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Ocular manifestations of IgG4-related disease in children. More common than anticipated? Review of the literature and case report

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Abstract

IgG4-related disease (IgG4-RD) is an entity with various clinical manifestations. Histopathologically, it is characterized by lymphoplasmacytic infiltrates enriched in IgG4 (+) plasmacytes and usually fibrosis of the affected tissue. Most of the patients have also increased IgG4 serum levels and they respond to glucocorticosteroids. In children, due to its rare occurrence, IgG4-RD is ill defined. From the published studies, so far, it appears that ocular manifestations are very common in the paediatric population with IgG4-RD. Herein, we describe a new case of a child with IgG4-RD with ocular involvement manifested with orbit and eyelid swelling, successfully treated with steroids. In addition, we review the clinical, laboratory, histopathologic and radiologic characteristics of the published paediatric cases with IgG4-RD and ocular involvement, critically comparing them with the characteristics of the adult population. It seems that ocular manifestations are more frequently observed in children than in adults. Also, the pattern of involvement is different, with extraocular muscles and soft tissues being more commonly affected than the lacrimal glands.

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Acknowledgements

We would like to thank Professor Haralampos M Moutsopoulos, MD, for critically reviewing our manuscript and for his continuous mentorship, guidance, inspiration and support.

We would also like to thank Dr. Ioannis Nikas for his valuable contribution in interpreting the radiological images of our case.

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Authors and Affiliations

  1. Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Mikras Asias 75 St., 11527, Athens, Greece

    Roubini G. Smerla & George E. Fragoulis

  2. Department of Pathology, “Evangelismos” General Hospital, Ipsilantou 45–47 St., 10676, Athens, Greece

    Dimitra Rontogianni

  3. Institute of Infection, Immunity and Inflammation, University of Glasgow, 120 University Place, GBRC Building G12 8TA, Glasgow, UK

    George E. Fragoulis

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  1. Roubini G. Smerla
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  2. Dimitra Rontogianni
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Correspondence to George E. Fragoulis.

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Smerla, R.G., Rontogianni, D. & Fragoulis, G.E. Ocular manifestations of IgG4-related disease in children. More common than anticipated? Review of the literature and case report. Clin Rheumatol 37, 1721–1727 (2018). https://doi.org/10.1007/s10067-017-3934-9

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  • DOI: https://doi.org/10.1007/s10067-017-3934-9

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