UNASSIGNED: Non-infectious inflammatory ocular diseases pose significant challenges in diagnosis and management, often requiring systemic immunosuppressive therapy. Since Janus kinase (JAK) inhibitors may represent a novel therapeutic option for these disorders, the present study aimed to expand current knowledge about their efficacy and safety in patients with these conditions.
UNASSIGNED: This prospective cohort study included 12 adult patients from the international AutoInflammatory Disease Alliance (AIDA) Network registries dedicated to non-infectious ocular inflammatory conditions. We assessed ocular flares, visual acuity, disease course, and complications before and after initiating JAK inhibitor therapy.
UNASSIGNED: Ocular inflammation was related to a systemic disease in 8 (66.7%) patients as follows: spondyloarthritis (n = 3), peripheral psoriatic arthritis (n = 1), rheumatoid arthritis (n = 1), antinuclear antibodies (ANA) positive juvenile idiopathic arthritis (n = 1), Behçet\'s syndrome (n = 1), Vogt-Koyanagi-Harada syndrome (n = 1). In total, 4 patients received baricitinib, 1 patient received tofacitinib, and 7 patients underwent upadacitinib treatment. The overall average duration of JAK inhibitors treatment was 8.6 ± 5.5 months (ranging from 3 to 20 months). At the last assessment, ocular disease control was complete in 12/12 patients. One patient discontinued baricitinib due to poor compliance after a 12-month relapse-free period. The incidence of ocular flares was 125 episodes/1.000 person-months prior to the initiation of JAK inhibitors and 28.6 episodes/1.000 person-months thereafter. The incidence rate ratio for experiencing a relapse before starting a JAK inhibitor compared to the following period was 4.37 (95% CI 1.3-14.7, p-value: 0.02).
UNASSIGNED: JAK inhibitors demonstrate efficacy and safety in controlling ocular inflammatory relapses, confirming that they represent a valuable treatment option for patients with non-infectious inflammatory ocular diseases resistant to conventional treatments.

UNASSIGNED: At present, the severity and grade of anterior scleritis are judged mainly based on the area and location of involvement, whether there is necrosis, etc. Quantitative measurement of sclera and surrounding tissues will help to accurately assess the severity of scleritis and provide quantitative indicators for the choice of treatment.
UNASSIGNED: We retrospectively analyzed the thickness of sclera and ciliary bodies detected by ultrasound biological microscopy (UBM) in noninfectious anterior scleritis patients who subsequently were treated with topical or systemic treatment, and visited our hospital from March 2014 to March 2021. Age- and sex-matched normal individuals were used as controls.
UNASSIGNED: A total of 185 patients (50 males and 135 females) with noninfectious anterior scleritis and 84 (31 males and 53 females) controls were included. In patients with noninfectious scleritis, the thickness of sclera and the ciliary body were significantly greater than those in the control group (p < 0.05). Before treatment, the thickness of sclera and the ciliary body in systemic treatment group was significantly higher than that in topical treatment group (p < 0.05). After treatment, both thicknesses of sclera and the ciliary body decreased significantly (p < 0.05). The ratio of ciliary body thickness from the site of inflammation to the normal position was significantly higher in the systemic treatment group than in the topical treatment group.
UNASSIGNED: UBM quantitatively shows a decrease in AST/CBT in patients with anterior scleritis after treatment. The ratio of ciliary body thickness at the site of information to that at the normal position may be a reference for the choice of treatment.

OBJECTIVE: To evaluate the outcomes of adalimumab (ADA) treatment of patients with non-infectious uveitis and scleritis, focusing on efficacy, retention rate, and safety.
METHODS: This retrospective, clinical cohort study included 62 patients (104 eyes) with active ocular inflammation treated with ADA. Primary outcomes were efficacy and cumulative drug retention rate (DRR) of ADA. The secondary outcomes included changes in ocular inflammatory parameters, changes in best-corrected visual acuity (BCVA) and central macular thickness (CMT), corticosteroid-sparing effect, impact of concomitant use of disease-modifying antirheumatic drug (DMARD) and ADA as first or ≥2nd biotherapy line on DRR, and adverse events.
RESULTS: Forty-five patients (72.6%) achieved inactive disease at the end of follow-up. DRR at 6, 12, 24, and 48 months was 96.8%, 89.2%, 63.1%, and 63.1%, respectively. Of the 18 patients whose bi-weekly ADA treatment was escalated to weekly ADA due to primary or secondary inefficacy, 10 patients had inactive disease finally. BCVA improved (p < 0.001) and CMT decreased (p < 0.001) significantly at 6, 12, and 24 months after ADA therapy compared to baseline. Percentage of patients treated with ≥10 mg/day corticosteroid (61.3% vs. 6.4%) and DMARDs combined with ADA (46.8% vs. 37.1%) were lower at 6 months than at baseline. Concomitant DMARDs (p = 0.579) and use of ADA as first or ≥2nd biotherapy line (p = 0.527) had no significant effect on DRR. Most common adverse event was tuberculosis-related infections.
CONCLUSIONS: ADA seems to be effective and safe with good DRR to control ocular inflammation. Escalation to weekly ADA treatment may be an effective option in patients with primary or secondary inefficacy.

