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Severe IgG4-Related Disease in a Young Child: A Diagnosis Challenge

Immunoglobulin G4-related disease (IgG4-RD) is an increasingly recognized syndrome that can appear with multiple organ involvement, typically with tumor-like swelling, lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, and elevated serum IgG4 concentrations. We report the case of a 22-...

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Autores principales: Corujeira, Susana, Ferraz, Catarina, Nunes, Teresa, Fonseca, Elsa, Vaz, Luísa Guedes
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4325228/
https://www.ncbi.nlm.nih.gov/pubmed/25705537
http://dx.doi.org/10.1155/2015/140753
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author Corujeira, Susana
Ferraz, Catarina
Nunes, Teresa
Fonseca, Elsa
Vaz, Luísa Guedes
author_facet Corujeira, Susana
Ferraz, Catarina
Nunes, Teresa
Fonseca, Elsa
Vaz, Luísa Guedes
author_sort Corujeira, Susana
collection PubMed
description Immunoglobulin G4-related disease (IgG4-RD) is an increasingly recognized syndrome that can appear with multiple organ involvement, typically with tumor-like swelling, lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, and elevated serum IgG4 concentrations. We report the case of a 22-month-old female child with failure to thrive and recurrent respiratory tract infections since 8 months of age. Physical examination was normal except for pulmonary auscultation with bilateral crackles and wheezes. Laboratory tests revealed elevated erythrocyte sedimentation rate, and elevated serum IgG and IgG4 with polyclonal hypergammaglobulinemia. Thoracic CT and MRI showed multiple mediastinal lymphadenopathies and a nodular posterior mediastinal mass in right paratracheal location with bronchial compression. Initial fine needle aspiration biopsy was compatible with reactive lymphadenopathy but after clinical worsening a thoracoscopic partial resection of the mass was performed and tissue biopsy revealed lymphoplasmacytic infiltrate and increased number of IgG4-positive plasma cells and a ratio of IgG4/IgG positive cells above 40%. Glucocorticoids therapy was started with symptomatic improvement, reduction in the size of the mass, and decrease of serum IgG4 levels after 6 weeks. There are very few reports of IgG4-RD in children. Long-term follow-up is necessary to monitor relapses and additional organ involvement.
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spelling pubmed-43252282015-02-22 Severe IgG4-Related Disease in a Young Child: A Diagnosis Challenge Corujeira, Susana Ferraz, Catarina Nunes, Teresa Fonseca, Elsa Vaz, Luísa Guedes Case Rep Pediatr Case Report Immunoglobulin G4-related disease (IgG4-RD) is an increasingly recognized syndrome that can appear with multiple organ involvement, typically with tumor-like swelling, lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, and elevated serum IgG4 concentrations. We report the case of a 22-month-old female child with failure to thrive and recurrent respiratory tract infections since 8 months of age. Physical examination was normal except for pulmonary auscultation with bilateral crackles and wheezes. Laboratory tests revealed elevated erythrocyte sedimentation rate, and elevated serum IgG and IgG4 with polyclonal hypergammaglobulinemia. Thoracic CT and MRI showed multiple mediastinal lymphadenopathies and a nodular posterior mediastinal mass in right paratracheal location with bronchial compression. Initial fine needle aspiration biopsy was compatible with reactive lymphadenopathy but after clinical worsening a thoracoscopic partial resection of the mass was performed and tissue biopsy revealed lymphoplasmacytic infiltrate and increased number of IgG4-positive plasma cells and a ratio of IgG4/IgG positive cells above 40%. Glucocorticoids therapy was started with symptomatic improvement, reduction in the size of the mass, and decrease of serum IgG4 levels after 6 weeks. There are very few reports of IgG4-RD in children. Long-term follow-up is necessary to monitor relapses and additional organ involvement. Hindawi Publishing Corporation 2015 2015-01-29 /pmc/articles/PMC4325228/ /pubmed/25705537 http://dx.doi.org/10.1155/2015/140753 Text en Copyright © 2015 Susana Corujeira et al. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Corujeira, Susana
Ferraz, Catarina
Nunes, Teresa
Fonseca, Elsa
Vaz, Luísa Guedes
Severe IgG4-Related Disease in a Young Child: A Diagnosis Challenge
title Severe IgG4-Related Disease in a Young Child: A Diagnosis Challenge
title_full Severe IgG4-Related Disease in a Young Child: A Diagnosis Challenge
title_fullStr Severe IgG4-Related Disease in a Young Child: A Diagnosis Challenge
title_full_unstemmed Severe IgG4-Related Disease in a Young Child: A Diagnosis Challenge
title_short Severe IgG4-Related Disease in a Young Child: A Diagnosis Challenge
title_sort severe igg4-related disease in a young child: a diagnosis challenge
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4325228/
https://www.ncbi.nlm.nih.gov/pubmed/25705537
http://dx.doi.org/10.1155/2015/140753
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