The different forms of IgG4-related disease
Immunoglobulin G4-related disease (IgG4-RD) is a newly recognised immune-mediated disease of unknown origin. IgG4-related disease predominantly affects the pancreas (autoimmune pancreatitis type 1), biliary tract (IgG4-associated cholangitis) and salivary glands. However, based on distinct histopathological findings, IgG4-RD has also been described in nearly every organ of the human body. Patient characteristics include elevated IgG4 serum levels, massive infiltration of lymphocytes - in particular IgG4-positive B and plasma cells - in affected tissues and a good response to immunosuppressive treatment. If left untreated, the chronic inflammation associated with IgG4-RD may lead to fibrosis and organ failure. Here, we discuss diagnostic challenges in two patients who were ultimately diagnosed with IgG4-RD. Additionally, we make suggestions on when to consider a possible diagnosis of IgG4-RD and how to approach the patient, thus enabling early diagnosis and treatment.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2018 |
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| Erschienen: |
2018 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:162 |
|---|---|
| Enthalten in: |
Nederlands tijdschrift voor geneeskunde - 162(2018) vom: 16. Aug. |
| Sprache: |
Niederländisch |
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| Weiterer Titel: |
De verschillende vormen van IgG4-gerelateerde ziekte |
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| Beteiligte Personen: |
Roeloffs, Maarten W K [VerfasserIn] |
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| Themen: |
Case Reports |
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| Anmerkungen: |
Date Completed 11.12.2018 Date Revised 11.12.2018 published: Electronic Citation Status MEDLINE |
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| Förderinstitution / Projekttitel: |
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NLM288491378 |
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| 520 | |a Immunoglobulin G4-related disease (IgG4-RD) is a newly recognised immune-mediated disease of unknown origin. IgG4-related disease predominantly affects the pancreas (autoimmune pancreatitis type 1), biliary tract (IgG4-associated cholangitis) and salivary glands. However, based on distinct histopathological findings, IgG4-RD has also been described in nearly every organ of the human body. Patient characteristics include elevated IgG4 serum levels, massive infiltration of lymphocytes - in particular IgG4-positive B and plasma cells - in affected tissues and a good response to immunosuppressive treatment. If left untreated, the chronic inflammation associated with IgG4-RD may lead to fibrosis and organ failure. Here, we discuss diagnostic challenges in two patients who were ultimately diagnosed with IgG4-RD. Additionally, we make suggestions on when to consider a possible diagnosis of IgG4-RD and how to approach the patient, thus enabling early diagnosis and treatment | ||
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