A Case Report of an Atypical Presentation of IgG4-Related Disease and Idiopathic CD4 Lymphocytopenia
The IgG4-related disease is a fibroinflammatory disease characterized by tumefactive lesions, a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis, and, often but not always, elevated serum levels of IgG4. Idiopathic CD4 lymphocytopenia is a heterogenic and rar...
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| Format: | Article |
| Language: | English |
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Wiley
2015-01-01
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| Series: | Case Reports in Medicine |
| Online Access: | http://dx.doi.org/10.1155/2015/512370 |
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| author | Francesco Rapisarda Luca Zanoli Grazia Portale Salvo Scuto Pietro Castellino |
| author_facet | Francesco Rapisarda Luca Zanoli Grazia Portale Salvo Scuto Pietro Castellino |
| author_sort | Francesco Rapisarda |
| collection | DOAJ |
| description | The IgG4-related disease is a fibroinflammatory disease characterized by tumefactive lesions, a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis, and, often but not always, elevated serum levels of IgG4. Idiopathic CD4 lymphocytopenia is a heterogenic and rare syndrome characterized by the detection of a persistent absolute CD4 T cells count <300 cells/mm3 (or <20% of total T cells) in more than one occasion and no evidence of HIV infection in absence of immunodeficiency or therapy associated with depressed levels of CD4 T cells. We report the case of a 50-year-old man with a multiorgan IgG4-related disease presenting in a temporal association with a profound and symptomatic idiopathic CD4 lymphocytopenia. Both clinical pictures improved after steroid treatment. Idiopathic CD4 lymphocytopenia has been associated with a number of autoimmune conditions but, to the best of our knowledge, this is the first case in which an association with the IgG4-related disease is reported. |
| format | Article |
| id | doaj-art-7ffe8c21d3114be8bcb6a6993a217d26 |
| institution | Kabale University |
| issn | 1687-9627 1687-9635 |
| language | English |
| publishDate | 2015-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Medicine |
| spelling | doaj-art-7ffe8c21d3114be8bcb6a6993a217d262025-02-03T01:02:58ZengWileyCase Reports in Medicine1687-96271687-96352015-01-01201510.1155/2015/512370512370A Case Report of an Atypical Presentation of IgG4-Related Disease and Idiopathic CD4 LymphocytopeniaFrancesco Rapisarda0Luca Zanoli1Grazia Portale2Salvo Scuto3Pietro Castellino4Section of Nephrology, Department of Internal Medicine, University of Catania, 95100 Catania, ItalySection of Nephrology, Department of Internal Medicine, University of Catania, 95100 Catania, ItalySection of Nephrology, Department of Internal Medicine, University of Catania, 95100 Catania, ItalySection of Nephrology, Department of Internal Medicine, University of Catania, 95100 Catania, ItalySection of Nephrology, Department of Internal Medicine, University of Catania, 95100 Catania, ItalyThe IgG4-related disease is a fibroinflammatory disease characterized by tumefactive lesions, a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis, and, often but not always, elevated serum levels of IgG4. Idiopathic CD4 lymphocytopenia is a heterogenic and rare syndrome characterized by the detection of a persistent absolute CD4 T cells count <300 cells/mm3 (or <20% of total T cells) in more than one occasion and no evidence of HIV infection in absence of immunodeficiency or therapy associated with depressed levels of CD4 T cells. We report the case of a 50-year-old man with a multiorgan IgG4-related disease presenting in a temporal association with a profound and symptomatic idiopathic CD4 lymphocytopenia. Both clinical pictures improved after steroid treatment. Idiopathic CD4 lymphocytopenia has been associated with a number of autoimmune conditions but, to the best of our knowledge, this is the first case in which an association with the IgG4-related disease is reported.http://dx.doi.org/10.1155/2015/512370 |
| spellingShingle | Francesco Rapisarda Luca Zanoli Grazia Portale Salvo Scuto Pietro Castellino A Case Report of an Atypical Presentation of IgG4-Related Disease and Idiopathic CD4 Lymphocytopenia Case Reports in Medicine |
| title | A Case Report of an Atypical Presentation of IgG4-Related Disease and Idiopathic CD4 Lymphocytopenia |
| title_full | A Case Report of an Atypical Presentation of IgG4-Related Disease and Idiopathic CD4 Lymphocytopenia |
| title_fullStr | A Case Report of an Atypical Presentation of IgG4-Related Disease and Idiopathic CD4 Lymphocytopenia |
| title_full_unstemmed | A Case Report of an Atypical Presentation of IgG4-Related Disease and Idiopathic CD4 Lymphocytopenia |
| title_short | A Case Report of an Atypical Presentation of IgG4-Related Disease and Idiopathic CD4 Lymphocytopenia |
| title_sort | case report of an atypical presentation of igg4 related disease and idiopathic cd4 lymphocytopenia |
| url | http://dx.doi.org/10.1155/2015/512370 |
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