0135: Janus Kinase Inhibitors in Severe and Refractory Inflammatory Ocular Pathology. Cases Reports and Literature Review

Lara Sánchez-Bilbao¹, Vanesa Calvo Río², José Luis Martín-Varillas³, José Luis Álvarez-Vega⁴, Emma Beltrán Catalán⁵, Olga Maiz⁶, Ignacio Torre⁷, Raúl Veroz⁸, Carmen Alvarez Reguera¹, Rosalía Demetrio-Pablo¹, Miguel Ángel González-Gay⁹ and Ricardo Blanco¹⁰, ¹Hospital Universitario Marqués de Valdecilla, Santander, Spain, ²Valdecilla Hospital, Santander, Spain, ³Hospital de Laredo, Laredo, Cantabria, Spain, ⁴Complejo Hospitalario Universitario de Badajoz, Badajoz, Spain, ⁵Hospital del Mar, Barcelona, Spain, ⁶Hospital Universitario de Donostia, San Sebastián, Spain, ⁷Hospital de Basurto, Basurto, Spain, ⁸Hospital de Mérida, Mérida, Spain, ⁹Department of Medicine and Psychiatry, Universidad de Cantabria; Rheumatology Division, Hospital Universitario Marqués de Valdecilla; Research group on genetic epidemiology and atherosclerosis in systemic diseases and in metabolic diseases of the musculoskeletal system, IDIVAL, Santander, Spain. Cardiovascular Pathophysiology and Genomics Research Unit, School of Physiology, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa, ¹⁰Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain

Background/Purpose: Inflammatory ocular pathology (IOP) includes internal (uveitis) and external [mainly ocular surface pathology such as epi/scleritis and peripheral ulcerative keratitis (PUK)] involvement. IOP may be severe ocular conditions refractory to conventional immunosuppressants and even biological therapy. Janus Kinase inhibitors (JAKINIB) had shown efficacy in refractory cases of different immune-mediated inflammatory diseases (IMID).

In patients with refractory IOP treated with JAKINIB our aims were a) to assess the patients of Spanish referral centers, b) Literature review.

Methods: Multicenter study of 8 patients with refractory IOP treated with JAKINIB. For Literature review a search was conducted in PubMed, Embase and the Cochrane library from their inception to 1^st January 2022, and conference proceedings from four major rheumatology conferences. Original research articles studying JAKINIB treatment in patients with IOP were included. In addition, a therapeutical approach of refractory IOP is proposed.

Results: We have identified 8 cases in six University Hospitals and 11 cases in the literature review. These 19 patients (15 women/ 4 men) (28 affected eyes), mean age 37.1±22.6 years, had different refractory IOP (uveitis=12; scleritis= 4, PUK= 3).

Most of IOP were associated with IMID (n=15, 78.9%) while 4 cases (21.1%), were idiopathic. The main underlying IMID were juvenile idiopathic arthritis (n=5, 26.3%), spondyloarthritis (n=4; 21.1%) and rheumatoid arthritis (n=2, 10.5%) (TABLE).

Uveitis (n=12) followed by ocular surface pathology (n=7) were the most frequent subtypes of IOP. Patterns of uveitis were panuveitis (n=6), anterior uveitis (n=5; 2 of them with Cystoid macular, and posterior (n=1). Ocular surface pathology was due to scleritis (n=4) and PUK (n=3).

In addition to systemic corticosteroids, before JAKINIB, conventional (n= 17; 89.5%) and biological immunosuppressive drugs (n=17; 89.5%) were required. The JAKINIB most widely used was tofacitinib (n= 10; 52.6%) followed by baricitinib (n=7; 36.8%). In one patient with Blau Syndrome and uveitis, tofacitinib was switched to baricitinib due to severe lymphopenia. No other patient experienced a serious adverse reaction.

After starting JAKINIB treatment, all patients presented clinical improvement, complete (n=17, 89.5%) or partial (n= 2; 10.5%).

Based on these data a therapeutical approach of refractory IOP was proposed (FIGURE).

Conclusion: JAKINIB may be an effective and safe therapy in IOP refractory to conventional or even biological immunosuppressive therapy.




Disclosures: L. Sánchez-Bilbao, Eli Lilly; V. Calvo Río, AbbVie/Abbott, Eli Lilly, Merck/MSD, UCB; J. Martín-Varillas, AbbVie/Abbott, Pfizer, Janssen, UCB, Celgene; J. Álvarez-Vega, None; E. Beltrán Catalán, None; O. Maiz, None; I. Torre, None; R. Veroz, None; C. Alvarez Reguera, None; R. Demetrio-Pablo, None; M. González-Gay, AbbVie/Abbott, Merck/MSD, Janssen, Roche, AbbVie/Abbott, Roche, Sanofi, Eli Lilly, Celgene, Sobi, Merck/MSD; R. Blanco, Eli Lilly, Pfizer, Roche, Janssen, MSD, AbbVie, Amgen, AstraZeneca, Bristol Myers Squibb, Galapagos, Novartis, Sanofi.