Conjunctival Infiltration in a Child as a Rare Manifestation of IgG4-Related Disease.
Angiogenesis Inhibitors
/ therapeutic use
Child
Conjunctival Diseases
/ diagnosis
Female
Glucocorticoids
/ therapeutic use
Humans
Immunoglobulin G4-Related Disease
/ diagnosis
Lacrimal Apparatus Diseases
/ diagnosis
Orbital Diseases
/ diagnosis
Prednisolone
/ therapeutic use
Rituximab
/ therapeutic use
Vascular Endothelial Growth Factor A
/ antagonists & inhibitors
Journal
Cornea
ISSN: 1536-4798
Titre abrégé: Cornea
Pays: United States
ID NLM: 8216186
Informations de publication
Date de publication:
01 Apr 2022
01 Apr 2022
Historique:
received:
21
01
2021
accepted:
05
04
2021
pubmed:
30
5
2021
medline:
12
3
2022
entrez:
29
5
2021
Statut:
ppublish
Résumé
Ocular manifestations of immunoglobulin G4 (IgG4)-related disease are common in children although remain ill-defined because of the disease's rarity. We describe a pediatric case of IgG4-related orbital disease (IgG4-ROD) who presented with persistent conjunctival infiltration before developing lacrimal gland enlargement 3 years later. This was a case report. An 8-year-old girl developed forniceal salmon-patch-like conjunctival lesions in her left eye that were refractory to topical corticosteroids. Investigations, including an orbital MRI and 2 conjunctival biopsies, were negative for lymphoma. She was treated with topical corticosteroids and then nonsteroidal antiinflammatory drops. The lesions decreased mildly, and no new lesion emerged. After 3 years, the patient developed a ptosis, new salmon-patch conjunctival lesions, and papillae. Vision deteriorated to 20/80 because of severe punctate epithelial erosions in the left eye, and the Schirmer test was significantly reduced. A repeat MRI revealed an enlarged left lacrimal gland. A biopsy was performed and was compatible with IgG4-ROD. An elevated IgG4 level of 4.61 g/L was also found. The patient was successfully treated with oral prednisone but flared on tapering the dosage. Rituximab was therefore initiated with excellent clinical response, and prednisone was discontinued. Vision returned to 20/20 after aggressive lubrification, punctal plugs, and autologous serum eye drops. Tear function came back to normal, and local treatments were stopped. This case describes a pediatric case of IgG4-ROD presenting initially with conjunctival follicular reaction, later developing lacrimal gland involvement. Therefore, it is important to consider IgG4-ROD in chronic atypical follicular conjunctival lesions in children, even in the absence of orbital disease.
Identifiants
pubmed: 34050069
doi: 10.1097/ICO.0000000000002773
pii: 00003226-202204000-00014
doi:
Substances chimiques
Angiogenesis Inhibitors
0
Glucocorticoids
0
VEGFA protein, human
0
Vascular Endothelial Growth Factor A
0
Rituximab
4F4X42SYQ6
Prednisolone
9PHQ9Y1OLM
Types de publication
Case Reports
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
496-498Informations de copyright
Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.
Déclaration de conflit d'intérêts
The authors have no funding or conflicts of interest to disclose.
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