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Purpose: To describe the frequency of uveitis recurrences in patients with non-infectious uveitis treated with the biosimilar infliximab-dyyb. Design: Retrospective case series. Methods: Records of uveitis patients treated with the biosimilar infliximab-dyyb between 2016 and 2022 at two institutions were reviewed. Data extracted included patient demographics, diagnosis, previous originator infliximab use, additional immunosuppression medications, infliximab-dyyb use, reason for switch, disease activity, and follow-up time. Results: A total of 14 patients were identified. Seven patients were switched from originator infliximab to a biosimilar for nonmedical/non-ocular reasons (insurance prompted the switch). One patient was started directly on infliximab-dyyb due to active joint disease despite well-controlled uveitis. None of these eight patients developed inflammation after the switch. Six patients were started directly on infliximab-dyyb due to poorly controlled uveitis. Of these, five patients achieved disease quiescence during follow-up. The mean dose of originator was 1.79 mg/kg/week, with a median dosing schedule of 4 weeks prior to therapy with infliximab-dyyb. The mean final infliximab-dyyb dosage was 1.81 mg/kg/week, with a median dosing schedule of 4 weeks. Conclusion: Infliximab-dyyb appears to be efficacious in achieving and maintaining uveitis control. [ABSTRACT FROM AUTHOR]

PurposeMethodsResultsConclusionTo report two cases of bilateral blepharokeratoconjunctivitis associated with hidradenitis suppurativa (HS).Case report and literature review. The clinical courses of two patients with HS, including ocular presentation and medical management, are described.Two female patients aged 18 and 23-years-old with severe HS presented with bilateral blepharokeratoconjunctivitis. Shared slit lamp findings included bilateral corneal neovascularization and inferior corneal thinning. Systemic immunosuppression was needed in the first case, which resulted in improvement in the patient’s ophthalmic and dermatological findings.We report two cases of bilateral blepharokeratoconjunctivitis in two patients with severe HS. To our knowledge, this association has not previously been described in the literature. Clinicians should be aware of this association given its potentially visually devastating manifestations and the need for early therapeutic interventions. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the prevalence and patterns of complementary and alternative medicine (CAM) use among patients with inflammatory eye disease. Material-Method: This cross-sectional study was conducted over three months through direct interviews with 150 consecutive patients at a tertiary care clinic specializing in inflammatory eye diseases. Sociodemographic data and clinical characteristics were extracted from clinical records for analysis. Descriptive and comparative analyses were performed using IBM SPSS Statistics software. Results: In this cohort, 110 of the 150 patients (73.3%) reported employing complementary and alternative medicine (CAM) strategies to mitigate their ocular conditions. The CAM interventions included vitamin supplementation (n=53, 35%), herbal remedies (n=41, 27%), acupuncture (n=23, 15%), and leech therapy (n=11, 7%). Notably, a significant portion—roughly one in three patients—engaged in multiple CAM therapies. Statistical analysis revealed a robust association between CAM applications and several demographic and clinical variables. Females were more likely to use CAM (p=0.04), as were individuals with higher occupational standing (p=0.03) and income levels (p=0.03). Furthermore, patients diagnosed with uveitis exhibited a higher propensity for CAM usage than those with other inflammatory eye diseases (p=0.03). Patients diagnosed with any inflammatory eye disease (≥5 years) exhibited a higher propensity for CAM use (0<0.01). Most patients using CAM therapies were convinced of their t herapeutic benefits to eye health, and adverse effects were infrequent. Referrals by healthcare providers constituted 57% of CAM informational resources, underscoring the need for healthcare professionals to be proactive in discussing CAM use with patients . Conclusion: The employment of CAM modalities is prevalent among patients with inflammatory eye diseases, indicating an essential role for these therapies in this patient population. The influence of CAM on disease trajectory, potential side effects, and interactions with established immunosuppressive treatments necessitates that ophthalmologists and other healthcare professionals maintain a comprehensive understanding of CAM usage to ensure optimized and safe patient care. [ABSTRACT FROM AUTHOR]

Patient Adherence to Immunosuppressive Therapy for Chronic Inflammatory Eye Disease To investigate adherence rates to immunosuppressive therapy (IMT) for treatment of noninfectious inflammatory eye disease (IED), adherence and disease control, and factors associated with nonadherence. Retrospective review of medical charts from 2015 to 2020 was conducted on patients with IED at 6 months, 1 and 2 years after initiation of IMT. Of 183 patients, adherence rates at 6 months and 1 year were 70% and 58% by 2 years. Eighty-two percent, 78%, and 65% of patients with disease quiescence were adherent at 6 months, 1 and 2 years, respectively. Adherent patients have 1.86 (95% CI 1.09, 3.20) times greater likelihood for disease control compared to nonadherent. Primary reason for nonadherence was patient self-discontinuation. No specific factors were associated with nonadherence. Patients on IMT for IED had steady adherence rates up to 1 year, with decreased adherence at 2 years. Adherence to IMT significantly correlates with disease quiescence. [ABSTRACT FROM AUTHOR]

