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The problem of treating purulent scleral infections, rare but extremely severe complication of ophthalmic surgeries, remains unresolved. This article presents a case of successful surgical treatment of purulent scleritis - interlamellar scleral abscess - that developed in a patient after repeat penetrating keratoplasty performed due to infectious lysis of the transplant. Although the first keratoplasty was performed for acanthamoeba keratitis, there were no signs of acanthamoeba invasion in the transplant at the time of the second surgery. Scleritis manifested as an infiltrate with pus penetrating the anterior chamber and development of keratoiridocyclitis. During surgery, the abscess cavity was opened, irrigated with an antiseptic solution, and drained into the subconjunctival space; the anterior chamber was irrigated with balanced salt solution through a separate paracentesis. No infection recurrences were noted in the postoperative period and the corneal transplant remained clear.
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Queratitis por Acanthamoeba , Queratoplastia Penetrante , Escleritis , Humanos , Queratoplastia Penetrante/métodos , Queratoplastia Penetrante/efectos adversos , Queratitis por Acanthamoeba/etiología , Queratitis por Acanthamoeba/diagnóstico , Queratitis por Acanthamoeba/cirugía , Escleritis/etiología , Escleritis/diagnóstico , Escleritis/cirugía , Resultado del Tratamiento , Complicaciones Posoperatorias/cirugía , Complicaciones Posoperatorias/diagnóstico , Complicaciones Posoperatorias/etiología , Masculino , Reoperación/métodos , Esclerótica/cirugía , Adulto , FemeninoRESUMEN
INTRODUCTION: This study aimed to explore ocular manifestations in ANCA-associated vasculitis (AAV), focusing on granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), and microscopic polyangiitis (MPA) and to examine the associations with laboratory parameters and other systemic manifestations. METHODS: This retrospective study reviewed data from 533 AAV patients across two major Chinese medical centers from January 2016 to November 2023. Data including diagnosis, cranial manifestations of disease, ocular complications, and laboratory parameters were analyzed. Univariate and multivariable logistic regression analyses assessed associations across disease manifestations. Machine learning models were also utilized to predict the risk of retinal/eye involvement in AAV patients. RESULTS: Among 533 patients (210 GPA, 217 MPA, 99 EGPA, and 7 unclassified AAV), ocular complications were observed in 20.64% of them, with a distribution of 36.67% in GPA, 7.37% in MPA, and 18.18% in EGPA. The most common ocular manifestations included scleritis and retro-orbital mass/dacryocystitis, which were notably prevalent in GPA patients. Retinal involvement was observed in 9.09% of EGPA cases. The machine learning models yielded that eosinophil percentage (EOS%), high-sensitivity C-reactive protein (hsCRP), and CD4 + T cell/CD8 + T cell ratio (T4/T8) can predict retinal involvement. Furthermore, the white blood cell, EOS%, APTT, IgA, hsCRP, PR3-ANCA, and T4/T8 can predict eye involvement. CONCLUSION: Ocular manifestations are a prevalent complication across all forms of AAV. Predictive models developed through machine learning offer promising tools for early intervention and tailored patient care. This necessitates a multidisciplinary approach, integrating rheumatology and ophthalmology expertise for optimal patient outcomes.
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Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos , Humanos , Masculino , Estudios Retrospectivos , Femenino , Persona de Mediana Edad , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/complicaciones , China/epidemiología , Anciano , Adulto , Oftalmopatías/etiología , Poliangitis Microscópica/complicaciones , Granulomatosis con Poliangitis/complicaciones , Aprendizaje Automático , Proteína C-Reactiva/análisis , Escleritis/etiología , Escleritis/epidemiología , Pueblos del Este de AsiaRESUMEN
BACKGROUND: Hodgkin's lymphoma (HL) is an extremely rare cause of ocular inflammation that is usually not considered in the typical workup of uveitis and other eye diseases. A few cases of ocular inflammation were reported previously showcasing HL with absence of typical symptoms of HL at presentation. Acknowledging the potential ocular inflammation associated with HL can prompt ophthalmologists to broaden their diagnostic approach and collaborate with internal medicine departments to investigate this rare yet significant etiology. CASE PRESENTATION: A 17-year-old Caucasian woman presenting unilateral panuveitis was later diagnosed with HL. The ocular findings were non-necrotizing scleritis, anterior uveitis, vitritis, white/yellowish chorioretinal lesions, papillitis and vasculitis. A left supra-clavicular lymph node biopsy confirmed the diagnosis of nodular sclerosing Hodgkin's lymphoma stage IIB. Other causes of uveitis were excluded. Chemotherapy led to remission of the disease and the ocular lesions became quiescent with persistent pigmented chorioretinal scars. CONCLUSIONS: Hodgkin's lymphoma should be considered in the differential diagnosis of diseases that can occasionally be revealed by unilateral ocular inflammation. A comprehensive, multidisciplinary approach is key to properly assessing such cases.
