Granulomatosis with Polyangiitis Discovered Because of Repeated Upper Eyelid Swelling.

Background and objectives: The initial symptom that triggers granulomatosis with polyangiitis (GPA) diagnosis is rarely ocular. We describe a case with a single ocular lesion identified as probable GPA due to proteinase 3 (PR3)-antineutrophil cytoplasmic antibody (ANCA)-positivity according to the diagnostic criteria of the Ministry of Health in Japan; the lesion repeatedly worsened. Materials and methods: A 25-year-old female visited the Department of Ophthalmology, Asahi General Hospital, with upper eyelid swelling and conjunctival and episcleral hyperemia of the left eye. Both hordeolum and eyelid cellulitis were suspected, as the condition was resistant to treatment with antibiotic eye drops. Episcleritis was suspected due to localized hyperemia in the upper part of the eye. Upon treatment with antibacterial agents and steroid eye drops, the swelling and the hyperemia repeatedly worsened every week. Results: Blood samples were positive for PR3-ANCA, and GPA with an isolated ocular lesion was considered. After oral steroid treatment, the patient had no recurrence for 4 years. There was no systemic involvement in the upper respiratory tract, lungs, or kidneys. Conclusions: Diagnosing GPA with ocular symptoms as initial manifestations is challenging. GPA should be considered in treatment-resistant eyelid, orbital, and episcleral lesions, even at a young age.

The Prevalence and Characteristics of Inflammatory Bowel Disease-related Ocular Involvement in Children.

BACKGROUND: Ocular manifestations (OMs) in patients with inflammatory bowel disease (IBD) are uncommon, particularly in children. We aimed to explore the prevalence and characteristics of IBD-associated OM in a large cohort study. METHODS: A cross-sectional study was performed using the Maccabi Healthcare Services (MHS) database. The eligible population included all patients diagnosed with IBD as children (<18 years) between January 2005 and July 2023. RESULTS: Out of 2567 children with IBD (males 55%, Crohn's disease 64%), 78 (3%) were diagnosed with OM at any time during the disease course. In 54 patients (69%), the ocular disease occurred after IBD diagnosis with a median time of 2.6 (0.47-7) years between the 2 events, whereas in 24 patients (31%), ocular involvement preceded IBD diagnosis with a median time of 2.1 (0.6-5.7) years. The presence of ocular involvement was associated with increased usage of systemic corticosteroids (P < .001) and biologic agents (P = .04). There were 55 patients with ocular involvement during childhood who were ever diagnosed with IBD. In this population, ocular involvement was also associated with increased usage of systemic corticosteroids (P < .001). The prevalence of OM among patients with IBD did not change significantly over time (P = .75), with a prevalence of 2.3% at the end of the study period. CONCLUSIONS: The prevalence of ocular involvement in children with IBD is rare and steady; it is also associated with a greater usage of systemic corticosteroids and biologic agents, potentially representing a more severe disease course.

The prevalence of ocular manifestations in children with inflammatory bowel diseases is 2.3%, steady over the years. Ocular manifestations were associated with systemic steroids and biologic agents treatment, potentially representing severe inflammatory bowel disease.

Anterior Non-Necrotizing Scleritis with Active Uveitis in Cases of Ocular Syphilis.

PURPOSE: To study clinical characteristics and management outcomes of cases of ocular syphilis co-presenting with scleritis and active uveitis. METHODS: A retrospective analysis of cases diagnosed with ocular syphilis between January 2020 and December 2023 was conducted at a tertiary eye care centre. Clinical records, investigations, and outcomes were reviewed to identify cases with scleritis with active uveitis. Demographic data, clinical features, treatment modalities, and resolution patterns were analyzed. RESULTS: Among the 135 eyes of 95 cases of ocular syphilis studied, scleritis with uveitis was observed in 3.70% of eyes (five eyes). All cases with scleritis and uveitis were unilateral and male, with ages ranging from 32 to 61 years. Concurrent features included placoid chorioretinitis, retinal vasculitis, and anterior uveitis. Misdiagnosis with subsequent oral steroid therapy precipitated scleritis as an exacerbation in two cases. Three cases, which were previously undiagnosed, were found to be HIV-positive. Scleritis manifested as anterior, non-necrotizing inflammation, often accompanied by chemosis, and responded rapidly to antibiotic and non-steroidal anti-inflammatory therapy. Scleritis resolution preceded that of chorioretinitis and retinal vasculitis. CONCLUSIONS: Non-necrotizing anterior scleritis with chemosis can be a rare presentation of active syphilitic uveitis. Large placoid chorioretinitis lesions, preceding inadvertent oral steroid and/or undiagnosed HIV status were the possible risk factors for the development of concurrent scleritis.

