RESUMO
Nonspecific orbital inflammation (NSOI), colloquially known as orbital pseudotumor, sometimes presents a diagnostic and therapeutic challenge in ophthalmology. This review aims to dissect NSOI through a molecular lens, offering a comprehensive overview of its pathogenesis, clinical presentation, diagnostic methods, and management strategies. The article delves into the underpinnings of NSOI, examining immunological and environmental factors alongside intricate molecular mechanisms involving signaling pathways, cytokines, and mediators. Special emphasis is placed on emerging molecular discoveries and approaches, highlighting the significance of understanding molecular mechanisms in NSOI for the development of novel diagnostic and therapeutic tools. Various diagnostic modalities are scrutinized for their utility and limitations. Therapeutic interventions encompass medical treatments with corticosteroids and immunomodulatory agents, all discussed in light of current molecular understanding. More importantly, this review offers a novel molecular perspective on NSOI, dissecting its pathogenesis and management with an emphasis on the latest molecular discoveries. It introduces an integrated approach combining advanced molecular diagnostics with current clinical assessments and explores emerging targeted therapies. By synthesizing these facets, the review aims to inform clinicians and researchers alike, paving the way for molecularly informed, precision-based strategies for managing NSOI.
Assuntos
Cristalino , Oftalmologia , Pseudotumor Orbitário , Humanos , Inflamação/diagnóstico , Inflamação/terapia , Pseudotumor Orbitário/diagnóstico , Pseudotumor Orbitário/patologia , Cristalino/patologia , CitocinasRESUMO
A 21-year-old female presented with periocular swelling, diplopia, and painful ophthalmoplegia in the OS. Orbital magnetic resonance imaging revealed an enhanced soft tissue mass involving the left medial rectus muscle. Laboratory test results revealed leukocytosis, elevated reactive C-reactive protein, and positive serum levels of anti-mumps immunoglobulin M (IgM) antibody without systemic manifestations of mumps infection. The clinical course was refractory, and the patient showed a poor response to high-dose steroids. An incisional biopsy revealed stromal fibrosis with focal lymphoid aggregates, indicating sclerosing inflammation. Myopathy of the medial rectus progressed to superior, inferior, and lateral recti involvement of the left orbit. Immunosuppressive agents, including steroids, were administered for 22 months after disease onset. The mumps IgM antibody level was positive for over 5 months and became negative upon testing performed 1 year after the first visit.
Assuntos
Anticorpos Antivirais , Imunoglobulina M , Pseudotumor Orbitário , Feminino , Humanos , Adulto Jovem , Anticorpos Antivirais/sangue , Biópsia , Imunoglobulina M/sangue , Imageamento por Ressonância Magnética , Músculos Oculomotores/patologia , Pseudotumor Orbitário/diagnóstico , Pseudotumor Orbitário/imunologiaRESUMO
PURPOSE: The high recurrence rate of idiopathic orbital inflammation (IOI) has been reported. This study aims to determine existing predictive factors for the recurrence of IOI. METHODS: This was an 11-year retrospective study with at least a 12-month follow-up. Fifty patients with biopsy-proven IOI admitted between 2006 and 2017 at our tertiary hospital were observed. We compared the clinical characteristics, histopathological profile, and biomarker expressions (mast cell, immunoglobulin G4, tumor necrosis factor-alpha, and transforming growth factor-beta) of 16 patients with recurrence (Group I) and 34 patients with no recurrence (Group II). Statistical comparison and multivariate analysis were performed to establish the predictive factors. RESULTS: We discovered five recurrence predictive factors: presentation of proptosis (odds ratio [OR] 4.96, 95% confidence interval [CI] 1.36-18.03), visual impairment (OR 15, 95% CI 1.58-142.72), extraocular muscle (EOM) restriction (OR 3.86, 95% CI 1.07-13.94), nonanterior involvement (OR 7.94, 95% CI 1.88-33.5), and corticosteroid (CS) alone treatment (OR 7.20, 95% CI 1.87-27.8). On multivariate analysis, nonanterior involvement and CS alone treatment were validated as predictive factors (area under the curve = 0.807 [95% CI 0.69-0.92]). Histopathological profile and biomarker expressions were not associated with recurrence. However, there was a 22-fold higher recurrence risk for granulomatous-type patients given CS alone treatment. CONCLUSION: Unlike the five clinical characteristics mentioned, both histopathology and biomarker variables were not associated with recurrence. CS alone treatment for patients with nonanterior involvement or granulomatous type is proven to increase the risk of recurrence. Therefore, we suggest not giving CS without any combination treatment with other modalities for this group of patients.
