New clues to the nature of immunoglobulin G4-related disease: a retrospective Japanese multicenter study of baseline clinical features of 334 cases
Abstract Background The aim was to further characterize immunoglobulin G4-related disease (IgG4-RD) by a large-scale multicenter study of its clinical and laboratory features conducted by multidisciplinary physicians of IgG4-RD in Japan. Methods Various specialists retrospectively evaluated IgG4-RD...
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| Language: | English |
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BMC
2017-12-01
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| Series: | Arthritis Research & Therapy |
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| Online Access: | http://link.springer.com/article/10.1186/s13075-017-1467-x |
| _version_ | 1819141598240309248 |
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| author | Kazunori Yamada Motohisa Yamamoto Takako Saeki Ichiro Mizushima Shoko Matsui Yuhei Fujisawa Satoshi Hara Hiroki Takahashi Hideki Nomura Shigeyuki Kawa Mitsuhiro Kawano |
| author_facet | Kazunori Yamada Motohisa Yamamoto Takako Saeki Ichiro Mizushima Shoko Matsui Yuhei Fujisawa Satoshi Hara Hiroki Takahashi Hideki Nomura Shigeyuki Kawa Mitsuhiro Kawano |
| author_sort | Kazunori Yamada |
| collection | DOAJ |
| description | Abstract Background The aim was to further characterize immunoglobulin G4-related disease (IgG4-RD) by a large-scale multicenter study of its clinical and laboratory features conducted by multidisciplinary physicians of IgG4-RD in Japan. Methods Various specialists retrospectively evaluated IgG4-RD patients diagnosed between 1996 and 2015 in five hospitals by analyzing their baseline clinical features, laboratory, imaging, and pathological test findings, and treatment. Results Of the 334 patients listed, 205 were male and median age at diagnosis was 65 years. The mean number of organs involved was 3.2 at diagnosis. The most frequently affected organs were the salivary glands, followed by the lacrimal glands, lymph nodes, pancreas, retroperitoneum/periaorta, kidneys, and lungs. The mean serum level of IgG4 was 755 mg/dl, and more than 95% of patients had elevated serum IgG4 levels. The median serum level of C-reactive protein (CRP) was 0.1 mg/dl and the level was less than 1 mg/dl in 90% of patients. A total of 34.7% of patients had low serum levels of C3. Serum levels of C3 and non-IgG4 IgG, calculated as the total IgG minus IgG4, showed an inverse correlation in patients with kidney lesions, while serum IgG4 levels were not correlated with serum C3 levels. Corticosteroid was administered in 78.0% of patients, and was effective in all. Conclusions The serum CRP level is generally low and the serum IgG4 level is elevated in most Japanese IgG4-RD patients, in contrast to western patients. These original findings suggest that these two parameters in IgG4-RD differ in some interesting ways from those hitherto reported in western populations. Additional studies, especially international comparative ones, are needed to elucidate the extent and significance of these differences between populations. Attention will also have to be paid to whether the existence of such differences requires consideration when devising international classification criteria. |
| first_indexed | 2024-12-22T11:57:00Z |
| format | Article |
| id | doaj.art-621cb92bb0a948b5b923451f4109a87e |
| institution | Directory Open Access Journal |
| issn | 1478-6362 |
| language | English |
| last_indexed | 2024-12-22T11:57:00Z |
| publishDate | 2017-12-01 |
| publisher | BMC |
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| series | Arthritis Research & Therapy |