Ocular lesions in patients with ulcerative colitis.

OBJECTIVE: Aim: To analyze the data and evaluate the prevalence of ocular lesions in patients with moderate ulcerative colitis. PATIENTS AND METHODS: Materials and Methods: We observed 112 patients aged 18-75 years old with clinically, endoscopically and histologically confirmed moderate ulcerative colitis which lasted at least 6 months. An ophthalmologic exam was performed to determine the presence of ocular symptoms. RESULTS: Results: Of the 112 patients with moderate ulcerative colitis, 21 (18,75%) had the following ocular lesions: episcleritis - 7 patients (6,25%), keratopathy - 5 patients (4,46%), uveitis - 5 patients (4,46%), cataract - 2 (1,78%) and scleritis - 2 (1.78%). CONCLUSION: Conclusions: Because ocular symptoms in patients with UC are often nonspecific, it may be beneficial to perform ophthalmologic examinations as a routine follow-up component of in such patients.

Syphilitic scleritis and episcleritis: A review.

Scleritis and episcleritis are rare, but potentially sight-threatening forms of syphilis. To provide a full description of this neglected subset of ocular syphilis, we evaluated the English literature for reports of syphilitic scleritis and episcleritis, recording the demographics, clinical characteristics, serological data, management practices, treatment responses, and visual outcomes. Previously published descriptions of 44 patients with syphilitic scleritis (50 eyes) and 9 patients with syphilitic episcleritis (14 eyes) were identified. The predominant type of scleritis was anterior scleritis, accounting for 92.9% of cases, with nodular anterior scleritis being the most frequent subtype at 58.1%. Almost one-quarter of patients were co-infected with human immunodeficiency virus (HIV). Initial misdiagnosis was common and led to delays in initiating treatment with appropriate antibiotics. Visual outcomes were often good in both scleritis and episcleritis, irrespective of HIV infection status, although complications including scleral thinning, keratitis, and uveitis, along with permanent visual loss and an association with neurosyphilis, were reported. Response to antibiotic treatment was typically rapid, often within 1 week. With the rising global incidence of syphilis, testing patients with scleritis or episcleritis for this infectious disease is important to ensure prompt diagnosis and treatment for best ocular and systemic outcomes.

Case report: Medical and surgical management of presumed canine nodular granulomatous episcleritis with progression to posterior nodular scleritis, choroiditis, and optic neuritis.

Nodular granulomatous episcleritis (NGE) typically presents as an elevated mass or elevated masses at the limbus and often infiltrates the cornea (episclerokeratitis). In the current report, a granulomatous lesion was observed subretinally in the right eye (OD) of a 5-year-old male castrated American Staffordshire Terrier dog. There was concurrent retinal hemorrhage and detachment OD; the right eye was not visual. Due to poor prognosis for vision and potential for a neoplastic etiology of the mass, staging with higher imaging was recommended but declined by the owner. Therefore, an enucleation was performed. Histopathology of the globe identified a subretinal mass, marked histiocytic and lesser lymphoplasmacytic choroiditis, posterior episcleritis, and optic neuritis with retinal detachment. The subretinal mass was composed of densely packed, large, spindle histiocytes mixed with occasional lymphocytes, plasma cells, and only rare neutrophils. Regions of the mass showed lymphocytes aggregate to form nodules. This histological presentation was a type of proliferative histiocytic disease with similarities to nodular granulomatous episcleritis or granulomatous/necrotizing scleritis. This is a novel presentation of NGE-like progression to subretinal scleral, choroidal, and retinal involvement and provides a new differential possibility for posterior segment masses observed on fundic examination.