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Enfermedad de Hodgkin , Humanos , Enfermedad de Hodgkin/diagnóstico , Femenino , Adolescente , Diagnóstico Diferencial , Escleritis/diagnóstico , Escleritis/etiología , Escleritis/tratamiento farmacológico , Uveítis/diagnóstico , Uveítis/tratamiento farmacológico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Panuveítis/diagnóstico , Panuveítis/tratamiento farmacológico , Panuveítis/etiologíaRESUMEN
Scleromalacia perforans, or necrotising anterior scleritis, is a rare and severe form of eye disease that usually occurs in patients suffering from long-standing systemic inflammatory diseases, with rheumatoid arthritis (RA) being the most common. Here, we report the case of a patient who presented with redness of the eye and discolouration of the sclera and was diagnosed with scleromalacia perforans without any further extraophthalmic systemic involvement. Serological workup revealed highly positive cyclic citrullinated peptide (CCP) antibody (CCP-IgG/anticitrullinated protein antibodies) and positive rheumatoid factor, serologies commonly associated with RA. The patient's symptoms responded very well to rituximab therapy.
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Artritis Reumatoide , Rituximab , Escleritis , Humanos , Artritis Reumatoide/complicaciones , Artritis Reumatoide/tratamiento farmacológico , Escleritis/tratamiento farmacológico , Escleritis/etiología , Escleritis/diagnóstico , Rituximab/uso terapéutico , Femenino , Factor Reumatoide/sangre , Antirreumáticos/uso terapéutico , Persona de Mediana Edad , Péptidos Cíclicos/inmunología , MasculinoRESUMEN
PURPOSE OF REVIEW: To highlight the clinical features of mpox with an emphasis on ocular manifestations and to review treatment options for this re-emerging infectious disease. RECENT FINDINGS: Ocular involvement of mpox varies by clade. The most recent 2022 outbreak appears to be associated with fewer conjunctivitis cases compared to previous outbreaks. However, the ocular findings occurring during this newly emerging clade can be visually threatening and include cases of keratitis, rapidly progressing scleritis, and necrotizing periorbital rashes. SUMMARY: Ocular mpox is an important clinical feature of systemic mpox virus (MPXV) infection. Heightened clinical suspicion allows for a timely diagnosis and the initiation of antiviral treatment, when appropriate. Randomized clinical trials for mpox systemic and ocular treatment efficacy are lacking. Prior clinical experience with smallpox and in-vitro mpox data support the use of systemic antivirals such as tecovirimat, cidofovir, brincidofovir and topical use of trifluridine in ocular mpox management, though treatment-resistant infection can occur and portend a poor prognosis.
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Antivirales , Infecciones Virales del Ojo , Humanos , Antivirales/uso terapéutico , Infecciones Virales del Ojo/diagnóstico , Infecciones Virales del Ojo/virología , Infecciones Virales del Ojo/tratamiento farmacológico , Orthopoxvirus , Escleritis/diagnóstico , Escleritis/tratamiento farmacológico , Escleritis/etiologíaRESUMEN
An 84-year-old man presented with decreased right-eye visual acuity. Upon initial examination, the rightand left-eye visual acuities were 0.03 and 1.2, respectively; moreover, the right- and left-eye intraocular pressure was 12 mmHg and 13 mmHg, respectively. Examination revealed a shallow anterior chamber of the right eye, anterior chamber inflammation, vitreous opacity, and marked retinochoroidal detachment. Optical coherence tomography (OCT) revealed retinal detachment (RD) and choroidal folds; moreover, B-scan ultrasonography (B-scan) showed RD as well as thickened sclera with fluid in Tenon's space. Fluorescent fundus angiography revealed hyperfluorescence in the optic disc and vascular hyperpermeability in the right eye. The left eye lacked extra-ocular symptoms or abnormalities. The right ocular axis measured 23.4 mm with no apparent subretinal fluid migration due to positional changes. Accordingly, the patient was diagnosed with panuveitis associated with posterior scleritis and immediately started on 40 mg prednisolone, which improved his symptoms. However, at 3 post-treatment months, choroidal folds were observed and was restarted on 20 mg prednisolone. The choroidal folds subsequently disappeared, with a current visual acuity of 0.3 in the right eye and no recurrence. Our findings indicated the utility of accurate diagnosis of posterior scleritis by B-scan and prompt systemic steroid administration.
