New Potential Weapons for Refractory Scleritis in the Era of Targeted Therapy

Abstract

Objective. To assess the efficacy of biologic drugs, beyond tumor necrosis factor- (TNF-) α inhibitors, in the management of noninfectious refractory scleritis, either idiopathic or associated with systemic immune-mediated disorders. Patients and Methods. This is a retrospective study assessing the efficacy of several biologic agents (rituximab, anakinra, tocilizumab, and abatacept) and the small molecule tofacitinib in the treatment of scleritis through assessment of scleral inflammation and relapses, as well as treatment impact on best-corrected visual acuity (BCVA) and safety profile. Results. Fourteen patients (19 eyes) were enrolled in the study. Scleritis inflammatory grading significantly improved from baseline to 3 months (p=0.002) and from baseline to the last follow-up visit (p=0.002). Scleritis relapses significantly decreased between the 12 months preceding and following biologic therapy (p=0.007). No differences regarding BCVA were observed (p=0.67). Regarding adverse events, only one patient developed pneumonia and septic shock under rituximab treatment. Conclusions. Our results, though limited to a low number of patients, highlight the effectiveness of different biologic therapies in the treatment of noninfectious refractory scleritis, showing to control scleral inflammation and allowing a significant reduction in the number of relapses.

Rituximab for non-infectious Uveitis and Scleritis

“…A total of 85.5% (71/83) reported no adverse events following treatment with RTX for non-infectious scleritis. However, two patients each (2/83, 2.4%) developed itching with infusion [ 70 ] and disease exacerbation [ 58 , 62 ], and one patient each (1/83, 1.2%) developed herpetic mouth ulcers [ 37 ], acute retinal necrosis [ 67 ], herpes zoster [ 56 ], hypotension during infusion of RTX [ 55 ], ocular hypertension [ 57 ], pneumonia with septic shock [ 60 ], dry eyes, the combination of weight loss, fatigue, and joint pain [ 63 ], and the combination of leukopenia, anemia, bronchitis, and bacterial pneumonia [ 56 ] attributed to RTX treatments.…”

Clinical Relevance of Autoantibodies and Inflammatory Parameters in Non-infectious Scleritis

“…4,6,7,16 In our series, the prevalence of these autoantibodies was slightly higher than previously reported. [17][18][19] This finding may be partly due to our tertiary referral center specialized in immunological disorders, or might in part be attributable to the use of different, more sensitive laboratory techniques for autoantibody detection.…”

Rheumatoid Arthritis Associated Episcleritis and Scleritis: An Update on Treatment Perspectives

“…In 2010, Iaccheri et al reported a case of acute stromal keratitis and scleritis, with only partial response to cyclophosphamide, who was successfully controlled after two infusions of rituximab, off all steroids and without recurrence after 9 months of follow-up [ 25 ]. In the retrospective series of Fabiani et al, 4 out of 5 patients treated with rituximab improved by at least 2 step of scleritis grading system [ 30 ]. Adverse effects may include reactivation of viruses, as reported with acute retinal necrosis [ 31 ].…”

“…Tocilizumab was reported effective to control inflammation and steroid sparing in 50% of patients with refractory scleritis [ 36 ]. In the retrospective series of Fabiani et al [ 30 ], 2 patients (3 eyes) were successfully treated with 162 mg/week of tocilizumab and 3 patients (4 eyes) treated for diffuse anterior scleritis, 2 patients (2 eyes) were successfully controlled with 125 mg weekly abatacept.…”

“…Tofacitinib is currently investigated for its efficacy in ocular inflammation ( (accessed on 11 May 2021) Identifier: NCT03580343). Its effectiveness in refractory scleritis has been suggested in case reports [ 30 , 37 ], including in patients with necrotizing scleritis [ 38 ].…”