Interstitial keratitis is defined as nonulcerative inflammation of the corneal stroma resulting from a bacterial or viral infection, parasitic antigen, or autoimmune response. Hidradenitis suppurativa is an inflammatory skin condition that presents with painful and inflamed boils in apocrine gland-bearing regions of the body, typically with onset around puberty, and ocular comorbidities exist in only a small percentage of these patients. Herein, we describe a unique ocular presentation of hidradenitis suppurativa including pronounced bilateral interstitial keratitis and association with high titer c-ANCA (antineutrophil cytoplasmic antibodies). [ABSTRACT FROM AUTHOR]

Background: Support groups in ophthalmology may be of utmost importance for patients and caregivers. We aimed to characterise members of an online support group for patients and parents of children with inflammatory eye disease and assess the members' expectations and perceived benefits. Methods: A survey based on a voluntary, anonymous web questionnaire was distributed to the members of the 'Lirot' association online support group. The questionnaire included demographic, ocular and systemic information, functional and psychosocial data, support group use patterns, expectations and perceived benefits. Analysis was done for patients and parents. Results: Out of 67 respondents, 43 (64%) were patients, and 24 (36%) were parents. Fifty‐eight (88%) were women. The mean age of respondents was 42.9 ± 1.34 years. Anterior uveitis was the most common, and most patients had good visual acuity (<0.3 logMAR). Parents reported higher anxiety levels (p = 0.044) and a more significant effect of their child's disease on their general function (p = 0.005). Most members sought several experts' opinions, psychological treatments and alternative medicine. All members used the WhatsApp group, while only approximately half used Facebook (p < 0.001). Members' expectations were fulfilled for social support, a sense of shared experience and being able to help others, but not for receiving information (p < 0.001). All members reported being willing to recommend the group to others. Conclusions: Support group participation benefited group members. We suggest that ophthalmologists encourage their inflammatory eye disease patients to use online support groups, which may significantly improve their well‐being. [ABSTRACT FROM AUTHOR]

Retinal vasculitis (RV) refers to an entity in which the retinal vasculature is inflamed, frequently with indications of inflammation elsewhere in the eye. Non-infectious RV can be idiopathic or associated with systemic disease, ocular conditions, and malignancy. It can also be classified based on the vessel affected: artery, vein, or both. Due to the lack of strong evidence-based treatment trials and algorithms for RV, physicians must often rely on their experience, which creates great variability in treating this entity. This article provides an overview of various treatment modalities used in the management of non-infectious RV, with a focus on immunomodulatory therapies. We outline a potential stepwise approach of starting with steroids to control the acute inflammation and subsequently changing to immunomodulatory therapy (IMT) for long-term treatment. [ABSTRACT FROM AUTHOR]

To determine the utility of routine screening ophthalmic exam in patients with systemic sarcoidosis and no history of uveitis. Prospective, single-center, observational study conducted at Northwestern University from October 11, 2012 to October 1, 2020 of new patients with biopsy-proven systemic sarcoidosis and no history of uveitis, referred by medical subspecialists for screening ophthalmic exam. Forty-nine patients, with mean age of 51 ± 8.7 years, 59% female, 47% African American, 43% Caucasian, were enrolled. The majority (55%) had no ocular symptoms. The most common location of ocular involvement was the adnexa, in the form of conjunctival nodules (62%) and aqueous tear deficiency (23%). Intraocular inflammation was detected in 6 patients (13%); only 2 had active disease requiring treatment (4%). No asymptomatic patient had ocular involvement necessitating treatment. Screening exams are indicated in sarcoidosis patients with ocular symptoms. No benefit of screening was demonstrated in asymptomatic patients. [ABSTRACT FROM AUTHOR]

Purpose: To describe a unique case of peripheral ulcerative keratitis secondary to isolated, severe hidradenitis suppurativa (HS). Observation: A 31-year-old male with HS presented with a red painful right eye with best corrected visual acuity of count fingers at 3 feet with peripheral corneal thinning, inferior descemetocele, and adjacent infiltrate. Work-up revealed negative corneal cultures and positive ANA, ANCA, and rheumatoid factor without other autoimmune or rheumatologic history or symptomatology. He was treated with topical corticosteroids with improvement until he was lost to follow-up before tumor necrosis factor-a inhibitor therapy could be started. Upon re-presentation, he was found to have corneal perforation. Conclusions and importance: Coexistence of inflammatory eye disease and HS is known but rare, and most commonly manifests as anterior uveitis. Here we present a unique case of peripheral ulcerative keratitis secondary to HS and demonstrate the importance of ophthalmologists’ familiarly with this systemic disease and its variety of ocular manifestations.