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Panuveítis , Prednisolona , Desprendimiento de Retina , Escleritis , Tomografía de Coherencia Óptica , Agudeza Visual , Humanos , Masculino , Escleritis/etiología , Escleritis/diagnóstico , Escleritis/diagnóstico por imagen , Escleritis/complicaciones , Desprendimiento de Retina/etiología , Desprendimiento de Retina/diagnóstico por imagen , Desprendimiento de Retina/diagnóstico , Anciano de 80 o más Años , Panuveítis/diagnóstico , Panuveítis/etiología , Panuveítis/complicaciones , Prednisolona/administración & dosificación , Resultado del Tratamiento , Angiografía con Fluoresceína/métodos , Coroides/diagnóstico por imagen , Coroides/patología , Enfermedades de la Coroides/etiología , Enfermedades de la Coroides/diagnóstico por imagen , Enfermedades de la Coroides/diagnóstico , Enfermedades de la Coroides/complicacionesRESUMEN
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.
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Colitis Ulcerosa , Humanos , Colitis Ulcerosa/complicaciones , Colitis Ulcerosa/epidemiología , Adulto , Persona de Mediana Edad , Masculino , Femenino , Anciano , Adulto Joven , Adolescente , Prevalencia , Escleritis/etiología , Escleritis/epidemiología , Uveítis/etiología , Uveítis/epidemiología , Oftalmopatías/etiología , Oftalmopatías/epidemiologíaAsunto(s)
Escleritis , Humanos , Escleritis/diagnóstico , Escleritis/etiología , Masculino , Enfermedades Orbitales/diagnóstico , Enfermedades Orbitales/complicaciones , Enfermedades Orbitales/etiología , Síndrome , Enzimas Activadoras de Ubiquitina/genética , Vacuolas/patología , Enfermedades Genéticas Ligadas al Cromosoma X/diagnóstico , Enfermedades Genéticas Ligadas al Cromosoma X/complicaciones , Enfermedades Genéticas Ligadas al Cromosoma X/genética , Enfermedades Autoinflamatorias Hereditarias/diagnóstico , Enfermedades Autoinflamatorias Hereditarias/complicaciones , Enfermedades Autoinflamatorias Hereditarias/genéticaRESUMEN
BACKGROUND: VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) is a hematoinflammatory disease that typically affects adults. It results from a somatic mutation of the E1 ubiquitin conjugating enzyme encoded by the UBA1 gene. VEXAS is frequently accompanied by myelodysplastic syndrome (MDS). The purpose of this study is to describe the ocular and orbital manifestations of VEXAS patients in a case series in our medical centre. METHODS: A retrospective chart review was performed for all patients who were diagnosed with VEXAS syndrome in a tertiary medical centre over two years. RESULTS: Eight patients were identified with VEXAS. In six patients, the diagnosis was confirmed by genomic sequencing. Two patients were identified based on their phenotype. All patients were males. The mean age at diagnosis was 78.7 years. In two patients, the ocular manifestation was the presenting symptom for VEXAS. Seven patients (87.5%) had history of MDS. Systemic inflammation manifestations include: skin rash (n = 5), recurrent fevers (n = 2), relapsing polychondritis (n = 2), pleuritis and pleural effusion (n = 2), poly arteritis nodosa- PAN (n = 1) and thrombophlebitis (n = 1). Seven (87%) patients were presented with periorbital oedema. Three patients showed orbital inflammation. Dacryoadenitis was observed in two patients, and extraocular muscle (EOM) myositis was detected in two patients. Four patients demonstrated ocular inflammation such as: episcleritis, scleritis and anterior uveitis. CONCLUSION: ocular manifestations in VEXAS include orbital inflammation, dacryoadenitis, myositis, uveitis, scleritis, episcleritis and periorbital oedema. We recommend that in old male patients, with history of haematological disorder, presenting with ocular symptom, VEXAS investigation should be taken into consideration.
