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Vol. 57. Issue 11.
Pages 702 (November 2021)
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Anterior mediastinal mass associated with febrile syndrome as a single manifestation of IgG4-related disease
Masa mediastínica anterior asociada a síndrome febril como única manifestación de enfermedad relacionada con IgG4
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María Dolores Rabadán-Caravaca
Corresponding author
mariadolrc@gmail.com

Corresponding author.
, Eduardo Ruiz-Carazo, María Dolores García-Roa
Departamento de Radiodiagnóstico, Hospital Universitario Virgen de las Nieves, Granada, Spain
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A 14-year-old boy with no significant clinical history consulted with a 6-month history of febrile syndrome. Labs were significant for leukocytosis and elevated C-reactive protein and serum IgG levels, specifically IgG4. Computed tomography, magnetic resonance imaging, and PET/CT showed a right paradiaphragmatic anterior mediastinal mass of infiltrating appearance with intense heterogeneous enhancement (Figs. 1A–C) and marked hypermetabolism (Fig. 1D). Pathology study of a percutaneous biopsy revealed an inflammatory lesion associated with IgG4-related disease. The clinical picture resolved completely after 4 months of treatment with glucocorticoids (Fig. 1E).

Fig. 1.

Chest-abdomen-pelvis computed tomography with intravenous contrast in axial (A) and coronal (B) planes, showing the anterior mediastinal mass with heterogeneous enhancement and an infiltrative appearance (red arrows). T2-weighted MRI image with fat suppression (C) identifying the hyperintense anterior mediastinal mass (red arrow). The lesion was markedly hypermetabolic on the initial PET/CT images (SUVmax = 11.9) (D) and disappeared on PET/CT after 4 months of treatment (E).

(0.08MB).

IgG4-related disease is an entity associated with autoimmune mechanisms, characterized by elevated IgG4 serum levels, fibrosis, tissue infiltration by IgG4-secreting plasma cells, and response to glucocorticoids1.

Mediastinal involvement usually manifests as lymphadenopathies, either in isolation or concomitantly with the enlargement of other organs1. Presentation in the form of a mass is exceptional2.

In the case of a patient with a mediastinal mass, IgG4-related disease should be included in the differential diagnosis. Proper diagnosis and conservative treatment can prevent unnecessary invasive diagnostic or therapeutic procedures and their associated comorbidities.

References
[1]
A. Martínez-de-Alegría, S. Baleato-González, R. García-Figueiras, A. Bermúdez- Naveira, I. Abdulkader-Nallib, J.A. Díaz-Peromingo.
IgG4-related disease from head to toe.
Radiographics., 35 (2015), pp. 2007-2025
[2]
R. Yoshikawa, H. Igai, F. Ohsawa, T. Yazawa, M. Kamiyoshihara.
Immunoglobulin G4 related disease association with an isolated anterior mediastinal tumor.
Ann Thorac Surg., 107 (2019), pp. 405-407

Please cite this article as: Rabadán-Caravaca MD, Ruiz-Carazo E, García-Roa MD. Masa mediastínica anterior asociada a síndrome febril como única manifestación de enfermedad relacionada con IgG4. Arch Bronconeumol. 2021;57:702.

Copyright © 2020. SEPAR
Archivos de Bronconeumología

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