Purpose : This study aims to report the long-term outcomes of uveitis-associated optic disc and epiretinal neovascularization (NV) treated with immunomodulatory therapy alone. Methods: This is a retrospective, multi-center chart review conducted at Northwestern University (Chicago, IL) and San Raffaele Scientific Institute (Milan, Italy) from 2014 to 2021 of patients with optic disc and/or retinal neovascularization associated with uveitis. The data collected included age at the time of NV detection, gender, medications, and follow-up period. Imaging was reviewed if available. Results: Eight eyes of six patients were identified. The mean age was 22 years (range 10–52 years); the median follow-up was 3 years (range 6 months to 7 years). All eyes presented with active NV at the time of uveitis onset; 7 eyes were treatment-naïve. None had clinical or angiographic evidence of retinal ischemia. All patients received a variable combination of local steroids, systemic steroids, and systemic immunosuppression. Complete resolution of uveitic NV occurred in all eyes within a median of 8 weeks (ranging 2–20 weeks) from initiating treatment. No NV recurrence was noted. Conclusion: Immunomodulatory therapy alone may be successful in achieving long-term control of uveitis-associated NV, without the use of destructive measures. [ABSTRACT FROM AUTHOR]

Scleritis is a manifestation of inflammatory eye disease that involves the sclera. It can be divided into multiple subtypes, including diffuse anterior, nodular anterior, necrotizing, and posterior scleritis. In many cases, scleritis is restricted to the eye; however, it can occur in the context of systemic illness, particularly autoimmune and infectious conditions. Patients with autoimmune conditions, such as rheumatoid arthritis, inflammatory bowel disease, systemic lupus erythematosus, and polyangiitis with granulomatosis, may develop scleritis flares that may require topical and systemic therapy. Initial therapy typically involves oral nonsteroidal anti-inflammatory drugs (NSAIDs); however, it is important to address the underlying condition, particularly if systemic. Other treatment regimens typically involve either local or systemic steroids or the use of immunomodulatory agents, which have a wide range of efficacy and documented use in the literature. There is a myriad of immunomodulatory agents used in the treatment of scleritis including antimetabolites, calcineurin inhibitors, biologics, and alkylating agents. In this review, we highlight the various subtypes of noninfectious scleritis and explore each of the mainstay agents used in the management of this entity. We explore the use of steroids and NSAIDs in detail and discuss evidence for various immunomodulatory agents. [ABSTRACT FROM AUTHOR]

Abstract The recent introduction of biological agents has revolutionized the treatment of chronic immune-inflammatory diseases; however, this new therapy did not come without significant side effects. Through large controlled studies indicating decrease in the number of uveitis flares, the role of TNF inhibitors therapy for non-infectious uveitis gained more ground. Paradoxically to its therapeutic effect, there are reports associating these drugs with the onset or recurrence of inflammatory eye disease. A number of studies have suggested possible roles for anti-TNF-α agents in precipitating or worsening an underlying inflammatory process, including the hypothesis of a disequilibrium in cytokine balance, but to date the mechanisms responsible for these adverse events are not fully understood. A PubMed literature search was performed using the following terms: ophthalmic complication, uveitis, inflammatory eye disease, optic neuritis, neuropathy, adverse events, anti-TNF, TNF alpha inhibitor, infliximab, etanercept, adalimumab, golimumab, certolizumab, and biologics. The data presented in this study was mainly derived from the use of TNF inhibitors in rheumatology, essentially because these drugs have been used for a longer period in this medical field. Many of the ocular adverse events reported on this review may be considered a paradoxical effect of anti-TNF therapy. We found a variety of data associating new onset of uveitis with anti-TNF therapy for rheumatic conditions, predominantly under etanercept. In conclusion, although there is increasing data on ocular adverse events, it remains to be seen whether the suggested link between TNF inhibitors and the onset of ocular inflammation is substantiated by more quality data. Nevertheless, the awareness of potential treatment side effects with anti-TNF should be highlighted.

This paper provides the summaries on nine (9) important and clinically relevant publications in the field of uveitis. The first is on the standardization of uveitis nomenclature, more popularly known by its acronym - SUN, which was a result of an international workshop participated by uveitis experts in 2004. Five (5) papers were large, multicenter, clinical trials that demonstrated safety and efficacy of two (2) corticosteroids delivery devices (dexamethasone implant [Ozurdex] and fluocinolone acetonide implant [RetisertTM]) and one (1) immunomodulatory drug (adalimumab). The POINT trial compared various delivery approaches when using corticosteroids for the treatment of uveitic macular edema. The FAST trial compared two (2) durable and commonly-prescribed steroidsparing immunosuppressants, methotrexate and mycophenolate mofetil, for the treatment of non-infectious uveitis. Lastly, the SITE study, which was a large retrospective cohort study, determined the risks of overall and malignancy-related deaths among patients with inflammatory eye diseases receiving systemic immunosuppressants. Findings of these studies provide basis and rationale for the care and management of patients with uveitis and lay the groundwork for future research. [ABSTRACT FROM AUTHOR]

