Peripheral Ulcerative Keratitis as an Initial Manifestation of Granulomatosis with Polyangiitis in a Pediatric Patient: A Case Report

Juanita Cardona-López; Catalina Mosquera; Alejandra de-la-Torre

doi:10.1080/09273948.2025.2573734

Peripheral Ulcerative Keratitis as an Initial Manifestation of Granulomatosis with Polyangiitis in a Pediatric Patient: A Case Report

Título traducido de la contribución: Queratitis ulcerosa periférica como manifestación inicial de granulomatosis con poliangitis en un paciente pediátrico: un caso clínico

Juanita Cardona-López
, Catalina Mosquera
, Alejandra de-la-Torre

Producción científica: Contribución a revista › Estudio breve › revisión exhaustiva

Resumen

Objetivo
Describir un caso raro de granulomatosis pediátrica con poliangeítis (GPA) que se presentó inicialmente como queratitis ulcerosa periférica (PUK), seguida de inflamación orbitaria, destacando los desafíos diagnósticos y la importancia del reconocimiento temprano.

Métodos
Un niño de 12 años con antecedentes de conjuntivitis alérgica presentó opacidades corneales periféricas unilaterales. Inicialmente, la afección se diagnosticó erróneamente y se trató como queratitis infecciosa y conjuntivitis alérgica, sin mejoría. Se realizaron evaluaciones clínicas, serológicas y de imagen exhaustivas, incluyendo pruebas de ANCA, tomografías computarizadas de tórax y órbita, y serologías infecciosas.

Resultados
Las pruebas de laboratorio revelaron marcadores inflamatorios elevados, p-ANCA positivos y títulos elevados de anticuerpos antimieloperoxidasa (MPO). La TC de tórax mostró nódulos pulmonares compatibles con vasculitis. Posteriormente, el paciente desarrolló pseudotumor orbitario y epistaxis recurrente, lo que consolidó el diagnóstico de GPA a pesar de la positividad para MPO-ANCA, que se asocia con mayor frecuencia a poliangeítis microscópica. El tratamiento inmunosupresor con corticosteroides sistémicos y metotrexato condujo a la remisión completa de la inflamación ocular, la resolución de la afectación orbitaria y la preservación de una agudeza visual bilateral de 20/20 tras un año de seguimiento.

Conclusión
Este caso representa una presentación pediátrica excepcional de GPA, con queratitis periférica atípica como única manifestación inicial que precede a la enfermedad sistémica. Esto subraya la necesidad de una mayor sospecha clínica en niños con queratitis periférica atípica que no responden al tratamiento estándar. El diagnóstico temprano y el tratamiento multidisciplinario son cruciales para prevenir la pérdida visual irreversible y las complicaciones sistémicas.

Título traducido de la contribución	Queratitis ulcerosa periférica como manifestación inicial de granulomatosis con poliangitis en un paciente pediátrico: un caso clínico
Idioma original	Inglés estadounidense
Publicación	Ocular Immunology and Inflammation
DOI	https://doi.org/10.1080/09273948.2025.2573734
Estado	Publicada - oct 10 2025

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@article{262ae35a7dee48669f128af5d84db2a1,

title = "Peripheral Ulcerative Keratitis as an Initial Manifestation of Granulomatosis with Polyangiitis in a Pediatric Patient: A Case Report",

abstract = "Purpose To describe a rare case of pediatric granulomatosis with polyangiitis (GPA) initially presenting as peripheral ulcerative keratitis (PUK), followed by orbital inflammation, highlighting the diagnostic challenges and importance of early recognition. Methods A 12-year-old boy with a prior history of allergic conjunctivitis presented with unilateral peripheral corneal opacities. The condition was initially misdiagnosed and treated as infectious keratitis and allergic conjunctivitis without improvement. Comprehensive clinical, serologic, and imaging evaluations were performed, including ANCA testing, chest and orbital CT scans, and infectious serologies. Results Laboratory workup revealed elevated inflammatory markers, positive p-ANCA, and high anti-myeloperoxidase (MPO) antibody titers. Chest CT showed pulmonary nodules consistent with vasculitic involvement. The patient subsequently developed orbital pseudotumor and recurrent epistaxis, consolidating the diagnosis of GPA despite MPO-ANCA positivity, which is more commonly linked to microscopic polyangiitis. Immunosuppressive therapy with systemic corticosteroids and methotrexate led to complete remission of ocular inflammation, resolution of orbital involvement, and preservation of 20/20 visual acuity bilaterally after one year of follow-up. Conclusion This case represents an exceptional pediatric presentation of GPA, with PUK as the sole initial manifestation preceding systemic disease. It underscores the need for heightened clinical suspicion in children with atypical peripheral keratitis unresponsive to standard therapy. Early recognition and multidisciplinary management are crucial to prevent irreversible visual loss and systemic complications.",