Assuntos
Pseudotumor Orbitário , Recidiva , Humanos , Estudos Retrospectivos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Pseudotumor Orbitário/diagnóstico , Seguimentos , Adulto Jovem , Biópsia , Adolescente , Idoso , Fatores de RiscoRESUMO
RESUMEN El origen del pseudotumor orbitario no es del todo conocido. Se admite su naturaleza inflamatoria granulomatosa e inespecífica en diferentes localizaciones. El pseudotumor orbitario se define como una respuesta inflamatoria celular pleomórfica, que está usualmente confinado a estructuras de la órbita y tiene una evolución limitada. En este trabajo se presenta una paciente femenina de 16 años, con diagnóstico de pseudotumor orbitario corroborado por biopsia y tomografía axial computarizada, refractaria al tratamiento con esteroides sistémicos, por lo que se decide iniciar con la aplicación de hialuronidasa y triamcinolona en el espacio peribulbar. Los casos agudos casi siempre responden rápidamente al tratamiento con cortocoesteroides, como prednisona, pero debemos tener en cuenta que existen pacientes que son refractarios al tratamiento, por lo que es necesario buscar procedimientos alternativos. Una opción es el uso de hialuronidasa para destruir las uniones extracelulares, y difundir un esteroide de manera local, como la triamcinolona, más efectiva dentro del tejido inflamatorio para provocar un efecto localizado de este. A los tres meses del tratamiento hubo una regresión total del cuadro en esta paciente(AU)
ABSTRACT The exact etiology of orbital pseudotumor is unknown, but its granulomatous unspecific inflammatory nature at various locations has been recognized. Orbital pseudotumor is defined as a cellular pleomorphic inflammatory response of limited evolution often confined to orbital structures. A case is presented of a female 16-year-old patient diagnosed with orbital pseudotumor confirmed by biopsy and computerized axial tomography, refractory to treatment with systemic steroids, due to which it is decided to start treatment with hyaluronidase and triamcinolone in the peribulbar space. Acute cases often respond fast to treatment with corticosteroids such as prednisone. It should be borne in mind that there are patients who are refractory to treatment for whom alternative treatments should be sought. An option is the use of hyaluronidase to destroy extracellular junctions and locally spread a steroid such as triamcinolone, most effectively within the inflammatory tissue to ensure its localized effect. Total regression of the patient's status was observed at three months of treatment(AU)
Assuntos
Humanos , Feminino , Adolescente , Triancinolona/uso terapêutico , Pseudotumor Orbitário/diagnóstico , Hialuronoglucosaminidase/uso terapêuticoRESUMO
Resumo O pseudotumor orbitário é uma doença inflamatória idiopática benigna. Os autores apresentam um caso manifestado em adolescente de 12 anos, diagnosticado por meio do exame clínico, laboratorial e radiológico. Houve boa resposta ao tratamento proposto com corticosteroides. O relato é seguido de breve retomada literária acerca do tema.
Abstract The orbital pseudotumor is a benign idiopathic inflammatory disease. The authors present a case manifested in 12 years old boy, diagnosed by clinical, laboratory and radiological examination. There was a good response to treatment with corticosteroids proposed. The report is followed by brief literary resume on the subject.
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Humanos , Masculino , Criança , Pseudotumor Orbitário/diagnóstico , Pseudotumor Orbitário/tratamento farmacológico , Órbita/diagnóstico por imagem , Recidiva , Prednisona/uso terapêutico , Imageamento por Ressonância Magnética , Tomografia Computadorizada por Raios XRESUMO
RESUMO O pseudotumor esclerosante de órbita é um subtipo raro de pseudotumor inflamatório idiopático de órbita. É mais comum em adultos e apresenta diagnóstico de exclusão. A primeira linha de tratamento são os esteroides. O subtipo esclerosante apresenta resposta moderada aos esteroides devido à predominância de fibrose e colágeno na histologia. Relatamos o caso de um paciente com diagnóstico histológico de pseudotumor esclerosante de órbita que teve boa resposta ao tratamento com corticoide associado à azatioprina.
ABSTRACT Sclerosing orbital pseudotumor is a rare subtype of idiopathic orbital inflammatory pseudotumor. It's more common in adults and presents diagnosis of exclusion. Steroids represent the first option of treatment. The sclerosing orbital pseudotumor subtype shows moderate response to steroids due to the predominance of fibrosis and collagen in its histology. We report on a case of a patient with histologic diagnosis of sclerosing orbital pseudotumor successfully treated with corticosteroid associated with azathioprine.
Assuntos
Humanos , Masculino , Adulto , Pseudotumor Orbitário/diagnóstico , Azatioprina/uso terapêutico , Biópsia , Prednisona/uso terapêutico , Pseudotumor Orbitário/patologia , Pseudotumor Orbitário/tratamento farmacológico , Túnica Conjuntiva/patologia , Cápsula de Tenon/patologia , Microscopia com Lâmpada de Fenda , InflamaçãoRESUMO
Orbital myositis implies orbital inflammation confined to one or more of the extraocular muscles. The acute form responds well to high doses of oral corticosteroids tapered gradually, but it may recur or become chronic. We describe a 38 years old female who has been suffering from rheumatoid arthritis for six years. She developed diplopia as a result of a paralysis of the right and left rectus medialis muscle. MRI showed inflammatory process and thickness of the referred muscles. The patient had a total recovery with oral use of 80 mg methylpredinisolone daily. Two months after the first episode she developed a bilateral ophthalmoplegy. The patient improved with oral use of steroids the second time, but a paresis of the left rectus lateralis muscle remained. From the 156 cases we reviewed only three have been related to rheumatic diseases and none has been previously related to rheumatoid arthritis.