The global impact imposed by the coronavirus disease 2019 (COVID-19) pandemic may be soon alleviated by the introduction and worldwide dissemination of safe and effective vaccines. This expedited timetable for development and approval of COVID-19 vaccines is an unprecedented extraordinary, concerted achievement by the scientific community. With the pending global rollout of vaccines, each with different mechanisms of action, physicians of various specialties will need to identify vulnerable patient groups for special considerations or advice. In this commentary, we analyse the important considerations for COVID-19 vaccines in patients with inflammatory eye diseases. Scrutiny of immunogenicity and adverse effects, particularly antibody-dependent enhancement, would better help in counselling these patients undergoing vaccination. More research on pharmacovigilance would allow for tailored guidelines and personalised management strategies. [ABSTRACT FROM AUTHOR]

To report a case of severe bilateral phlyctenular keratoconjunctivitis (PKC) associated to hidradenitis suppurativa (HS). A 26-year-old male with reactivation of HS in the last few months presented with concurrent pain and vision loss secondary to bilateral PKC resistant to topical treatment. There were no other infectious or autoimmune disorders. Systemic immunosuppression was needed, with simultaneous improvement of the ophthalmological and dermatological findings. Different inflammatory eye diseases have been reported in the context of HS. Acute inflammation in HS reactivation would trigger an autoimmune response, acting as a common causal mechanism in this association. We have reported a new case of inflammatory eye disease – HS in the form of PKC, not previously described in the literature, and consistent with immune dysregulation where the systemic Staphylococcus aureus burden due to HS may act as an additional causal factor. [ABSTRACT FROM AUTHOR]

Abstract Hidradenitis suppurativa (HS) is a relatively common chronic inflammatory disease with immune dysregulation. While eye manifestations of HS are rare, a dilemma arises when these patients seek treatment for refractive errors. Although excimer laser surgery can be safely performed in patients with autoimmune and immune-mediated inflammatory disease, there are caveats. Aside from the routine laser-assisted in situ keratomileusis (LASIK) screening tests, in some instances, we recommend additional screening tests in patients with HS, such as dry eye tests, consultation with specialists regarding HS diagnosis and treatment, careful assessment of the eyelids and periorbital structures, and thorough history of past and current lesions and treatments. After these patients undergo LASIK, careful, frequent, and long-term follow-up is necessary. Any adverse event or complication should be managed immediately. Funding Research to Prevent Blindness funded the study. Hoopes Vision funded the Rapid Service Fees.

The recent introduction of biological agents has revolutionized the treatment of chronic immune-inflammatory diseases; however, this new therapy did not come without significant side effects. Through large controlled studies indicating decrease in the number of uveitis flares, the role of TNF inhibitors therapy for non-infectious uveitis gained more ground. Paradoxically to its therapeutic effect, there are reports associating these drugs with the onset or recurrence of inflammatory eye disease. A number of studies have suggested possible roles for anti-TNF-α agents in precipitating or worsening an underlying inflammatory process, including the hypothesis of a disequilibrium in cytokine balance, but to date the mechanisms responsible for these adverse events are not fully understood. A PubMed literature search was performed using the following terms: ophthalmic complication, uveitis, inflammatory eye disease, optic neuritis, neuropathy, adverse events, anti-TNF, TNF alpha inhibitor, infliximab, etanercept, adalimumab, golimumab, certolizumab, and biologics. The data presented in this study was mainly derived from the use of TNF inhibitors in rheumatology, essentially because these drugs have been used for a longer period in this medical field. Many of the ocular adverse events reported on this review may be considered a paradoxical effect of anti-TNF therapy. We found a variety of data associating new onset of uveitis with anti-TNF therapy for rheumatic conditions, predominantly under etanercept. In conclusion, although there is increasing data on ocular adverse events, it remains to be seen whether the suggested link between TNF inhibitors and the onset of ocular inflammation is substantiated by more quality data. Nevertheless, the awareness of potential treatment side effects with anti-TNF should be highlighted. [ABSTRACT FROM AUTHOR]

Hidradenitis suppurativa (HS) is a relatively common chronic inflammatory disease with immune dysregulation. While eye manifestations of HS are rare, a dilemma arises when these patients seek treatment for refractive errors. Although excimer laser surgery can be safely performed in patients with autoimmune and immune-mediated inflammatory disease, there are caveats. Aside from the routine laser-assisted in situ keratomileusis (LASIK) screening tests, in some instances, we recommend additional screening tests in patients with HS, such as dry eye tests, consultation with specialists regarding HS diagnosis and treatment, careful assessment of the eyelids and periorbital structures, and thorough history of past and current lesions and treatments. After these patients undergo LASIK, careful, frequent, and long-term follow-up is necessary. Any adverse event or complication should be managed immediately. Funding: Research to Prevent Blindness funded the study. Hoopes Vision funded the Rapid Service Fees. [ABSTRACT FROM AUTHOR]

