Ocular involvement (conjunctivitis/scleritis) as a form of presentation in ER-IgG4 in pediatric age

Autores: Luisina Zunino, Romina Calvo, Alberto Ortiz, Sergio Paira

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IgG4 related disease (ER-IgG4) in pediatric age has manifestations that differ from the ones of adults. There are only case reports and some systematic reviews in the literature. Furthermore, ocular involvement in this population (EOR-IgG4), although common in children, continues to be poorly defined due to the rarity of the disease and the lack of suspicion of it, which leads to a late diagnosis. The clinical case of a 12-year-old patient with EOR-IgG4 with a diagnostic delay of 4 years is described. She presented conjunctivitis, scleritis, ptosis, edema and ocular pain, unilaterally, without extra-ophthalmic involvement. Increased IgG4 and serum IgG4/IgG ratio, involvement of the central belly of the left internal rectus muscle and involvement of the eyelid on images. The pathology anatomy sample showed a lymphoplasmacytic infiltrate, with immunohistochemistry compatible with ER-IgG4. We highlight the importance of pediatric doctors taking this disease into account and knowing its different forms of presentation in this population.