Complete remission of central nervous system manifestations of IgG4-related disease with rituximab – a case report
IgG4-related disease (IgG4-RD) is an emerging immune-mediated chronic fibrotic disease characterized by tumour-like mass formation. Reports of brain parenchymal involvement in IgG4-RD are rare and complete treatment-related remission of lesions has never been reported. Here, we present a woman in he...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
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SAGE Publishing
2023-07-01
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| Series: | Therapeutic Advances in Neurological Disorders |
| Online Access: | https://doi.org/10.1177/17562864231186091 |
| _version_ | 1797777469943578624 |
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| author | Aleksi J. Sihvonen Sini M. Laakso Olli Tynninen Heikki Saaren-Seppälä Mervi Löfberg |
| author_facet | Aleksi J. Sihvonen Sini M. Laakso Olli Tynninen Heikki Saaren-Seppälä Mervi Löfberg |
| author_sort | Aleksi J. Sihvonen |
| collection | DOAJ |
| description | IgG4-related disease (IgG4-RD) is an emerging immune-mediated chronic fibrotic disease characterized by tumour-like mass formation. Reports of brain parenchymal involvement in IgG4-RD are rare and complete treatment-related remission of lesions has never been reported. Here, we present a woman in her mid-50s who developed headache and seizures. Brain magnetic resonance imaging revealed frontal bilateral pachymeningitis and a left frontal lobe parenchymal lesion, and pathologic findings were consistent with an IgG4-RD central nervous system manifestation. She had a history of tumour-like growth around the right optic nerve, orbital and maxillary cavities treated successfully with corticosteroids 28 years ago, and was receiving infliximab as a maintenance therapy for uveitis for the last 14 years. After initial high-dose corticosteroid treatment, the patient was treated with rituximab, and after 3 months, the patient presented with complete remission of IgG4-RD lesions and associated symptoms. This case illustrates the chronic, decades-spanning nature of IgG4-RD, and a complete response to rituximab even with intracerebral mass lesions that had emerged despite the use of infliximab, a therapy previously reported successful in IgG4-RD. |
| first_indexed | 2024-03-12T23:05:25Z |
| format | Article |
| id | doaj.art-1f80d3ec2c2d403a8665451b368d90dc |
| institution | Directory Open Access Journal |
| issn | 1756-2864 |
| language | English |
| last_indexed | 2024-03-12T23:05:25Z |
| publishDate | 2023-07-01 |
| publisher | SAGE Publishing |
| record_format | Article |
| series | Therapeutic Advances in Neurological Disorders |
| spelling | doaj.art-1f80d3ec2c2d403a8665451b368d90dc2023-07-19T01:03:37ZengSAGE PublishingTherapeutic Advances in Neurological Disorders1756-28642023-07-011610.1177/17562864231186091Complete remission of central nervous system manifestations of IgG4-related disease with rituximab – a case reportAleksi J. SihvonenSini M. LaaksoOlli TynninenHeikki Saaren-SeppäläMervi LöfbergIgG4-related disease (IgG4-RD) is an emerging immune-mediated chronic fibrotic disease characterized by tumour-like mass formation. Reports of brain parenchymal involvement in IgG4-RD are rare and complete treatment-related remission of lesions has never been reported. Here, we present a woman in her mid-50s who developed headache and seizures. Brain magnetic resonance imaging revealed frontal bilateral pachymeningitis and a left frontal lobe parenchymal lesion, and pathologic findings were consistent with an IgG4-RD central nervous system manifestation. She had a history of tumour-like growth around the right optic nerve, orbital and maxillary cavities treated successfully with corticosteroids 28 years ago, and was receiving infliximab as a maintenance therapy for uveitis for the last 14 years. After initial high-dose corticosteroid treatment, the patient was treated with rituximab, and after 3 months, the patient presented with complete remission of IgG4-RD lesions and associated symptoms. This case illustrates the chronic, decades-spanning nature of IgG4-RD, and a complete response to rituximab even with intracerebral mass lesions that had emerged despite the use of infliximab, a therapy previously reported successful in IgG4-RD.https://doi.org/10.1177/17562864231186091 |
| spellingShingle | Aleksi J. Sihvonen Sini M. Laakso Olli Tynninen Heikki Saaren-Seppälä Mervi Löfberg Complete remission of central nervous system manifestations of IgG4-related disease with rituximab – a case report Therapeutic Advances in Neurological Disorders |
| title | Complete remission of central nervous system manifestations of IgG4-related disease with rituximab – a case report |
| title_full | Complete remission of central nervous system manifestations of IgG4-related disease with rituximab – a case report |
| title_fullStr | Complete remission of central nervous system manifestations of IgG4-related disease with rituximab – a case report |
| title_full_unstemmed | Complete remission of central nervous system manifestations of IgG4-related disease with rituximab – a case report |
| title_short | Complete remission of central nervous system manifestations of IgG4-related disease with rituximab – a case report |
| title_sort | complete remission of central nervous system manifestations of igg4 related disease with rituximab a case report |
| url | https://doi.org/10.1177/17562864231186091 |
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