Objective: The objective of this study was to compare the frequency of comorbidities among patients with ax-SpA in the general population and to evaluate the impact of these comorbidities on the functional status and quality of life. Methods: Patients with ax-SpA fulfilling the modified New York 1984 criteria for Ankylosing Spondylitis (AS) and/or Assessment of SpondyloArthritis International Society (ASAS) 2009 criteria for patients with ax-SpA belonging to the ESPAXIA cohort ("Estudio de eSPondiloartritis Axial Irep Argentina") were included. Data regarding sociodemographics, comorbidities, and disease characteristics were recorded. Statistical analysis included descriptive statistics using the student t-test, Chi-square, and Fisher's exact test. Multiple logistic regression analysis was performed. A p value <0.05 was considered significant. Results: In total, 86 patients were included, 80% were males with a median age of 46 years (interquartile range [IQR]: 32-58) and a median disease duration of 19 years (IQR: 13-31). The most frequent comorbidities reported were hypertension (26.7%), gastritis (25.6%), dyslipidemia (24.4%), gallstone (12.8%), nephrolithiasis (11.6%), anemia (10.5%), hypothyroidism (7%), and type 2 diabetes (6%). The prevalence of these comorbidities in patients with ax-SpA was similar to that observed in the general population, with the exception of a higher frequency of nephrolithiasis among patients with ax-SpA (11.6% in ax-SpA vs 3.96% in the general population). We further categorized the study population into three groups: patients with no comorbidities, those with 1 or 2 comorbidities, and those with =3 comorbidities. The presence of =3 comorbidities was associated with older age, longer disease duration, worse disease activity, functional status, and quality of life as compared with the patients without comorbidities. In multivariate analysis, older age was the only variable independently associated with the presence of comorbidities. Conclusion: The frequency of comorbidities in the ax-SpA cohort was high, and the only variable associated with a higher prevalence of comorbidities was older age. Nephrolithiasis was more frequent in the patients with ax-SpA than that reported in the general population. [ABSTRACT FROM AUTHOR]

Objective: Hidradenitis suppurativa (HS) is an inflammatory skin condition that can cause profound morbidity. Patients can present with recurrent nodules, sinus tract formation, abscesses, and/or scarring, mainly affecting the intertriginous areas. Case reports have documented the coexistence of HS and inflammatory eye disease (IED). Herein, we aimed to assess the types of IED associated with HS and the outcomes of IED treatment in patients with HS. Methods: All the cases with a diagnosis of HS and any IED were identified. Patients with episcleritis and sicca were excluded, and only those with chronic IED were included. An independent ophthalmologist reviewed notes to ensure accuracy of IED diagnosis. Demographics, IED patterns, comorbidities, treatments, and outcomes were retrieved from patient charts. Results: Twenty patients [16 females (80%); 12 African Americans (60%)] were included in this study after the exclusion of 436 cases due to lack of data. The mean age at the diagnosis of HS and IED was 42.1 and 43.6 years, respectively. Thirteen patients had uveitis (65%), six had scleritis (30%), and one had peripheral ulcerative keratitis. Thirteen out of 20 patients (65%) had multiple autoimmune and/or inflammatory comorbidities, including inflammatory bowel disease, rheumatoid arthritis, ankylosing spondylitis, multiple sclerosis, and lupus. Seven patients (35%) did not have any comorbid inflammatory/autoimmune conditions. Conclusion: One-third of the patients with HS and IED did not have any autoimmune or inflammatory comorbidity that could explain the eye involvement. The potential association between HS and IED might be a manifestation of a common immune dysregulation phenomenon. Furthermore, the management of IED required an escalation of therapy to systemic immunosuppressive agents in 70% of patients with HS. [ABSTRACT FROM AUTHOR]

Purpose: To report the pattern of uveitis in patients attending a tertiary uveitis service in Sydney, Australia. Methods: The charts of patients seen between January 2009 and December 2015 were retrospectively reviewed. Data pertaining to patient demographics, eye examination on presentation, work-up and final diagnoses were collected. Results: The total number of patients with uveitis seen over this period was 1165. There were 650 males (56%) and 515 females (44%). There were 838 patients aged 17–60 years (72%) and 327 patients aged >60 years (28%). Uveitis was anterior in 735 patients (63%), posterior in 234 patients (20%), pan in 109 patients (9%), and intermediate in 87 patients (8%). The most common associations were HLA-B27+ve (264 patients; 22.8%), sarcoidosis (78 patients; 6.7%) and Fuchs (33 patients; 2.8%), while VZV (51 patients; 4.4%), HSV (49 patients; 4.2%), tuberculosis (49 patients; 4.2%) and toxoplasmosis (48 patients; 4.1%) were the most common infectious causes of uveitis. No identifiable association was found in 389 patients (33.4%). HLA-B27 was more common in the younger age group compared with the older age group (p<0.001, χ2-test), but there was no difference between the age groups for no identifiable cause (p value 0.24) and sarcoidosis (p value 0.08). Conclusions: This retrospective case review reveals a broad spectrum of uveitis in a tertiary referral service in Sydney, Australia. It is comparable with other major studies around the world. [ABSTRACT FROM AUTHOR]