Multiple ocular manifestations in a patient diagnosed with herpes zoster ophthalmicus: case report.

Objective: Our purpose was to present a case of a patient diagnosed with herpes zoster ophthalmicus with multiple ocular manifestations. Case presentation: A 70-year-old Caucasian male presented to the hospital for headache and skin hyperesthesia on the scalp and forehead on the left side. The diagnoses of herpes zoster ophthalmicus and acute conjunctivitis were made for the left eye. The patient was followed up for 6 months and during that period the following diagnoses were made for the same eye: peripheral sterile corneal infiltrates, episcleritis, and hypertensive anterior uveitis. Discussions: Herpes zoster ophthalmicus occurs when the reactivation of the dormant virus involves the ophthalmic division of the trigeminal nerve. The most frequent ocular presentations are conjunctivitis, keratitis, uveitis, episcleritis, and scleritis. The standard therapy consists of antivirals, such as acyclovir, valacyclovir, and famciclovir to limit the replication of the virus. The patient's risk factors, the course of treatment, and the severity of the disease, all affect the prognosis, which is highly variable. Prevention of the disease consists of vaccination with one of the following two vaccines, Zostavax and Shingrix. Conclusions: Final visual acuity for the left eye remained 1 despite numerous manifestations of the disease. Abbreviations: VZV = Varicella-zoster virus, BCVA = best-corrected visual acuity, OU = both eyes, OD = right eye, OS = left eye, IOP = intraocular pressure, NCT = non-contact tonometer, ZVX = Zostavax vaccine.

COVID-19 and Its Ophthalmic Manifestations: A Literature Review.

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is a novel coronavirus discovered in late 2019 in Wuhan, Hubei Province, China. The virus has now developed into a full-scale global pandemic affecting hundreds of millions of people to date. A majority of cases present with nonspecific acute upper respiratory symptoms. A wide range of systemic symptoms has been reported, with some patients presenting with nonspecific extrapulmonary symptoms. Recently, there has been an increased association of COVID-19-positive patients presenting with ocular symptoms. As an increasing number of patients present with ophthalmic manifestations, recognizing these visual symptoms is of utmost importance. Some patients may present with ocular symptoms as the first indication of COVID-19 infection; quickly isolating and starting treatment can aid in stopping the spread of this novel coronavirus. This review will describe the current epidemiology and pathophysiology of SARS-CoV-2, emphasizing the ophthalmic manifestations and their clinical course progression. Further, we will be reporting on the growing number of rare ocular manifestations that have occurred in some COVID-19-positive patients, along with the route of transmission, specific manifestations, and the treatment methods for both these pulmonary and extrapulmonary symptoms, specifically the ocular manifestations.

Mimickers of anterior uveitis, scleritis and misdiagnoses- tips and tricks for the cornea specialist.

BACKGROUND: Anterior uveitis, inflammation of the anterior chamber and related structures, is a cohort of diseases that can present to almost any general or sub-specialty Ophthalmology practice. Its features classically involve anterior chamber cell and flare. Below the surface of these two signs exist a panoply of diagnoses. BODY: The purpose of this review is to provide a general framework for diagnoses of anterior uveitis that are often missed as well as non-uveitic pathologies that often mimic anterior uveitis. Diagnostic deviation in either direction can have vision-threatening and rarely life-threatening consequences for patients. Using a comprehensive literature review we have collected a broad spectrum of etiologies of anterior uveitis that are easily missed and non-uveitic pathologies that can masquerade as anterior uveitis. CONCLUSIONS: We present a focused review on specific misdiagnosed anterior uveitis pathologies and some of the conditions that can masquerade as anterior uveitis and scleritis.

Ocular manifestations of granulomatosis with polyangiitis: report from a tertiary eye care center.

