HHE 2018 | Page 197

Quote here kpkp pk pkpk pk ppkp pk pkpk pkpk pk kpkp pk pkpk pk ppkp pk pkpk pkpk pk kpkp pk pkpk pk ppkp pk pkpk pkpk pk kpkp pk pkpk pk ppkp pk pkpk pkpk pk kpkp safety and efficacy of Octagam 10% in patients with DM (NCT02728752); and an open label study of subcutaneous immunoglobulin in patients with DM (NCT02271165). Tocilizumab, an interleukin-6 (pro- inflammatory cytokine) receptor antagonist, is approved for the treatment of rheumatoid arthritis and systemic juvenile idiopathic arthritis. 12 The ongoing tocilizumab study is a multicentre, randomised, placebo-controlled trial to determine effectiveness of tocilizumab in the treatment of patients with refractory adult PM and DM (NCT02043548). Abatacept, a modulator of T-cell activation by binding to CD80 and CD86 molecules on antigen- presenting cells, thereby blocking interaction with CD28 on T cells, 13 is approved for the treatment of adult rheumatoid arthritis, polyarticular juvenile idiopathic arthritis and psoriatic arthritis. Recently, results of a randomised, open-label, ‘delayed-start’ treatment trial in DM/PM were published (ARTEMIS; NCT01315938). In this pilot study, treatment with abatacept resulted in lower disease activity in nearly half of the patients (42%). In patients with repeat muscle biopsies, an increased frequency of Foxp3+ Tregs at six months suggested a positive effect of treatment in muscle tissue. 14 Abatacept is currently being studied in a randomised, double- blinded, controlled pilot trial to evaluate the efficacy and safety of subcutaneous abatacept in treating interstitial lung disease associated with anti-synthetase syndrome (NCT03215927); in an open label study of abatacept for the treatment of refractory juvenile DM (NCT02594735) and Phase III, randomised, double blind clinical trial to evaluate efficacy and safety of abatacept in adults with active IIM (NCT02971683). The later study is unique in that it includes not only DM and PM but also IMNM cases and adult JDM patients as well. Siponimod is a selective modulator of sphingosine 1-phosphate receptor (S1P1,5) that inhibits the movement of lymphocytes out of lymph nodes. 15 A double-blind, randomised, placebo-controlled study of siponimod in patients with active DM (NCT02029274) completed recruitment in 2016 and a previous multicentre double-blind, placebo controlled study of siponimod in PM (NCT01801917) was prematurely stopped in 2016 due to slow recruitment and small sample size. Ajulemic acid (JBT-101) is a synthetic compound that selectively binds to the cannabinoid receptor type 2 (CB2) 16 promoting anti-inflammatory and anti-fibrotic effects by increasing production of PGJ2 (an endogenous anti-inflammatory ligand) and decreasing collagen neosynthesis by fibroblasts. 17 Ajulemic acid is currently being studied in a Phase II, double-blinded, randomised, placebo-controlled study to investigate the safety, tolerability and efficacy in patients with DM (NCT02466243). Interferon (IFN)-Kinoid is an inactivated form of IFNα2b that has been studied as a therapeutic vaccine in patients with systemic lupus erythematosus. It has been shown to induce a polyclonal response against most of the IFNα subtypes, decreasing IFN and B cell-associated transcripts. 18 It is currently being studied in a single-blind, randomised, proof of concept study to evaluate the production of anti-IFNα antibodie