trials, glucocorticoids are considered the mainstay of initial treatment for idiopathic inflammatory myopathy (IIMs) and myositis-associated ILD (MA-ILD). Glucocorticoid-sparing agents are often given concomitantly with other immunosuppressive agents, particularly in patients with moderate or severe disease. As treatment of refractory cases of idiopathic inflammatory myopathies has been challenging, there is growing interest in evaluating newer therapies including biologics that target various pathways involved in the pathogenesis of IIMs. In a large clinical trial of rituximab in adult and juvenile myositis, the primary outcome was not met, but the definition of improvement was met by most of this refractory group of myositis patients. Rituximab use was also associated with a significant glucocorticoid-sparing effect. Intravenous immune globulin (IVIg) can be used for refractory IIMs or those with severe dysphagia or concomitant infections. Anti-tumor necrosis factor (anti-TNF) utility in IIMs is generally limited by previous negative studies along with recent reports suggesting their potential for inducing myositis. Further research is required to assess the role of new therapies such as tocilizumab (anti-IL6), ACTH gel, sifalimumab (anti-IFNα), and abatacept (inhibition of T cell co-stimulation) given their biological plausibility and encouraging small case series results. Other potential novel therapies include alemtuzumab (a humanized monoclonal antibody which binds CD52 on B and T lymphocytes), fingolimod (a sphingosine 1-phosphate receptor modulator that traps T lymphocytes in the lymphoid organs), eculizumab, and basiliximab. The future investigations in IIMs will depend on well-designed controlled clinical trials using validated consensus core set measures and improvements in myositis classification schemes based on serologic and histopathologic features.
authors.affiliation: Department of Medicine, Myositis Center and Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, 3601 5th Avenue, Suite 2B, Pittsburgh, PA, 15261, USA.
authors.forename: Siamak
authors.initials: S
authors.lastname: Moghadam-Kia
authors.affiliation: Department of Medicine, Myositis Center and Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, 3601 5th Avenue, Suite 2B, Pittsburgh, PA, 15261, USA.
authors.forename: Chester V
authors.initials: CV
authors.lastname: Oddis
authors.affiliation: Department of Medicine, Myositis Center and Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, 3601 5th Avenue, Suite 2B, Pittsburgh, PA, 15261, USA. aggarwalr@upmc.edu.
authors.forename: Rohit
authors.initials: R
authors.lastname: Aggarwal
mesh_terms: D001685:Biological Factors / Q000627:therapeutic use*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D009220:Myositis / Q000188:drug therapy*
chemical_list: D001685:Biological Factors; D007166:Immunosuppressive Agents
keywords: Biologic agents; Dermatomyositis; Idiopathic inflammatory myopathy; Myositis; Polymyositis; Treatment