PURPOSE: To report demographic characteristics and ophthalmological manifestations of patients with granulomatosis with polyangiitis (GPA) in a tertiary eye care center in Turkey. METHODS: Medical records of patients with GPA-related ocular manifestations evaluated between 2013 and 2023 were included. Demographic and clinical characteristics of the patients including nature of systemic involvement, ophthalmologic symptoms and signs, laboratory investigations and treatment modality were reviewed. RESULTS: Twelve eyes of 10 patients (5 female/5 male) were included. The mean age was 57.2 ± 12.2 (35-71) years. Five (50%) patients were already diagnosed with GPA. Ocular involvement was the first manifestation of GPA in 3 patients. The remaining 2 patients had simultaneous systemic and ocular symptoms at presentation. Conjunctival hyperemia (9 eyes) and pain (7 eyes) were the most frequent presenting symptoms followed by blurred vision (3 eyes). The frequencies of ocular manifestations were as follows:episcleritis (3 eyes), isolated peripheral ulcerative keratitis (PUK) (3 eyes), scleritis (3 eyes), simultaneous PUK and scleritis (2 eyes) and periorbital mass (1 eye). CONCLUSION: Ophthalmological manifestations can be the initial findings in GPA. Since GPA can affect different structures of the eye, it sometimes might be challenging for ophthalmologists. Therefore, it is crucial for ophthalmologists to be well-informed about GPA-related ocular findings and to have a high index of suspicion for GPA. Although PUK associated with scleritis is highly suggestive for GPA, isolated cases of PUK or scleritis can be seen in GPA. Therefore, it is important to adopt a multidisciplinary approach, consider GPA in differential diagnosis, and benefit from accurate diagnostic tests.

Ophthalmological Manifestations in Inflammatory Bowel Diseases: Keep an Eye on It.

BACKGROUND AND AIMS: Inflammatory bowel diseases (IBD) are multifactorial chronic inflammatory disorders affecting the gastrointestinal tract. However, a broad spectrum of extraintestinal manifestations (EIMs) is associated with IBD, affecting several organs and systems, such as the skin, musculoskeletal and hepatobiliary systems, and, not least, the eye. Approximately 10% of IBD patients can develop ocular EIMs (O-EIMs) with a higher prevalence in Crohn's disease (CD). Eye-redness, photophobia, pain, and blurred vision are the common symptoms, with a wide rate of severity and clinical impact on the quality of life. This narrative review aims to summarize the prevalence, pathogenesis, and current evidence-based management of O-EIMs, underlying the importance of a holistic approach and specialties collaboration for a prompt diagnosis and treatment. METHODS: PubMed was searched up to December 2023 to identify relevant studies investigating the pathogenesis, epidemiology, and treatment of O-EIMs in IBD patients. RESULTS: The mechanisms underlying O-EIMs are partially unknown, encompassing immune dysregulation, shared antigens between the eye and the gut, genetic predisposition, and systemic inflammation driven by high levels of interleukins and cytokines in IBD patients. The complexity of O-EIMs' pathogenesis reflects in the management of these conditions, varying from topical and systemic steroids to immunomodulatory molecules and biologic therapy, such as anti-tumor necrosis factor (TNF)-alpha. A multidisciplinary approach is the backbone of the management of O-EIMs.

Etanercept-associated episcleritis: a pediatric case report of a paradoxical adverse reaction and review of the literature.