Cogan syndrome is an autoimmune disease characterized by vestibular symptoms, bilateral sensorineural hearing loss, and inflammatory ocular manifestations, which may be accompanied by systemic vasculitis. We herein present the case of a patient with bilateral sensorineural hearing loss who presented with pain over her cochlear implantation incision site. She was later found to have evidence of ocular disease and underlying vasculitis leading to a diagnosis of Cogan syndrome.

Uveitis is a sight-threatening disease that poses a heavy burden on the quality of life. The treatment of uveitis has been revolutionized in the past two decades. Most remarkable among these is the emergence of biologics, which have shown to be effective and safer therapeutic option in noninfectious uveitis. Biologics are very useful when conventional immunomodulator therapy has failed or has been poorly tolerated. The most widely used biologics are tumor necrosis factor-α inhibitors (infliximab and adalimumab) with promising results. Other drugs include anti-CD20 inhibitors (rituximab), interleukin-6R-inhibitor (tocilizumab), interleukin-1R-inhibitor (anakinra), and Janus-associated kinase inhibitor (tofacitinib).
UNASSIGNED: A retrospective review of all cases of noninfectious uveitis and scleritis presenting to our center from July 2019 to January 2021 and had been treated with biological therapy were included.
UNASSIGNED: We included 12 eyes of 10 patients. The mean age was 42.10±9.71 years. Anterior nongranulomatous uveitis comprised 70% of the cases and the most common etiology of anterior uveitis was spondyloarthritis (seven cases among which five cases were nonradiographic) axial spondyloarthritis (human leukocyte antigen B27 positive) followed by radiographic axial spondyloarthritis (two cases). The first line of treatment in all cases was conventional synthetic disease-modifying antirheumatic agents among which 50% (n=5) had received methotrexate (≥15 mg/week). As a second line of treatment, one or more biologics was used. Majority of the patients received oral tofacitinib 50% (n=5) followed by Inj adalimumab 30% (n=3). One case of Behcet\'s disease required sequential biologics (Inj adalimumab followed by oral tofacitinib). All patients tolerated and responded well to the treatment and no recurrences were observed after discontinuation of biologics drugs during the follow-up period of 1 year.
UNASSIGNED: Biologics are a relatively safe and effective modality of treatment in refractory, recurrent noninfectious uveitis.

BACKGROUND: Ocular manifestations of granulomatosis with polyangiitis (GPA) have been reported in a limited number of studies and with largely variable frequency. Here we report on the clinical, diagnostic, and therapeutic features of a cohort of 63 GPA patients, with particular regard to 22 of them with ophthalmic involvement (35%).
METHODS: Clinical manifestations, results of immunological findings, histopathological pictures, imaging data, Birmingham Vasculitis Activity Score, therapeutic regimens, and outcomes were retrospectively analyzed. At diagnosis, in addition to a structured clinical assessment, all patients underwent a comprehensive ophthalmologic examination.
RESULTS: The most frequently involved organs were kidneys, lungs, ear/nose/throat, and eyes. Ocular manifestations were bilateral in 32%. The three most commonly diagnosed ophthalmologic manifestations were scleritis (36%), retro-orbital pseudotumor or orbital mass (23%), and episcleritis (13%). Ocular and systemic involvement were simultaneously present at onset in 41% of the patients; systemic involvement was followed by ocular lesions in 36%; ocular inflammation was followed by systemic manifestations in 18%; and an orbital mass in the absence of systemic disease characterized 5%. Glucocorticoids plus cyclophosphamide and glucocorticoids plus rituximab were the combined therapies most frequently employed during remission induction and remission maintenance, respectively. Persistent ophthalmologic and extra-ocular remissions were achieved in 77 and 64% of the patients, respectively. One to three systemic relapses were diagnosed in 7 patients (31.8%). At the last follow-up, a visual outcome 20/40 or better in 31 (70%) of 44 eyes was determined.
CONCLUSIONS: The eye was involved in over one third of our patients with GPA. Increased awareness, early diagnosis, and multi-specialty collaboration are critical in achieving a favorable outcome of GPA.