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Enfermedades Orbitales , Humanos , Masculino , Estudios Retrospectivos , Anciano , Anciano de 80 o más Años , Enfermedades Orbitales/etiología , Enfermedades Orbitales/diagnóstico , Persona de Mediana Edad , Enzimas Activadoras de Ubiquitina/genética , Síndromes Mielodisplásicos/genética , Síndromes Mielodisplásicos/complicaciones , Síndromes Mielodisplásicos/diagnóstico , Escleritis/diagnóstico , Escleritis/etiología , Oftalmopatías/etiología , Oftalmopatías/genética , Oftalmopatías/diagnóstico , Mutación , Enfermedades Autoinflamatorias Hereditarias/genética , Enfermedades Autoinflamatorias Hereditarias/diagnóstico , Enfermedades Autoinflamatorias Hereditarias/complicacionesRESUMEN
Scleritis, an inflammatory disease of the eye affecting scleral tissue, presents unique challenges in the older adult population. Unlike their younger counterparts, older individuals manifest a distinct spectrum of the disease with different underlying etiologies, co-morbidities, altered immune function, and an increased risk of systemic side effects from medication choices. Addressing these complexities necessitates a comprehensive and multidisciplinary approach. Treatment of choice will depend on any underlying cause but generally involves non-steroidal anti-inflammatory drugs, systemic or local corticosteroids, and potentially disease-modifying anti-rheumatic drugs. Utilization of these therapeutic agents in older adults warrants careful consideration because of their potential side-effect profiles. This article critically examines the specific concerns for the use of these drugs in older patients and reviews the existing literature on their use in this specific cohort.
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Escleritis , Humanos , Anciano , Escleritis/tratamiento farmacológico , Escleritis/etiología , Inmunosupresores/uso terapéutico , Resultado del Tratamiento , Antiinflamatorios no Esteroideos/efectos adversos , Corticoesteroides/efectos adversosRESUMEN
A 67-year-old woman with history of mild suture hyper-sensitivity presented with localized scleritis after strabismus surgery. After infection was ruled out, the patient was prescribed topical and systemic non-steroidal anti-inflammatory drugs and systemic steroids, which led to full clinical resolution. [J Pediatr Ophthalmol Strabismus. 2024;61(1):e4-e6.].
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Escleritis , Estrabismo , Femenino , Humanos , Anciano , Escleritis/diagnóstico , Escleritis/tratamiento farmacológico , Escleritis/etiología , Complicaciones Posoperatorias , Estrabismo/cirugía , Músculos Oculomotores/cirugía , Suturas/efectos adversosRESUMEN
OBJECTIVES: To describe the characteristics, treatment and outcome of isolated ANCA-associated scleritis at diagnosis compared with idiopathic scleritis with negative ANCA tests. METHODS: This retrospective multicentre case-control study was performed within the French Vasculitis Study Group (FVSG) network and in three French tertiary ophthalmologic centres. Data from patients with scleritis without any systemic manifestation and with positive ANCA results were compared with those of a control group of patients with idiopathic scleritis with negative ANCA tests. RESULTS: A total of 120 patients, including 38 patients with ANCA-associated scleritis and 82 control patients, diagnosed between January 2007 and April 2022 were included. The median follow-up was 28 months (IQR 10-60). The median age at diagnosis was 48 years (IQR 33-60) and 75% were females. Scleromalacia was more frequent in ANCA-positive patients (P = 0.027) and 54% had associated ophthalmologic manifestations, without significant differences. ANCA-associated scleritis more frequently required systemic medications, including glucocorticoids (76% vs 34%; P < 0.001), and rituximab (P = 0.03) and had a lower remission rate after the first- and second-line treatment. Systemic ANCA-associated vasculitis (AAV) occurred in 30.7% of patients with PR3- or MPO-ANCA, after a median interval of 30 months (IQR 16.3-44). Increased CRP >5 mg/l at diagnosis was the only significant risk factor of progression to systemic AAV [adjusted hazard ratio 5.85 (95% CI 1.10, 31.01), P = 0.038]. CONCLUSION: Isolated ANCA-associated scleritis is mostly anterior scleritis with a higher risk of scleromalacia than ANCA-negative idiopathic scleritis and is more often difficult to treat. One-third of patients with PR3- or MPO-ANCA scleritis progressed to systemic AAV.
