Orbital inflammation and colitis in pediatric IgG4-related disease: A case report and review of the literature.



Tille, Lissy ORCID: 0000-0002-2880-3126, Schnabel, Anja ORCID: 0000-0001-8716-2552, Laass, Martin W, Hahn, Gabriele, Taut, Heike, Leszczynska, Anna ORCID: 0000-0002-6668-0438, Pablik, Jessica ORCID: 0000-0003-1044-2972, Berner, Reinhard ORCID: 0000-0002-6216-9173, Brück, Normi and Hedrich, Christian M
(2019) Orbital inflammation and colitis in pediatric IgG4-related disease: A case report and review of the literature. European journal of rheumatology. 1 - 7.

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Abstract

IgG4-related disease (IgG4-RD) is an inflammatory disorder characterized by tumor-like swelling in one or more organs, elevated serum IgG4 levels, and histological alterations with infiltration of IgG4-positive plasma cells. IgG4-RD is rare and likely underdiagnosed in children. We report a case of a 16-year-old girl with IgG4-positive colitis that developed weeks after IgG4-related ophthalmic disease and discuss diagnosis and treatment in the context of the literature available. Since the pathophysiology of IgG4-RD is unknown, treatment options are empiric and, for the most part, untargeted. Systemic corticosteroid treatment is the basis of anti-inflammatory treatment in IgG4-RD and induced early remission in our patient. During corticosteroid taper, the patient developed weight loss and intestinal inflammation. Histopathological assessment of the intestinal walls confirmed IgG4-positive colitis. Immune-modulating treatment with non-biologic (e.g., methotrexate (MTX) and mycophenolate mofetil) or biologic (rituximab) disease-modifying antirheumatic drugs has been reported in treatment refractory or corticosteroid-dependent patients. The patient responded to treatment with anti-inflammatory therapy with food rich in TGF-β2 (modulen) and MTX. This is one of the first pediatric patients reported with IgG4-related colitis extending the phenotype of pediatric IgG4-RD. International collaboration to prospectively document clinical presentation and treatment responses may help to further establish the phenotype and treatment options and to raise awareness for IgG4-RD.

Item Type: Article
Depositing User: Symplectic Admin
Date Deposited: 21 Feb 2020 14:08
Last Modified: 03 Mar 2021 10:39
DOI: 10.5152/eurjrheum.2019.19165
Open Access URL: https://www.eurjrheumatol.org/en/orbital-inflammat...
URI: https://livrepository.liverpool.ac.uk/id/eprint/3076044