Objective. To characterize the clinical correlates and outcome of inflammatory ocular disease (IOD) among patients with ANCA-associated vasculitides (AAV). Methods. Medical records of potential cases of AAV seen at Mayo Clinic from 2003 to 2013, inclusive, were reviewed to identify confirmed cases meeting the diagnosis of AAV using the Chapel Hill Consensus Conference 2012 descriptors. Records of confirmed cases of AAV were then further reviewed for IOD, and clinical characteristics, treatment and outcomes abstracted. Results. A total of 1171 confirmed cases of AAV were identified of which 183 patients (mean age 49.0 years; 51% female; 95% Caucasian) had IOD. The most common manifestation of IOD was injection of the eye (57%) followed by eye pain (46%) and visual acuity loss (18%). Scleritis was the most common type of IOD (22%) followed by episcleritis (21%), orbital inflammation (18%), lacrimal duct stenosis (10%) and uveitis (9%). Oral glucocorticoids were used to treat IOD in the majority of patients (96%). CYC and rituximab were the most frequently used immunosuppressive agents (54 and 36%, respectively). Of those with orbital inflammation, 52% underwent therapeutic surgical intervention. Clinical remission of IOD was achieved in 91% of patients but relapses were seen in 23%. Significant visual acuity loss was observed in only six patients. Conclusion. IOD is a common manifestation of AAV and seen in about 16% of patients with AAV. Scleritis, episcleritis and orbital inflammation are the most common subtypes. Most patients respond well to glucocorticoids and immunosuppression, but relapse of IOD is common. [ABSTRACT FROM AUTHOR]

Introduction: Inflammatory eye diseases (IED), which encompasses uveitis and scleritis, often require systemic immunosuppressive therapy (IMT) to induce disease control or remission. Immunosuppressive therapy, including glucocorticoids, has been implicated in accelerating atherosclerosis and increasing cardiovascular morbidity and mortality in patients with rheumatic diseases or solid organ transplant recipients. Areas covered: Literature review conducted with the use of PubMed, MEDLINE and Cochrane library for peer reviewed articles then stratified for strength of evidence in reference to traditional risk factors such as hypertension, lipid profile and insulin resistance. Evidence of cardiovascular events were included. Commonly used immunosuppressive agent used in the treatment of IED including azathioprine, methotrexate, mycophenolate, cyclosporine, tacrolimus and the biologics. Each immunosuppressive agent was considered in terms of effects on hypertension, lipid profile, insulin resistance and cardiovascular outcomes. Expert commentary: Cyclosporine is associated with increased risk of hypertension and tacrolimus with insulin resistance. There is little evidence of cardiovascular disease with the use of methotrexate, azathioprine or mycophenolate. There is some evidence that the newer biologics may have protective effects on cardiovascular parameters. However, there is heterogeneity of the study populations and limited evidence in the ophthalmological setting. [ABSTRACT FROM PUBLISHER]

The association of sensorineural hearing loss and vertigo with inflammatory eye disease, usually interstitial keratitis, has been called Cogan’s syndrome. The pathogenesis of Cogan’s syndrome is unknown, but it has been assumed to be an immune mediated disorder with vasculitis. The histopathology of the inner ear in Cogan’s syndrome has been described in 6 case reports. Although common pathologic findings in these reports include degeneration of the auditory and vestibular neuroepithelium, endolymphatic hydrops, fibrosis, and new bone formation, direct pathologic evidence of a vasculitis has not been published. A possible reason for this failure to identify vasculitis was a substantial delay (range, 4-40 years) between the onset of symptoms and examination of the otopathology. In the current case report, the patient had both auditory and vestibular symptoms and interstitial keratitis with a time delay of only 2 to 4 weeks between symptoms and death. Evidence of a vasculitis as a possible underlying etiology included H&E histopathology and anti-CD45 immunostaining of vessels both in the auditory and vestibular systems, supporting the hypothesis of a vasculitis as a mechanism in this disorder. [ABSTRACT FROM AUTHOR]

Background: The etiology of Autoimmune chronic uveitis (ACU) is still unknown; NOD2/CARD15 gene mutations are responsible for the Blau Syndrome and can induce uveitis in animal models. Presentation of the hypothesis: Aim of our study was to assess if NOD2/CARD15 variants have a role in the etiology or in the clinical course of patients with ACU, either idiopathic or associated with other inflammatory diseases. Testing the hypothesis: We consecutively enrolled 25 patients (19 pediatric and 6 adults) affected with ACU. For each patient medical history was reviewed and clinical data were recorded. Allelic and genotypic frequencies of NOD2/CARD15 variations were calculated in patients and matched with those of 25 healthy controls. The statistical analysis was performed. Fifteen patients showed the polymorphism P268S/SNP5 (SNP rs2066842) as heterozygous carriers while two patients were homozygous for the same polymorphism; one patient carried also the variant c647 18-16 TCT on intron 3, not previously reported in the literature. Statistical analysis for NOD2/CARD15 genotyping showed significant differences between patients and controls for allelic frequencies (p = 0.04, OR: 4.03, 95 %; CI = 1.2-13.5) but not for genotypic frequencies. We could not identify a significant phenotype-genotype correlation. Implications of the hypothesis: In our cohort of Italian patients, the NOD2/CARD15 common variant P268S/SNP5 could potentially be significantly associated with ACU. [ABSTRACT FROM AUTHOR]

