Drug Rediscovery for Rare Immune Mediated Inflammatory Diseases

Summary

Research into novel therapies for rare, immune-mediated inflammatory diseases (IMIDs) is limited due to small patient populations. Patients with Behçet's disease (BD), idiopathic inflammatory myopathy (IIM, also known as myositis) and IgG4-related disease (IgG4-RD) are treated with high-dosed glucocorticoids, methotrexate, azathioprine and mycophenolate mofetil, mostly for long periods of time with attendant risks of long-term toxicity, including infections. Therefore, there is an urgent need for new, more specific anti-inflammatory therapies such as targeted synthetic and biological disease-modifying antirheumatic drugs. Due to the role of type 1 interferon in both BD, IIM and IgG4-RD, JAK-STAT inhibition may be a promising treatment strategy in these conditions, because JAK1 is critical for the signal transduction of pro-inflammatory cytokine receptors. Previous research showed that JAK1 inhibition reduces activation of type 1 interferon-regulated proteins and key chemokines that control tissue inflammation.

Timeline

Start

2024-03-12

Primary completion

2026-12

Completion

2027-12

Publications

• Geertsema-Hoeve BC, van Laar JAM, Raaphorst J, Tas SW, Welsing PMJ, Goekoop RJ, Checa CM, Thurlings RM, Rekers NH, Present E, van Laar JM. Multicentre, 26-week, open-label phase 2 trial of the JAK inhibitor filgotinib in Behcet's disease, idiopathic inflammatory myopathies and IgG4-related disease: DRIMID study protocol. BMJ Open. 2025 Feb 6;15(2):e089827. doi: 10.1136/bmjopen-2024-089827.

Drugs

Indications