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

Tille, Lissy, Schnabel, Anja, Laass, Martin W, Hahn, Gabriele, Taut, Heike, Leszczynska, Anna, Pablik, Jessica, Berner, Reinhard, Bruck, Normi and Hedrich, Christian M ORCID: 0000-0002-1295-6179
(2020) Orbital inflammation and colitis in pediatric IgG4-related disease: A case report and review of the literature. EUROPEAN JOURNAL OF RHEUMATOLOGY, 7 (Suppl1). S21-S27.

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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
Uncontrolled Keywords: IgG4, IgG4-related disease, pediatric, orbital inflammation, colitis
Depositing User: Symplectic Admin
Date Deposited: 21 Feb 2020 14:08
Last Modified: 22 Aug 2023 00:13
DOI: 10.5152/eurjrheum.2019.19165
Open Access URL: https://www.eurjrheumatol.org/en/orbital-inflammat...
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URI: https://livrepository.liverpool.ac.uk/id/eprint/3076044