Ocular inflammatory disease is often vision threatening to an individual, has significant socioeconomic impact to society and constitutes one of the more complex problems in ophthalmology. Adequate care of patients with ocular inflammatory disease requires enormous diagnostic and therapeutic efforts. Ocular immunologists need comprehensive training in several aspects of ophthalmology, and frequently, additional support from other specialties. Many reports have demonstrated the benefits of steroid-sparing agents in controlling inflammation and reducing the rate of adverse events, but it has to be stressed that poor adherence to current guidelines continues. The reasons for this are multifactorial, including unfamiliarity with the medications and concern for causing adverse events. We stress the importance of developing collaborations with rheumatologists, onocologists, allergists, dermatologists and infectious disease immunologists and adding trained ocular immunologists to faculty positions. In such ways, comprehensive ophthalmologists and those in training may gain comfort with steroid-sparing approaches to ocular inflammatory disease, reducing overreliance on chronic corticosteroids. [ABSTRACT FROM AUTHOR]

Non-infectious uveitis encompasses a group of site-threatening intraocular inflammatory diseases. Several classes of immunosuppressive medications, including corticosteroids, T cell inhibitors, antimetabolites and, more recently, biologic agents have been shown to be effective in reducing intraocular inflammation in non-infectious uveitis. However, these agents also pose significant risks if not administered and monitored properly. Further research into the pathogenic mechanisms of non-infectious uveitis will hopefully aid in the development of more targeted and safer therapies. [ABSTRACT FROM AUTHOR]

Systemic autoimmune diseases based on an immune pathogenesis produce autoantibodies and circulating immune complexes, which cause inflammation in the tissues of various organs. In most cases, these diseases have a bad prognosis without treatment. Therapeutic apheresis in combination with immunosuppressive therapies has led to a steady increase in survival rates over the last 35 years. Here we provide an overview of the most important pathogenic aspects indicating that therapeutic apheresis can be a supportive therapy in some systemic autoimmune diseases, such as systemic lupus erythematosus, antiphospholipid syndrome, rheumatoid arthritis, and inflammatory eye disease. With the introduction of novel and effective biologic agents, therapeutic apheresis is indicated only in severe cases, such as in rapid progression despite immunosuppressive therapy and/or biologic agents, and in patients with renal involvement, acute generalized vasculitis, thrombocytopenia, leucopenia, pulmonary, cardiac, or cerebral involvement. In mild forms of autoimmune disease, treatment with immunosuppressive therapies and/or biologic agents seems to be sufficient. The prognosis of autoimmune diseases with varying organ manifestations has improved considerably in recent years, due in part to very aggressive therapy schemes. [ABSTRACT FROM AUTHOR]

Objective: To investigate oral nonsteroidal anti-inflammatory drug (NSAID) therapy in the prevention of recurrences of uveitis in patients with recurrent nongranulomatous, idiopathic, or HLA-B27-associated acute anterior uveitis (AAU). Methods: Retrospective case series of 59 patients with recurrent AAU treated with celecoxib or diflunisal. Results: The average duration of NSAID therapy was 21.2 ± 5.7 months. The average number of relapses for all patients prior to systemic NSAID therapy was 2.84 per person-year follow-up. These relapses declined to 0.53 per person-year follow-up with NSAID therapy ( p < .001). The relapse rates prior to and after treatment in the HLA-B27-positive group ( n = 21) were compared with the relapse rates prior to and after treatment in the HLA-B27-negative group ( n = 38) and were also statistically significant ( p < .001). Conclusion: Morbid attacks and the cumulative exposure to corticosteroids can be prevented with systemic NSAID therapy in patients with recurrent AAU. [ABSTRACT FROM AUTHOR]

Heat shock protein (Hsp) 90 inhibitors, such as 17-allylamino-17-demethoxy-geldanamycin (17-AAG), constitute promising novel therapeutic agents. We investigated the anti-inflammatory activity of 17-AAG in endotoxin-induced uveitis (EIU) in rats. After the induction of EIU with a footpad injection of lipopolysaccharide (LPS), female Lewis rats received a single intraperitoneal. (i.p.) injection of 17-AAG or vehicle. Twenty-four hours later, the retinas were extracted and assayed for leukocyte adhesion; blood-retinal barrier breakdown; VEGF, TNF-α, IL-1β, and CD14 protein levels; NF-κB and HIF-1α activity; hsp90 and 70 levels and expression and phosphorylation of the tight junction proteins ZO-1 and occludin. 17-AAG treatment significantly suppressed the LPS-induced increase in retinal leukocyte adhesion; vascular leakage; NF-κB, HIF-1α, p38, and PI-3K activity; and VEGF, TNF-α, and IL-1β levels. 17-AAG also suppressed phosphorylation of ZO-1 and occludin by inhibiting their association with p38 and PI-3K Although 17-AAG treatment did not reduce the LPS-induced increase in total CD14 levels in leukocytes, it significantly decreased membrane CD14 levels. These data suggest that Hsp90 inhibition suppresses several cardinal manifestations of endotoxin-induced uveitis in the rat. 17-AAG has demonstrated a favorable safety profile in clinical trials in cancer patients and represents a promising therapeutic agent for the treatment of inflammatory eye disease. [ABSTRACT FROM AUTHOR]