| spelling | doaj.art-621cb92bb0a948b5b923451f4109a87e2022-12-21T18:26:43ZengBMCArthritis Research & Therapy1478-63622017-12-0119111010.1186/s13075-017-1467-xNew clues to the nature of immunoglobulin G4-related disease: a retrospective Japanese multicenter study of baseline clinical features of 334 casesKazunori Yamada0Motohisa Yamamoto1Takako Saeki2Ichiro Mizushima3Shoko Matsui4Yuhei Fujisawa5Satoshi Hara6Hiroki Takahashi7Hideki Nomura8Shigeyuki Kawa9Mitsuhiro Kawano10Division of Rheumatology, Kanazawa University HospitalDepartment of Rheumatology and Clinical Immunology, Sapporo Medical University School of MedicineDepartment of Internal Medicine, Nagaoka Red Cross HospitalDivision of Rheumatology, Kanazawa University HospitalHealth Administration Center, University of ToyamaDivision of Rheumatology, Kanazawa University HospitalDivision of Rheumatology, Kanazawa University HospitalDepartment of Rheumatology and Clinical Immunology, Sapporo Medical University School of MedicineHealth Administration Center, University of ToyamaDepartment of Internal Medicine, Matsumoto Dental UniversityDivision of Rheumatology, Kanazawa University HospitalAbstract Background The aim was to further characterize immunoglobulin G4-related disease (IgG4-RD) by a large-scale multicenter study of its clinical and laboratory features conducted by multidisciplinary physicians of IgG4-RD in Japan. Methods Various specialists retrospectively evaluated IgG4-RD patients diagnosed between 1996 and 2015 in five hospitals by analyzing their baseline clinical features, laboratory, imaging, and pathological test findings, and treatment. Results Of the 334 patients listed, 205 were male and median age at diagnosis was 65 years. The mean number of organs involved was 3.2 at diagnosis. The most frequently affected organs were the salivary glands, followed by the lacrimal glands, lymph nodes, pancreas, retroperitoneum/periaorta, kidneys, and lungs. The mean serum level of IgG4 was 755 mg/dl, and more than 95% of patients had elevated serum IgG4 levels. The median serum level of C-reactive protein (CRP) was 0.1 mg/dl and the level was less than 1 mg/dl in 90% of patients. A total of 34.7% of patients had low serum levels of C3. Serum levels of C3 and non-IgG4 IgG, calculated as the total IgG minus IgG4, showed an inverse correlation in patients with kidney lesions, while serum IgG4 levels were not correlated with serum C3 levels. Corticosteroid was administered in 78.0% of patients, and was effective in all. Conclusions The serum CRP level is generally low and the serum IgG4 level is elevated in most Japanese IgG4-RD patients, in contrast to western patients. These original findings suggest that these two parameters in IgG4-RD differ in some interesting ways from those hitherto reported in western populations. Additional studies, especially international comparative ones, are needed to elucidate the extent and significance of these differences between populations. Attention will also have to be paid to whether the existence of such differences requires consideration when devising international classification criteria.http://link.springer.com/article/10.1186/s13075-017-1467-xIgG4-related diseaseClinical featuresHypocomplementemia |
| spellingShingle | Kazunori Yamada Motohisa Yamamoto Takako Saeki Ichiro Mizushima Shoko Matsui Yuhei Fujisawa Satoshi Hara Hiroki Takahashi Hideki Nomura Shigeyuki Kawa Mitsuhiro Kawano New clues to the nature of immunoglobulin G4-related disease: a retrospective Japanese multicenter study of baseline clinical features of 334 cases Arthritis Research & Therapy IgG4-related disease Clinical features Hypocomplementemia |
| title | New clues to the nature of immunoglobulin G4-related disease: a retrospective Japanese multicenter study of baseline clinical features of 334 cases |
| title_full | New clues to the nature of immunoglobulin G4-related disease: a retrospective Japanese multicenter study of baseline clinical features of 334 cases |
| title_fullStr | New clues to the nature of immunoglobulin G4-related disease: a retrospective Japanese multicenter study of baseline clinical features of 334 cases |
| title_full_unstemmed | New clues to the nature of immunoglobulin G4-related disease: a retrospective Japanese multicenter study of baseline clinical features of 334 cases |
| title_short | New clues to the nature of immunoglobulin G4-related disease: a retrospective Japanese multicenter study of baseline clinical features of 334 cases |
| title_sort | new clues to the nature of immunoglobulin g4 related disease a retrospective japanese multicenter study of baseline clinical features of 334 cases |
| topic | IgG4-related disease Clinical features Hypocomplementemia |
| url | http://link.springer.com/article/10.1186/s13075-017-1467-x |
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