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Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos , Escleritis , Femenino , Humanos , Masculino , Anticuerpos Anticitoplasma de Neutrófilos , Escleritis/diagnóstico , Escleritis/tratamiento farmacológico , Escleritis/etiología , Estudios de Casos y Controles , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/complicaciones , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/tratamiento farmacológico , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/diagnóstico , Rituximab/uso terapéutico , Estudios Retrospectivos , Peroxidasa , MieloblastinaRESUMEN
OBJECTIVES: To determine clinical features and outcomes of posterior scleritis masquerading as uveal melanoma following vaccination against COVID-19 and/or COVID-19 infection. SUBJECTS/METHODS: All patients with posterior scleritis referred to our service to rule out intraocular tumour between February 2021 and June 2022, who previously had COVID-19 vaccination and/or infection (n = 8). A retrospective detailed review of patient charts and imaging was carried out. RESULTS: Previous COVID-19 vaccination was documented in 6 patients (75%) and previous COVID-19 infection and vaccination in 2 patients (25%). Demographic features included mean age of 59 years (median 68, range 5-86 years), white race (n = 7, 87%), and male sex (n = 5, 63%). Mean visual acuity at presentation was 0.24 LogMAR (median 0.18, range 0.0-0.70). The main presenting symptom was blurred vision with pain (n = 5, 63%). Features that suggested scleritis and not uveal melanoma included pain (n = 6, 75%), anterior scleritis (n = 3, 38%), disc oedema (n = 1, 13%), choroidal detachment (n = 3, 38%), choroidal folds (n = 3, 38%), diffusely thickened scleral wall on ultrasonography (n = 2, 25%), Tenon's oedema (n = 5, 63%), and scleral nodule with medium/high internal reflectivity on ultrasonography (n = 4, 50%). Follow-up information at mean of 2 months (range 0.25-7 months) revealed visual acuity at date last seen was mean 0.30 LogMAR (median 0.29, range 0.0-0.54). By 2 months, resolution of "tumour" was noted in 5/6 (83%) patients with follow-up. CONCLUSIONS: Posterior scleritis following COVID-19 vaccination and/or infection can masquerade as choroidal melanoma. At 2 months duration, partial or complete resolution of features with minimal visual consequence was noted.
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COVID-19 , Melanoma , Escleritis , Humanos , Masculino , Preescolar , Niño , Adolescente , Adulto Joven , Adulto , Persona de Mediana Edad , Anciano , Anciano de 80 o más Años , Escleritis/diagnóstico , Escleritis/etiología , Melanoma/diagnóstico , Vacunas contra la COVID-19 , Estudios Retrospectivos , Edema , DolorRESUMEN
Purpose: This study aims to examine scleral thickness in patients with systemic lupus erythematosus (SLE) without clinically evident scleritis and episcleritis, utilizing swept-source optical coherence tomography (SS-OCT). Methods: This cross-sectional single center study compared scleral thickness (Nasal scleral thickness 1mm, 2mm, 3mm, 6mm from scleral spur; Temporal scleral thickness 1mm, 2mm, 3mm, 6mm from scleral spur) in 73 SLE patients without clinically evident scleritis and episcleritis and 48 healthy volunteers with SS-OCT. Further, we investigated the correlation between scleral thickness in SLE patients and various parameters including laboratory markers, disease duration, disease activity, and organ involvement. Results: Across all measured sites (nasal scleral thickness at distances of 1mm, 2mm, 3mm, and 6mm from the scleral spur, and temporal scleral thickness at the same distances), the scleral thickness in the SLE group was significantly greater than that in the control group (all p-values <0.001). SLE patients with a disease duration of 5 years or less exhibited a higher scleral thickness compared to those with a more prolonged disease duration. Patients with a higher erythrocyte sedimentation rate (ESR) had a thinner temporal scleral thickness. However, no significant associations were identified between scleral thickness and disease activity, organ involvement, or other laboratory markers. Conclusion: Scleral thickness measured by SS-OCT was higher in SLE patients than healthy controls. Changes in scleral thickness in SLE patients are related to disease duration and ESR. SS-OCT can detect asymptomatic structural changes in SLE patients and may be a useful tool in the evaluation of early scleral abnormality.
