Background <p>Necrotizing scleritis is a rare but severe ocular inflammatory disorder, frequently associated with systemic autoimmune diseases such as rheumatoid arthritis (RA). Although biologic agents targeting cytokines such as TNF-α and IL-6 have improved disease outcomes, some patients remain refractory to these treatments. We describe a case of necrotizing scleritis resistant to multiple biologic agents that responded favorably to the Janus kinase (JAK) inhibitor upadacitinib.</p> Case presentation <p>A 43-year-old woman with rheumatoid arthritis presented with bilateral conjunctival injection and left ocular pain. Despite systemic corticosteroid therapy (30&#xa0;mg/day) and switching between biologics, ocular inflammation and scleral thinning continued to progress. Consequently, oral administration of the selective JAK1 inhibitor upadacitinib (15&#xa0;mg/day) was initiated. Two months after treatment, ocular pain and redness resolved completely, with marked regression of nodular inflammation and stabilization of scleral thinning. Systemic corticosteroids were successfully tapered to 5&#xa0;mg/day, and the patient remained relapse-free for six months without any adverse events.</p> Conclusion <p>Upadacitinib, through selective inhibition of the JAK-STAT signaling pathway, provided rapid and sustained control of inflammation in a case of necrotizing scleritis unresponsive to biologic agents. Although this observation suggests that JAK inhibitors may represent a potential therapeutic option for refractory ocular inflammation, further studies are required to clarify their long-term efficacy and safety in patients with scleritis.</p>

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Necrotizing scleritis resistant to biologics successfully managed with the JAK inhibitor upadacitinib: a case report

  • Tomoyuki Oyama,
  • Shunsaku Nakai,
  • Kenji Miyao,
  • Masaya Imazeki,
  • Masaru Takeuchi

摘要

Background

Necrotizing scleritis is a rare but severe ocular inflammatory disorder, frequently associated with systemic autoimmune diseases such as rheumatoid arthritis (RA). Although biologic agents targeting cytokines such as TNF-α and IL-6 have improved disease outcomes, some patients remain refractory to these treatments. We describe a case of necrotizing scleritis resistant to multiple biologic agents that responded favorably to the Janus kinase (JAK) inhibitor upadacitinib.

Case presentation

A 43-year-old woman with rheumatoid arthritis presented with bilateral conjunctival injection and left ocular pain. Despite systemic corticosteroid therapy (30 mg/day) and switching between biologics, ocular inflammation and scleral thinning continued to progress. Consequently, oral administration of the selective JAK1 inhibitor upadacitinib (15 mg/day) was initiated. Two months after treatment, ocular pain and redness resolved completely, with marked regression of nodular inflammation and stabilization of scleral thinning. Systemic corticosteroids were successfully tapered to 5 mg/day, and the patient remained relapse-free for six months without any adverse events.

Conclusion

Upadacitinib, through selective inhibition of the JAK-STAT signaling pathway, provided rapid and sustained control of inflammation in a case of necrotizing scleritis unresponsive to biologic agents. Although this observation suggests that JAK inhibitors may represent a potential therapeutic option for refractory ocular inflammation, further studies are required to clarify their long-term efficacy and safety in patients with scleritis.