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A new conceptualization for Mikulicz's disease as an IgG4-related plasmacytic disease
Mikulicz's disease (MD) has been included within the diagnosis of primary Sjögren's syndrome (SS), but it represents a unique condition involving persistent enlargement of the lacrimal and salivary glands characterized by few autoimmune reactions and good responsiveness to glucocorticoids,...
Autores principales: | , , , , , , , |
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Formato: | Texto |
Lenguaje: | English |
Publicado: |
Springer-Verlag
2006
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2785894/ https://www.ncbi.nlm.nih.gov/pubmed/17164992 http://dx.doi.org/10.1007/s10165-006-0518-Y |
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author | Yamamoto, Motohisa Takahashi, Hiroki Ohara, Mikiko Suzuki, Chisako Naishiro, Yasuyoshi Yamamoto, Hiroyuki Shinomura, Yasuhisa Imai, Kohzoh |
author_facet | Yamamoto, Motohisa Takahashi, Hiroki Ohara, Mikiko Suzuki, Chisako Naishiro, Yasuyoshi Yamamoto, Hiroyuki Shinomura, Yasuhisa Imai, Kohzoh |
author_sort | Yamamoto, Motohisa |
collection | PubMed |
description | Mikulicz's disease (MD) has been included within the diagnosis of primary Sjögren's syndrome (SS), but it represents a unique condition involving persistent enlargement of the lacrimal and salivary glands characterized by few autoimmune reactions and good responsiveness to glucocorticoids, leading to the recovery of gland function. Mikulicz's disease was recently reported to be associated with elevated immunoglobulin G4 (IgG4) concentrations in the serum and prominent infiltration of plasmacytes expressing IgG4 into the lacrimal and salivary glands. The following features were used for diagnosis: (1) visual confirmation of symmetrical and persistent swelling in more than two lacrimal and major salivary glands; (2) prominent mononuclear cell infiltration of lacrimal and salivary glands; and (3) exclusion of other diseases that present with glandular swelling, such as sarcoidosis and lymphoproliferative disease. These features are not observed in most SS cases. The complications of MD include autoimmune pancreatitis, retroperitoneal fibrosis, tubulointerstitial nephritis, autoimmune hypophysitis, and Riedel's thyroiditis, all of which show IgG4 involvement in their pathogenesis. Mikulicz's disease thus differs from SS and may be a systemic IgG4-related plasmacytic disease. |
format | Text |
id | pubmed-2785894 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2006 |
publisher | Springer-Verlag |
record_format | MEDLINE/PubMed |
spelling | pubmed-27858942009-12-04 A new conceptualization for Mikulicz's disease as an IgG4-related plasmacytic disease Yamamoto, Motohisa Takahashi, Hiroki Ohara, Mikiko Suzuki, Chisako Naishiro, Yasuyoshi Yamamoto, Hiroyuki Shinomura, Yasuhisa Imai, Kohzoh Mod Rheumatol Review Article Mikulicz's disease (MD) has been included within the diagnosis of primary Sjögren's syndrome (SS), but it represents a unique condition involving persistent enlargement of the lacrimal and salivary glands characterized by few autoimmune reactions and good responsiveness to glucocorticoids, leading to the recovery of gland function. Mikulicz's disease was recently reported to be associated with elevated immunoglobulin G4 (IgG4) concentrations in the serum and prominent infiltration of plasmacytes expressing IgG4 into the lacrimal and salivary glands. The following features were used for diagnosis: (1) visual confirmation of symmetrical and persistent swelling in more than two lacrimal and major salivary glands; (2) prominent mononuclear cell infiltration of lacrimal and salivary glands; and (3) exclusion of other diseases that present with glandular swelling, such as sarcoidosis and lymphoproliferative disease. These features are not observed in most SS cases. The complications of MD include autoimmune pancreatitis, retroperitoneal fibrosis, tubulointerstitial nephritis, autoimmune hypophysitis, and Riedel's thyroiditis, all of which show IgG4 involvement in their pathogenesis. Mikulicz's disease thus differs from SS and may be a systemic IgG4-related plasmacytic disease. Springer-Verlag 2006-12-20 2006-12 /pmc/articles/PMC2785894/ /pubmed/17164992 http://dx.doi.org/10.1007/s10165-006-0518-Y Text en © Japan College of Rheumatology 2006 |
spellingShingle | Review Article Yamamoto, Motohisa Takahashi, Hiroki Ohara, Mikiko Suzuki, Chisako Naishiro, Yasuyoshi Yamamoto, Hiroyuki Shinomura, Yasuhisa Imai, Kohzoh A new conceptualization for Mikulicz's disease as an IgG4-related plasmacytic disease |
title | A new conceptualization for Mikulicz's disease as an IgG4-related plasmacytic disease |
title_full | A new conceptualization for Mikulicz's disease as an IgG4-related plasmacytic disease |
title_fullStr | A new conceptualization for Mikulicz's disease as an IgG4-related plasmacytic disease |
title_full_unstemmed | A new conceptualization for Mikulicz's disease as an IgG4-related plasmacytic disease |
title_short | A new conceptualization for Mikulicz's disease as an IgG4-related plasmacytic disease |
title_sort | new conceptualization for mikulicz's disease as an igg4-related plasmacytic disease |
topic | Review Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2785894/ https://www.ncbi.nlm.nih.gov/pubmed/17164992 http://dx.doi.org/10.1007/s10165-006-0518-Y |
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