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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| Format: | Article |
| Language: | English |
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Wiley
2015-01-01
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| Series: | Case Reports in Pediatrics |
| Online Access: | http://dx.doi.org/10.1155/2015/140753 |
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| author | Susana Corujeira Catarina Ferraz Teresa Nunes Elsa Fonseca Luísa Guedes Vaz |
| author_facet | Susana Corujeira Catarina Ferraz Teresa Nunes Elsa Fonseca Luísa Guedes Vaz |
| author_sort | Susana Corujeira |
| collection | DOAJ |
| 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. |
| format | Article |
| id | doaj-art-38349501ad034c63b45afe2f05a8e9f2 |
| institution | Kabale University |
| issn | 2090-6803 2090-6811 |
| language | English |
| publishDate | 2015-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Pediatrics |
| spelling | doaj-art-38349501ad034c63b45afe2f05a8e9f22025-02-03T06:01:32ZengWileyCase Reports in Pediatrics2090-68032090-68112015-01-01201510.1155/2015/140753140753Severe IgG4-Related Disease in a Young Child: A Diagnosis ChallengeSusana Corujeira0Catarina Ferraz1Teresa Nunes2Elsa Fonseca3Luísa Guedes Vaz4Pediatric Department, Centro Hospitalar São João, Alameda Professor Hernâni Monteiro, 4200-319 Porto, PortugalPediatric Pulmonology Unit, Pediatric Department, Centro Hospitalar São João, Alameda Professor Hernâni Monteiro, 4200-319 Porto, PortugalPediatric Pulmonology Unit, Pediatric Department, Centro Hospitalar São João, Alameda Professor Hernâni Monteiro, 4200-319 Porto, PortugalPathology Department, Centro Hospitalar São João, Alameda Professor Hernâni Monteiro, 4200-319 Porto, Porto, PortugalPediatric Pulmonology Unit, Pediatric Department, Centro Hospitalar São João, Alameda Professor Hernâni Monteiro, 4200-319 Porto, PortugalImmunoglobulin 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.http://dx.doi.org/10.1155/2015/140753 |
| spellingShingle | Susana Corujeira Catarina Ferraz Teresa Nunes Elsa Fonseca Luísa Guedes Vaz Severe IgG4-Related Disease in a Young Child: A Diagnosis Challenge Case Reports in Pediatrics |
| 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 |
| url | http://dx.doi.org/10.1155/2015/140753 |
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