Management of Challenging Cases in Myositis

Before closing, she made note of two ongoing NIH-sponsored trials for IMNM, including one for IVIG and another for ublituximab. IVIG, although approved by the U.S. Food & Drug Administration (FDA) for dermatomyositis, is not yet approved for IMNM.

Management of Recalcitrant Dermatomyositis

Dr. Alisa Femia

The third speaker was Alisa Femia, MD, associate professor in the Department of Dermatology at NYU Gross School of Medicine with an expertise in management of patients with dermatomyositis. She highlighted that, distinct from patients with SLE, antimalarials are not as effective for dermatomyositis (DM), and that adverse cutaneous reactions to hydroxychloroquine are seen in up to 30% of patients with DM.⁶ More effective therapies include IVIG, which has an important role in recalcitrant cutaneous disease, given its corticosteroid sparing effect, reduction in CDASI skin scores, and overall patient tolerance.⁷ IVIG has even demonstrated survival benefit in patients with DM, likely attributable to patients being able to discontinue other DMARDs.⁸

Dr. Femia contrasted this with the more underwhelming results for rituximab, which likely has greater efficacy in muscle disease and showed no difference in skin severity indices in a pilot trial.⁹

Given the correlation of interferon signatures with disease activity in DM, she demonstrated case examples of success with tofacitinib, and looked ahead to agents like dazukibart, a humanized IgG1 neutralizing monoclonal antibody against interferon beta, which is currently being studied in a global phase 3 trial for patients with active DM.¹⁰ She also shared experience of positive results with anifrolumab in recalcitrant DM, and introduced the promising new agent brepocitinib, a TYK2 and JAK1 inhibitor, which functions via marked inhibition of type I and II interferon, IL-6 and IL-12/23 signaling.¹¹

Brepocitinib for Dermatomyositis

Dr. Paik

Before the conclusion of the session, Julie Paik, MD associate professor of medicine and co-director of the Myositis Center at the Johns Hopkins University School of Medicine, took to the podium to share the exciting results from a randomized, double-blind, multi-center, placebo-controlled, phase 3 VALOR trial, which studied the safety and efficacy of brepocitinib for patients with active dermatomyositis. The study met its primary and all key secondary end points for brepocitinib 30 mg, with clinically meaningful benefits on measures of myositis response, and skin, muscle, physical function and corticosteroid tapering. Benefits were observed by week 4 and sustained out to 52 weeks of treatment. Importantly, brepocitinib was well tolerated with a favorable safety profile comparable to approved TY2K and JAK inhibitors.