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Lupus Eritematoso Sistémico , Escleritis , Humanos , Esclerótica/diagnóstico por imagen , Escleritis/diagnóstico por imagen , Escleritis/etiología , Tomografía de Coherencia Óptica/métodos , Estudios Transversales , Lupus Eritematoso Sistémico/diagnóstico por imagen , BiomarcadoresRESUMEN
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)
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Humanos , Masculino , Anciano , Enfermedades Autoinflamatorias Hereditarias/complicaciones , Escleritis/etiología , Edema/etiología , SíndromeRESUMEN
A case of nodular posterior scleritis in a 25-year-old male who presented with a 14-day history of unilateral decline in vision, pain, and redness in his right eye. Slit lamp examination of the right eye revealed dilated episcleral vessels present nasally as well as a choroidal mass at the nasal periphery of the fundus, associated with choroidal oedema. Systemic evaluation and imaging of the choroidal mass were performed to rule out choroidal tuberculoma and choroidal metastasis. Ultrasound B-scan of the right eye showed marked thickening of the nasalsclera resulting in sympathetic choroidal oedema without the characteristic T-sign. Nodular posterior scleritis with associated choroiditis, was diagnosed without any underlying systemic illness. The patient was immediately started on systemic steroids and later on subcutaneous Methotrexate as advised by the rheumatologist, to which he responded well and his vision significantly improved from 6/60 to 6/9, gradually during his treatment course.
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Neoplasias de la Coroides , Coroiditis , Escleritis , Masculino , Humanos , Adulto , Neoplasias de la Coroides/diagnóstico por imagen , Escleritis/diagnóstico , Escleritis/etiología , Diagnóstico por Imagen , EdemaRESUMEN
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.
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Oftalmopatías , Escleritis , Masculino , Humanos , Anciano , Escleritis/tratamiento farmacológico , Escleritis/etiología , Ojo , Celulitis (Flemón) , Edema/etiologíaRESUMEN
Surgically induced necrotizing scleritis (SINS) is an uncommon but devastating complication that may occur days to years after ocular surgery. We report the case of a 32-year-old man who underwent uncomplicated strabismus surgery for large-angle exotropia and developed SINS characterized by painless scleral inflammation, choroidal exposure, and globe ectasia within days of surgery. Work-up revealed no associated infectious process or underlying systemic inflammatory condition. Clinical resolution occurred with oral immunosuppression alone, without need for graft.
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Exotropía , Oftalmología , Escleritis , Masculino , Humanos , Adulto , Escleritis/diagnóstico , Escleritis/tratamiento farmacológico , Escleritis/etiología , Procedimientos Quirúrgicos Oftalmológicos/efectos adversos , Esclerótica/trasplante , Exotropía/cirugíaRESUMEN
BACKGROUND: Uveitis and posterior scleritis are sight-threatening diseases with undefined pathogenesis and accurate diagnosis remains challenging. METHODS: Two plasma-derived extracellular vesicle (EV) subpopulations, small and large EVs, obtained from patients with ankylosing spondylitis-related uveitis, Behcet's disease uveitis, Vogt-Koyanagi-Harada syndrome, and posterior scleritis were subjected to proteomics analysis alongside plasma using SWATH-MS. A comprehensive bioinformatics analysis was performed on the proteomic profiles of sEVs, lEVs, and plasma. Candidate biomarkers were validated in a new cohort using ELISA. Pearson correlation analysis was performed to analyze the relationship between clinical parameters and proteomic data. Connectivity map database was used to predict therapeutic agents. RESULTS: In total, 3,668 proteins were identified and over 3000 proteins were quantified from 278 samples. When comparing diseased group to healthy control, the proteomic profiles of the two EV subgroups were more correlated with disease than plasma. Comprehensive bioinformatics analysis highlighted potential pathogenic mechanisms for these diseases. Potential biomarker panels for four diseases were identified and validated. We found a negative correlation between plasma endothelin-converting enzyme 1 level and mean retinal thickness. Potential therapeutic drugs were proposed, and their targets were identified. CONCLUSIONS: This study provides a proteomic landscape of plasma and EVs involved in ankylosing spondylitis-related uveitis, Behcet's disease uveitis, Vogt-Koyanagi-Harada syndrome, and posterior scleritis, offers insights into disease pathogenesis, identifies valuable biomarker candidates, and proposes promising therapeutic agents.