Hyper IgG4-Related Disease Presenting with Orbital Tumor and Immune Deficiency
We report a case of IgG4-RD in a patient with high IgG4 levels, low functional antibodies, and low IgM levels. He presented with bilateral orbital pseudotumors and, after initial improvement on corticosteroids, relapsed with recurrent pleural effusion and pelvic pseudotumor. He had a grossly elevate...
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| Format: | Article |
| Language: | English |
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Wiley
2021-01-01
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| Series: | Case Reports in Immunology |
| Online Access: | http://dx.doi.org/10.1155/2021/9260051 |
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| author | Caroline G. Olson Nancy Y. Olson |
| author_facet | Caroline G. Olson Nancy Y. Olson |
| author_sort | Caroline G. Olson |
| collection | DOAJ |
| description | We report a case of IgG4-RD in a patient with high IgG4 levels, low functional antibodies, and low IgM levels. He presented with bilateral orbital pseudotumors and, after initial improvement on corticosteroids, relapsed with recurrent pleural effusion and pelvic pseudotumor. He had a grossly elevated serum IgG (1905 mg/dl) with elevations in all IgG subclasses but marked elevation in IgG4 (412 mg/dl), low IgM, and low pneumococcal antibodies. Orbital mass biopsy showed polyclonal lymphocytic infiltration and increased IgG4 plasma cells. The patient was started on prednisone and tried several immunosuppressive medications including mycophenolate mofetil, methotrexate, hydroxychloroquine, and azathioprine with decrease in size of the orbital pseudotumor. During a period when the patient stopped his medications, the pseudotumor enlarged with new development of recurrent pleural effusions. He was also found to have a pelvic mass that was biopsy positive for IgG4 proliferation. This case with progression to multiorgan involvement highlights the importance of identifying patients with IgG4-related disease. In contrast to previous cases with normal-to-high IgM, the IgM was low with impaired functional antibodies. |
| format | Article |
| id | doaj-art-42bdcfdbf19a4f70bafb5a64b055282e |
| institution | Kabale University |
| issn | 2090-6609 2090-6617 |
| language | English |
| publishDate | 2021-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Immunology |
| spelling | doaj-art-42bdcfdbf19a4f70bafb5a64b055282e2025-02-03T07:23:59ZengWileyCase Reports in Immunology2090-66092090-66172021-01-01202110.1155/2021/92600519260051Hyper IgG4-Related Disease Presenting with Orbital Tumor and Immune DeficiencyCaroline G. Olson0Nancy Y. Olson1University of Missouri-Kansas City, School of Medicine, Kansas City, MO, USAAllergy and Rheumatology Clinic of Kansas City, Overland Park, KS, USAWe report a case of IgG4-RD in a patient with high IgG4 levels, low functional antibodies, and low IgM levels. He presented with bilateral orbital pseudotumors and, after initial improvement on corticosteroids, relapsed with recurrent pleural effusion and pelvic pseudotumor. He had a grossly elevated serum IgG (1905 mg/dl) with elevations in all IgG subclasses but marked elevation in IgG4 (412 mg/dl), low IgM, and low pneumococcal antibodies. Orbital mass biopsy showed polyclonal lymphocytic infiltration and increased IgG4 plasma cells. The patient was started on prednisone and tried several immunosuppressive medications including mycophenolate mofetil, methotrexate, hydroxychloroquine, and azathioprine with decrease in size of the orbital pseudotumor. During a period when the patient stopped his medications, the pseudotumor enlarged with new development of recurrent pleural effusions. He was also found to have a pelvic mass that was biopsy positive for IgG4 proliferation. This case with progression to multiorgan involvement highlights the importance of identifying patients with IgG4-related disease. In contrast to previous cases with normal-to-high IgM, the IgM was low with impaired functional antibodies.http://dx.doi.org/10.1155/2021/9260051 |
| spellingShingle | Caroline G. Olson Nancy Y. Olson Hyper IgG4-Related Disease Presenting with Orbital Tumor and Immune Deficiency Case Reports in Immunology |
| title | Hyper IgG4-Related Disease Presenting with Orbital Tumor and Immune Deficiency |
| title_full | Hyper IgG4-Related Disease Presenting with Orbital Tumor and Immune Deficiency |
| title_fullStr | Hyper IgG4-Related Disease Presenting with Orbital Tumor and Immune Deficiency |
| title_full_unstemmed | Hyper IgG4-Related Disease Presenting with Orbital Tumor and Immune Deficiency |
| title_short | Hyper IgG4-Related Disease Presenting with Orbital Tumor and Immune Deficiency |
| title_sort | hyper igg4 related disease presenting with orbital tumor and immune deficiency |
| url | http://dx.doi.org/10.1155/2021/9260051 |
| work_keys_str_mv | AT carolinegolson hyperigg4relateddiseasepresentingwithorbitaltumorandimmunedeficiency AT nancyyolson hyperigg4relateddiseasepresentingwithorbitaltumorandimmunedeficiency |