OBJECTIVE: This study aims to describe the clinical characteristics of scleritis in a large cohort of Colombian patients and identify factors associated with the clinical presentation.
METHODS: Retrospective case series of patients with scleritis from 2015 to 2020. Clinical records were obtained from seven uveitis referral centers in Colombia. Patients with a diagnosis of episcleritis were excluded.
RESULTS: We evaluated 389 patients with scleritis (509 eyes). There was a female predominance (75.6%) with a mean age of 51 ± 15 years. Most cases were noninfectious (94.8%) and unilateral (69.2%). The most frequent type of inflammation was diffuse anterior scleritis (41.7%), followed by nodular scleritis (31.9%) and necrotizing scleritis (12.3%). Systemic autoimmune diseases were found in 41.3% of patients, the most common being rheumatoid arthritis (18.5%) and granulomatosis with polyangiitis (5.9%). Polyautoimmunity was found in 10.4% of those with a systemic autoimmune disease. The most frequent treatment was systemic steroids (50.9%), followed by systemic NSAIDs (32.4%). Steroid-sparing immunosuppression was required in 49.1% of patients. Systemic autoimmune diseases were more common in patients with necrotizing scleritis and those older than 40 years of age. Best-corrected visual acuity of 20/80 or worse at presentation was more common in necrotizing scleritis and subjects with associated uveitis, ocular hypertension, or who were over 40 years of age.
CONCLUSIONS: This is the first study in Colombia and the largest in Latin America describing the clinical characteristics and presentation patterns of scleritis. The most common presentation was in females, with unilateral, anterior diffuse noninfectious scleritis. Systemic autoimmune diseases and polyautoimmunity were frequent, as was the need for steroid-sparing immunosuppression. Age over 40 and necrotizing scleritis were associated with higher odds of having a systemic autoimmune disease and worse visual acuity at presentation.

This nationwide, population-based, retrospective, matched case-control study included 111,960 newly diagnosed patients with scleritis who were identified by the International Classification of Diseases, Ninth Revision, Clinical Modification code 379.0, selected from the Taiwan National Health Insurance Research Database. Demographic characteristics, Sjögren syndrome, and comorbid conditions within 1 year before the scleritis diagnosis were examined using univariate logistic regression analyses, and a paired t-test was used for continuous variables. Adjusted logistic regression was used to compare the prognosis odds ratio (OR) of the patients with scleritis with the controls. After adjustment for confounders, patients with Sjögren syndrome were remarkably more likely to have scleritis than the controls (OR = 33.53, 95% confidence interval (CI) = 27.43-40.97, p < 0.001). Other conditions found to have increased odds of scleritis included post ocular pterygium, glaucoma, and scleral surgery (OR  =  4.01, 95% CI  =  3.64-4.43; OR  =  3.16, 95% CI  =  2.24-4.47; OR  =  6.83, 95% CI  =  5.34-8.74, respectively); systemic infections, such as syphilis, tuberculosis, and a human herpes viral infection (OR  =  4.01, 95% CI  =  2.93-5.50; OR  =  2.24, 95% CI  =  1.94-2.58; OR  =  8.54, 95% CI  =  8.07-9.03, respectively); and systemic diseases, such as rheumatoid arthritis, granulomatous vasculitis, systemic lupus erythematosus, ankylosing spondylitis, and gout (OR  =  2.93, 95% CI  =  2.66-3.23; OR  =  7.37, 95% CI  =  3.91-13.88; OR  =  3.18, 95% CI  =  2.63-3.85; OR  =  5.57, 95% CI  =  4.99-6.22; OR  =  2.84, 95% CI  =  2.72-2.96, respectively). The results strongly support an association between Sjögren syndrome, post ocular surgery, systemic infection disease, systemic autoimmune disease, and scleritis.

Chronic inflammatory diseases can cause significant psychosocial stress in affected patients. Few studies have examined the psychological effects of ocular inflammatory disease and no studies have examined the psychological effects of scleritis. In this study we evaluate the prevalence of mental health disorders in scleritis patients and we conduct a comprehensive review of the literature on the mental health effects of ocular inflammatory diseases. 162 patients (195 eyes) presenting to a tertiary care center with scleritis were identified. At least one comorbid mental health disorder was diagnosed in 35 patients (21.6%), most commonly major depression in 11.7%, generalized anxiety disorder in 9.3%, and substance use disorder in 6.2%. There were no significant differences in the length of an episode of scleritis or in the probability of symptom resolution between patients with a mental health disorder and other patients. In a review of the literature, 30 manuscripts met the inclusion criteria. The majority of manuscripts (83.3%) were focused on uveitis patients. Eight of these studies were focused on patients with uveitis in the context of systemic disease. The most commonly reported mental health disorders reported were anxiety and depression. An average of 31.3% of patients with ocular inflammatory disease had depression and 35.0% had anxiety. Similar to other chronic illnesses, ocular inflammatory disease may be a significant psychosocial stressor. Future studies will further elucidate the relationship between these diseases and mental health.

Ocular involvement in pemphigus vulgaris (PV) is relatively rare. The conjunctiva and eyelids are considered the most common affected sites in ocular pemphigus. Scleritis is rarely reported as a manifestation of PV. We present a case report of anterior scleritis as a manifestation of PV and its response to rituximab therapy.