European Journal of Rheumatology
Invited Review

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

1.

Department of Pediatrics, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden, Germany

2.

Department of Radiology, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden, Germany

3.

Department of Ophthalmology, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden, Germany

4.

Department of Pathology, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden, Germany

5.

Department of Women's and Children's Health, Institute of Translational Medicine, University of Liverpool School of Life Sciences, Liverpool, UK

6.

Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, UK

Eur J Rheumatol 2020; 7: Supplement S21-S27
DOI: 10.5152/eurjrheum.2019.19165
Read: 91 Downloads: 67 Published: 31 January 2020

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.

Cite this article as: Tille L, Schnabel A, Laass MW, Hahn G, Taut H, Leszczynska A, et al.  Orbital inflammation and colitis in pediatric IgG4- related disease: A case report and review of the literature. Eur J Rheumatol 2020; 7(Suppl 1): S21-S28.

Files
ISSN2147-9720 EISSN 2148-4279
3