Scleritis is an inflammation of the episcleral and scleral tissues, characterized by injection in both superficial and deep episcleral vessels. When only episcleral tissue is involved, it is referred to as episcleritis. Episcleritis is mainly idiopathic but may be secondary to an underlying rheumatologic disease. Despite being rare, drug-associated episcleritis and scleritis should also be included in the differential diagnosis. Tumor necrosis factor-alpha (TNF-α) inhibitors are generally well-tolerated, but etanercept, in particular, has the potential to cause paradoxical adverse reactions including ocular inflammations, such as uveitis, scleritis, and ocular myositis. Etanercept differs in its mechanism of action from other TNF-α inhibitors as it acts as a decoy receptor, and this may partly explain the more frequently reported etanercept-associated ocular inflammation. Etanercept may also be ineffective in preventing ocular inflammation. However, the dechallenge and rechallenge phenomena have proven there is a causative link between etanercept and new-onset ocular inflammation. We report a case of a 15-year-old boy with enthesitis-related arthritis and familial Mediterranean fever who presented with episcleritis and blepharitis while receiving etanercept treatment and subsequently showed dechallenge and rechallenge reactions. Therefore, physicians should also be aware that episcleritis should be considered a paradoxical adverse reaction to etanercept and can occur in pediatric patients. We also reviewed the English literature to provide an overview and evaluate intervention options.

Clinical and pathological findings in five dogs with nodular histiocytic iritis in the United Kingdom: A case series.

PURPOSE: The aim of this study was to describe the clinical presentation, histopathology, management, and outcome of nodular histiocytic iritis, an intraocular variant of nodular granulomatous episcleritis (NGE). METHODS: A retrospective review of the medical records of five dogs with intraocular NGE-type inflammation as diagnosed by histopathology. RESULTS: Four Border Collies and one crossbreed dog, aged 1.5-3.4 years (mean age 2.38 years). The clinical presentation was an extensive, raised, pale iris lesion of variable location. All cases were unilateral. The physical examination was normal. Complete blood count/serum biochemistry (n = 1) and thoracic radiography (n = 1) were normal. Ocular ultrasound (n = 2) was normal apart from increased iris thickness. Enucleation (n = 4) or excisional biopsy (iridectomy, n = 1) was performed because of suspected neoplasia. Following enucleation, the remaining, contralateral eye did not develop additional lesions (9 days-3.7 years follow-up). There was no recurrence following sector iridectomy with 5 months topical 1% prednisolone acetate (3.9 years follow-up). The histopathologic findings in all five cases indicated a focal histiocytic and lymphoplasmacytic anterior uveitis (iritis), similar to that seen in cases of NGE. CONCLUSION: Nodular histiocytic iritis presents as unilateral iris thickening in isolation and young Collies appear to be predisposed. The histopathological findings are similar to NGE. Although the clinical presentation resembles intraocular neoplasia, an inflammatory process should be considered, which may be amenable to medical management. Definitive diagnosis may be obtained by iris sampling.

Idiopathic granulomatous mastitis with extramammary manifestations: a case report.

Introduction and Importance: Granulomatous mastitis is a rare inflammatory disorder of the breast, which can be either idiopathic or due to secondary etiology. This disease affects women of reproductive age. The exact pathophysiology underlying idiopathic granulomatous mastitis (IGM) remains unclear, but it is believed to be mediated by immunological processes. Establishing a diagnosis of this condition could be challenging due to the long list of differential diagnoses that it creates. Case Presentation: We report a 24-year-old Syrian female presented to the clinic complaining of a 2-week history of fatigue, fever and chills, swelling, and localized pain in her left breast. Physical examination revealed erythema nodosum, episcleritis, and arthralgia in the wrists, ankles, and elbows. An excisional biopsy was done and a microscopic examination of the lesion confirmed granulomatous perilobular mastitis. Symptoms had resolved after the surgical excision and follow-up evaluation showed no signs of recurrence. Clinical Discussion: IGM typically presents as an enlarging breast mass that can be mistaken for breast cancer or an abscess. The diagnostic approach should consider the presence of extramammary symptoms such as fever, arthralgia, and fatigue. Treatment options include corticosteroids, surgical excision, or steroid-sparing agents, but relapse rates vary. Conclusions: Episcleritis should be considered as a potential extramammary manifestation in cases of IGM. We highlight the importance of recognizing and investigating the potential systemic involvement in patients with IGM. Accurate interpretation of pathological and radiological findings by a multidisciplinary breast team can facilitate the diagnosis and reduce unnecessary interventions.

[Ophthalmic manifestations of COVID-19]. / Oftal'mologicheskie proyavleniya COVID-19.