Regulatory T cells (Tregs) are critical for the maintenance of immune tolerance and the suppression of excessive inflammation. Many inflammatory autoimmune disorders, including autoimmune uveitis, involve the loss of the suppressive capacities of Tregs. Over the past decade, Tregs' therapeutic potential in uveitis has garnered increasing attention. Specific subsets of Tregs, including TIGIT+ and PD-1+ Tregs, have emerged as potent immunosuppressors that may be particularly well-suited to cell-based therapeutics. Studies have elucidated the interaction between Treg development and the gut microbiome as well as various intracellular signaling pathways. Numerous cell-based therapies and therapeutic molecules have been proposed and investigated using the murine experimental autoimmune uveitis (EAU) model. However, certain challenges remain to be addressed. Studies involving the use of Tregs in human patients with uveitis are lacking, and there are concerns regarding Tregs' production and purification for practical use, their plasticity towards inflammatory phenotypes, immunogenicity, and tumorigenicity. Nevertheless, recent research has brought Tregs closer to yielding viable treatment options for uveitis., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Lee and Foulsham.)

Cytomegalovirus (CMV) is well known as pathogenic agent of intraocular inflammatory diseases. Light microscopy and immunohistochemical studies have limitations in the identification of this virus, but detection and typing viral DNA with Polymerase Chain Reaction (PCR) offers a rapid, highly specific, and easily interpretable means of identifying CMV in patients with ophthalmic lesions. Two patients (34-year-old male and 48-year-old male) who developed retinitis with unknown causative agent were studied for presence of CMV DNA. We used PCR kit for the qualitative detection of Cytomegalovirus (CMV 500/800 IC) provided for us by "Sacace Biotechnologies". The target of the PCR reaction was the "Major Immediate-Early" (MIE) gene. Positive and negative controls were used to avoid false results. Our PCR analysis showed the presence of CMV-DNA within the samples. [ABSTRACT FROM AUTHOR]

Objective: To characterize the clinical correlates and outcome of inflammatory ocular disease (IOD) among patients with ANCA-associated vasculitides (AAV)., Methods: Medical records of potential cases of AAV seen at Mayo Clinic from 2003 to 2013, inclusive, were reviewed to identify confirmed cases meeting the diagnosis of AAV using the Chapel Hill Consensus Conference 2012 descriptors. Records of confirmed cases of AAV were then further reviewed for IOD, and clinical characteristics, treatment and outcomes abstracted., Results: A total of 1171 confirmed cases of AAV were identified of which 183 patients (mean age 49.0 years; 51% female; 95% Caucasian) had IOD. The most common manifestation of IOD was injection of the eye (57%) followed by eye pain (46%) and visual acuity loss (18%). Scleritis was the most common type of IOD (22%) followed by episcleritis (21%), orbital inflammation (18%), lacrimal duct stenosis (10%) and uveitis (9%). Oral glucocorticoids were used to treat IOD in the majority of patients (96%). CYC and rituximab were the most frequently used immunosuppressive agents (54 and 36%, respectively). Of those with orbital inflammation, 52% underwent therapeutic surgical intervention. Clinical remission of IOD was achieved in 91% of patients but relapses were seen in 23%. Significant visual acuity loss was observed in only six patients., Conclusion: IOD is a common manifestation of AAV and seen in about 16% of patients with AAV. Scleritis, episcleritis and orbital inflammation are the most common subtypes. Most patients respond well to glucocorticoids and immunosuppression, but relapse of IOD is common., (© The Author 2017. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com)

Systemic autoimmune diseases based on an immune pathogenesis produce autoantibodies and circulating immune complexes, which cause inflammation in the tissues of various organs. In most cases, these diseases have a bad prognosis without treatment. Therapeutic apheresis in combination with immunosuppressive therapies has led to a steady increase in survival rates over the last 35 years. Here we provide an overview of the most important pathogenic aspects indicating that therapeutic apheresis can be a supportive therapy in some systemic autoimmune diseases, such as systemic lupus erythematosus, antiphospholipid syndrome, rheumatoid arthritis, and inflammatory eye disease. With the introduction of novel and effective biologic agents, therapeutic apheresis is indicated only in severe cases, such as in rapid progression despite immunosuppressive therapy and/or biologic agents, and in patients with renal involvement, acute generalized vasculitis, thrombocytopenia, leucopenia, pulmonary, cardiac, or cerebral involvement. In mild forms of autoimmune disease, treatment with immunosuppressive therapies and/or biologic agents seems to be sufficient. The prognosis of autoimmune diseases with varying organ manifestations has improved considerably in recent years, due in part to very aggressive therapy schemes., Competing Interests: The authors report no conflicts of interest in this work.