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Humanos , Feminino , Adulto , Artrite Reumatoide/complicações , Pseudotumor Orbitário/complicações , Anti-Inflamatórios/uso terapêutico , Imageamento por Ressonância Magnética , Metilprednisolona/uso terapêutico , Pseudotumor Orbitário/diagnóstico , Pseudotumor Orbitário/tratamento farmacológico , RecidivaRESUMO
El propósito de este estudio fue la revisión de nuestra experiencia clínica con 24 casos observados de enfermedad inflamatoria idiopática de la órbita entre 1980 y 1996, el cuadro clínico de los pacientes fue evaluado retrospectivamente en la Unidad de Neuro-Oftalmología del Hospital Vargas de Caracas. Pacientes con promedio de edades desde 13 a 76 (media 42,66) años. Los tres signos principales más comunes fueron: Proptosis (74,99 por ciento), Dolor periorbitario (58,33 por ciento), Diplopía (54,16 por ciento). El diagnóstico fue realizado por clínica y estudios complementarios. Patrones imagenológicos fueron observados en 10 casos, siendo la enfermedad inflamatoria difusa la más común, segudo por el Síndrome Tolosa Hunt (3 casos). Dacrioadenitis (3 casos) y periescleritis (1 caso). La respuesta a tratamiento fue conocida para 18 de los 24 casos clasificados imagenológicamente. El tratamiento con altas dosis de corticosteroides fue bueno en 11 de 24 (45,83 por ciento) de los casos. Dos casos resistentes a corticosteroides fueron tratados con radioterapia. Un caso resistente respondió a ciclofosfamida. Tres casos adicionales tuvieron remisión espontánea. Sin embargo, 16 de 24 pacientes (66,67 por ciento) tuvieron una eventual buen resulatdo. En base a estos resultados recomendamos el uso de corticosteroides como tratamiento inicial en pacientes con EIIO
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Humanos , Masculino , Feminino , Doenças Orbitárias , Radioterapia , Pseudotumor Orbitário/classificação , Pseudotumor Orbitário/diagnóstico , Pseudotumor Orbitário/patologia , Corticosteroides/uso terapêutico , OftalmologiaRESUMO
Os autores apresentam análise retrospectiva de 20 pacientes com processo inflamatório intra-orbital, estudados no Hospital Universitário Clementino Fraga Filho, da Universidade Federal do Rio de Janeiro. Os processos inflamatórios observados foram a celulite, os pseudotumores e a miosite. Na celulite, os principais achados tomográficos foram o aumento das partes moles e a exoftalmia. Outros achados que ajudam neste diagnóstico säo: velamento dos seios paranasais, velamento da gordura retrobulbar, comprometimento muscular, gás em partes moles e aumento da glândula lacrimal. Nos pseudotumores, ausência de erosäo óssea. Em relaçäo à miosite, encontraram-se proptose do globo ocular e espessamento muscular
Assuntos
Humanos , Masculino , Feminino , Lactente , Pré-Escolar , Criança , Adolescente , Adulto , Pessoa de Meia-Idade , Celulite (Flegmão)/diagnóstico , Pseudotumor Orbitário/diagnóstico , Tomografia Computadorizada por Raios XRESUMO
Enfermedad inflamatoria idiopática y atrofia hemifacial progresiva o síndrome de Parry-Romberg. La autora comunica el primer caso donde dos entidades de etiología desconocida se presentan en asociación, compartiendo características clínicas e histopatológicas. Se describe el caso de una paciente femenina cuya enfermedad se inicia a los 13 años de edad, con atrofia hemifacial izquierda, sin compromiso óseo, acentuándose en forma progresiva en el tiempo, hasta evidenciarse en la frente el signo "coup de sabre". A los 17 años presenta neuropatía óptica, restricción de la motilidad ocular y edema hemifacial izquierdo, simulando una celulitis orbitaria. Realizados los estudios adecuados y descartándose otras patologías inflamatorias de la órbita, se diagnostica una enfermedad inflamatoria aguda de la órbita, se inicia un tratamiento con prednisona y la respuesta terapéutica es favorable desde las primeras cuarenta y ocho horas, desapareciendo los signos inflamatorios, pero dejando como secuelas una neuropatía óptica residual expresada por la disminución de la agudeza visual, escotoma central y palidez del disco óptico izquierdo