In March 2020 the World Health Organization (WHO) declared a pandemic of the novel coronavirus infection (COVID-19) caused by the SARS-CoV-2 respiratory virus. The high spread rate of the virus and the severity of the course of the disease are of great clinical and epidemiological significance, making it relevant for ophthalmologists to study the mechanisms of how SARS-CoV-2 infects ocular structures, as well as possible clinical manifestations of the infection in the organ of vision. This review contains analysis, systematization, and generalization of epidemiological and clinical data on SARS-CoV-2 ocular lesions and was carried out with the data found in scientific abstract databases. The article presents main clinical ophthalmic manifestations of COVID-19, lists the utilized schemes of etiotropic and symptomatic therapy, recommended preventive measures, and considers the possible ophthalmic complications after vaccination against COVID-19.

Treatment of Steroid-Resistant Nodular Episcleritis With Tacrolimus: A Case Report.

A 46-year-old male, with no chronic medical illness, complained of pain, tearing, and redness for one-month duration, with no photophobia, discharge, or decrease in visual acuity. Examination revealed a small, painful, red swelling in the left sclera. Slit-lamp examination using a narrow bright slit beam revealed edema of the episcleral layer and injection of the superficial episcleral blood vessels. The rest of the anterior segment exam and fundoscopy were normal. The laboratory investigations and systemic workup were normal. The patient was initially treated with prednisolone acetate (Pred Forte) 1% every three hours per day for one week, and then four times per day for another week, and tapered gradually over eight weeks with systemic nonsteroidal anti-inflammatory drugs (NSAIDs) as diclofenac sodium for eight weeks with mild improvement of clinical symptoms, but the size of the lesion remained without any change and the patient started to have a relapse of symptoms at the end of the course. Topical tacrolimus drops of 0.1% concentration were prepared in the pharmacy under complete sterile precautions and were used four times per day for the following six weeks duration instead of the initial therapy (steroids and NSAIDs). Tacrolimus drops were then tapered gradually over another six weeks duration. The patient showed dramatic suppression of inflammation and exceptional remission of symptoms with complete resolution of the episcleritis. Topical tacrolimus is very effective in the treatment of nodular episcleritis, which is resistant to steroid therapy. Patients with nodular episcleritis suffer from prolonged bouts of inflammation that are characteristically more painful than the diffuse type and may be associated with other systemic diseases. The case is steroid-resistant nodular episcleritis, which did not respond to the usual treatment and showed a good response to treatment with tacrolimus, which was first introduced in episcleritis. Tacrolimus is being used in other ocular diseases, but its use in episcleritis is unique.

Epiescleritis y edema periorbitario secundario a síndrome VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) / Episcleritis and periorbital edema secondary to VEXAS syndrome

El síndrome vacuolas, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) es una nueva entidad autoinflamatoria descrita recientemente, producida por una mutación del gen UBA-1. Entre los síntomas más frecuentes están la fiebre, las citopenias, la policondritis, los infiltrados pulmonares y hasta en un 40% afectación ocular en forma de edema periorbitario, uveítis, epiescleritis, escleritis y vasculitis retiniana. Los pacientes responden a altas dosis de corticoterapia, sin embargo muchos terminan siendo refractarios a las mismas y a los inmunosupresores clásicos. Se describe el caso de un paciente varón de 77 años con afectación ocular en forma de epiescleritis y edema periorbitario que posteriormente fue diagnosticado de síndrome VEXAS. El paciente, tras fracasar al tratamiento con inmunosupresores, en la actualidad está en tratamiento con esteroides orales y tocilizumab. Los especialistas en oftalmología deben estar al corriente de la afectación oftalmológica de las enfermedades autoinflamatorias, y en especial de esta nueva entidad descrita, como es el síndrome VEXAS (AU)

VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a newly described autoinflammatory entity caused by a UBA-1 gene mutation. Among the most frequent symptoms it produces fever, cytopenias, polychondritis, pulmonary infiltrates and up to 40% ocular involvement such as periorbital edema, uveitis, episcleritis, scleritis and retinal vasculitis. Patients respond to high doses of corticosteroids, however, many end up being refractory to them and to the classic immunosuppressants. We described the case of a 77-year-old male patient with ocular involvement in the form of episcleritis and periorbital edema who was later diagnosed with VEXAS syndrome. The patient, after failing treatment with immunosuppressants, is currently receiving treatment with oral steroids and tocilizumab. Ophthalmologist must be aware of the ophthalmological affectation of autoinflammatory diseases and especially of this new entity described as the VEXAS syndrome (AU)

An Atypical Presentation of Relapsing Polychondritis Manifesting As Acute Angle Closure.

Relapsing polychondritis (RPC) is a rare autoimmune disease characterized by recurrent inflammation of cartilaginous and proteoglycan-rich tissues throughout the body. The most commonly affected sites are the auricular pinna, nose, and joints with ocular tissue involvement occurring in up to 50% of patients. The most common ocular manifestations are scleritis, episcleritis, uveitis, and conjunctivitis. Less common ocular issues include keratitis, retinopathy, optic neuropathy, extraocular muscle palsy, and orbital inflammation. Due to the variable clinical presentation and rarity of the disease, the diagnosis of RPC is often delayed or it is misdiagnosed. It is important that ophthalmologists be aware of relapsing polychondritis because ocular symptoms may present as the initial manifestation of the disease.

Uveítis e inflamaciones oculares asociadas a COVID-19 / Uveitis and Ocular Inflammations Associated with COVID-19

La enfermedad por el coronavirus 2019 (COVID-19) es causada por el coronavirus 2 del síndrome de distrés respiratorio del adulto (SARS-CoV-2). Esta se declaró enfermedad pandémica por la Organización Mundial de la Salud el 11 de enero de 2020. Dentro de sus manifestaciones clínicas oftalmológicas destacan las afecciones en la superficie ocular, de ellas la conjuntivitis folicular, como la de mayor incidencia encontradas en los pacientes infectados. De igual manera, las uveítis, escleritis y epiescleritis han sido reportadas en sus diversas formas de presentación precediendo o acompañando las manifestaciones clínicas generales de la enfermedad, así como en el período pos-COVID-19. El objetivo de esta revisión fue realizar una búsqueda de información para establecer la relación entre uveítis y COVID-19, a pesar de no haber sido reconocida como una de las manifestaciones oculares reportadas con más frecuencia(AU)

Coronavirus 2019 (COVID-19) disease is caused by adult respiratory distress syndrome coronavirus 2 (SARS-CoV-2). It was declared a pandemic disease by the World Health Organization on January 11, 2020. Among its ophthalmologic clinical manifestations, ocular surface disorders stand out, of which follicular conjunctivitis is the most frequent found in infected patients. Similarly, uveitis, scleritis and episcleritis have been reported in their various forms of presentation preceding or accompanying the general clinical manifestations of the disease, as well as in the post-COVID-19 period. The aim of this review was to search for information to establish the relationship between uveitis and COVID-19, although it has not been recognized as one of the most frequently reported ocular manifestations(AU)

Episcleritis and periorbital edema secondary to VEXAS syndrome.

VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a newly described autoinflammatory entity caused by a UBA-1 gene mutation. Among the most frequent symptoms it produces fever, cytopenias, polychondritis, pulmonary infiltrates and up to 40% ocular involvement such as periorbital edema, uveitis, episcleritis, scleritis and retinal vasculitis. Patients respond to high doses of corticosteroids, however, many end up being refractory to them and to the classic immunosuppressants. We described the case of a 77-year-old male patient with ocular involvement in the form of episcleritis and periorbital edema who was later diagnosed with VEXAS Syndrome. The patient, after failing treatment with immunosuppressants, is currently receiving treatment with oral steroids and tocilizumab. Ophthalmologist must be aware of the ophthalmological affectation of autoinflammatory diseases and especially of this new entity described as the VEXAS Syndrome.