Current pharmacological treatment of idiopathic inflammatory myopathies

Abstract

The idiopathic inflammatory myopathies are uncommon and heterogeneous disorders. Their classification is based on distinct clinicopathologic features. Although idiopathic inflammatory myopathies share some similarities, different subtypes may have variable responses to therapy, so it is very important to distinguish the correct subtype.There are few randomised, double blind placebo controlled studies to support the current treatment.High dose corticosteroids continue to be the first line therapy and other immunosupressive drugs are used in refractory cases, as well as steroid-sparing agents.Some novel therapeutic approaches have emerged as potential treatment including tacrolimus, intravenous immunoglobulin and rituximab, following good outcomes reported in case studies.However, more randomised controlled trials are needed.This review considers the current and the potential future therapies for inflammatory myopathies.

Treatment of Juvenile Dermatomyositis: An Update

“…It necessitates monitoring during the first few months to ensure its trough serum level is optimal (90-150mg/mL)Furthermore, ciclosporin is toxic to many organs, i.e. : the kidneys, liver and bone marrow [69].…”

“…Mycophenolate mofetil (MMF) is emerging as a promising drug, especially when used in refractory IIM [69] and in patients with interstitial lung disease (ILD) refractory to steroids [72]. Two retrospective studies where 12 [73] and 50 [74] JDM patients were treated with MMF demonstrated a good response and safety profile.…”

“…A recently published review of current pharmacological treatment of idiopathic inflammatory myopathies [69] described three cases of refractory myositis treated with tocilizumab with promising results [118][119][120] and three case reports showing beneficial effects of co-stimulation blockade using Abatacept in myositis [121][122][123]. A randomized clinical trial is underway to test this hypothesis (ClinicalTrials.gov Identifier: NCT02594735).…”

Orbital inflammatory disease management

“…Corticosteroids are often the first-line treatment for orbital DM [93,158]. In two cases of orbital myositis associated with DM, methotrexate was used concurrently with prednisone to good result [152,154], and in another case of DM-associated orbital myositis, methylprednisolone and IVIG led to resolution [153].…”

“…Minimal literature exists on the treatment of strictly orbital DM with other biologics and immunosuppressants. A recent review of the treatment of systemic DM noted good treatment responses in patients who received abetacept and rituximab and varied responses in patients who received infliximab and etanercept [158].…”