author = "Juanita Cardona-L{\'o}pez and Catalina Mosquera and Alejandra de-la-Torre",

year = "2025",

month = oct,

day = "10",

doi = "10.1080/09273948.2025.2573734",

language = "English (US)",

journal = "Ocular Immunology and Inflammation",

issn = "0927-3948",

publisher = "Taylor and Francis Ltd.",

}

TY - JOUR

T1 - Peripheral Ulcerative Keratitis as an Initial Manifestation of Granulomatosis with Polyangiitis in a Pediatric Patient: A Case Report

AU - Cardona-López, Juanita

AU - Mosquera, Catalina

AU - de-la-Torre, Alejandra

PY - 2025/10/10

Y1 - 2025/10/10

N2 - Purpose To describe a rare case of pediatric granulomatosis with polyangiitis (GPA) initially presenting as peripheral ulcerative keratitis (PUK), followed by orbital inflammation, highlighting the diagnostic challenges and importance of early recognition. Methods A 12-year-old boy with a prior history of allergic conjunctivitis presented with unilateral peripheral corneal opacities. The condition was initially misdiagnosed and treated as infectious keratitis and allergic conjunctivitis without improvement. Comprehensive clinical, serologic, and imaging evaluations were performed, including ANCA testing, chest and orbital CT scans, and infectious serologies. Results Laboratory workup revealed elevated inflammatory markers, positive p-ANCA, and high anti-myeloperoxidase (MPO) antibody titers. Chest CT showed pulmonary nodules consistent with vasculitic involvement. The patient subsequently developed orbital pseudotumor and recurrent epistaxis, consolidating the diagnosis of GPA despite MPO-ANCA positivity, which is more commonly linked to microscopic polyangiitis. Immunosuppressive therapy with systemic corticosteroids and methotrexate led to complete remission of ocular inflammation, resolution of orbital involvement, and preservation of 20/20 visual acuity bilaterally after one year of follow-up. Conclusion This case represents an exceptional pediatric presentation of GPA, with PUK as the sole initial manifestation preceding systemic disease. It underscores the need for heightened clinical suspicion in children with atypical peripheral keratitis unresponsive to standard therapy. Early recognition and multidisciplinary management are crucial to prevent irreversible visual loss and systemic complications.

AB - Purpose To describe a rare case of pediatric granulomatosis with polyangiitis (GPA) initially presenting as peripheral ulcerative keratitis (PUK), followed by orbital inflammation, highlighting the diagnostic challenges and importance of early recognition. Methods A 12-year-old boy with a prior history of allergic conjunctivitis presented with unilateral peripheral corneal opacities. The condition was initially misdiagnosed and treated as infectious keratitis and allergic conjunctivitis without improvement. Comprehensive clinical, serologic, and imaging evaluations were performed, including ANCA testing, chest and orbital CT scans, and infectious serologies. Results Laboratory workup revealed elevated inflammatory markers, positive p-ANCA, and high anti-myeloperoxidase (MPO) antibody titers. Chest CT showed pulmonary nodules consistent with vasculitic involvement. The patient subsequently developed orbital pseudotumor and recurrent epistaxis, consolidating the diagnosis of GPA despite MPO-ANCA positivity, which is more commonly linked to microscopic polyangiitis. Immunosuppressive therapy with systemic corticosteroids and methotrexate led to complete remission of ocular inflammation, resolution of orbital involvement, and preservation of 20/20 visual acuity bilaterally after one year of follow-up. Conclusion This case represents an exceptional pediatric presentation of GPA, with PUK as the sole initial manifestation preceding systemic disease. It underscores the need for heightened clinical suspicion in children with atypical peripheral keratitis unresponsive to standard therapy. Early recognition and multidisciplinary management are crucial to prevent irreversible visual loss and systemic complications.

U2 - 10.1080/09273948.2025.2573734

DO - 10.1080/09273948.2025.2573734

M3 - Short survey

C2 - 41074492

SN - 0927-3948

JO - Ocular Immunology and Inflammation

JF - Ocular Immunology and Inflammation

ER -

Peripheral Ulcerative Keratitis as an Initial Manifestation of Granulomatosis with Polyangiitis in a Pediatric Patient: A Case Report

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