Persistent Lymphadenopathy due to IgG4-Related Disease

A 28-year-old healthy female presented to her primary care physician with lymphadenopathy, fatigue, malaise, and night sweats. Symptoms persisted despite conservative treatment and eventually the patient underwent multiple lymph node resections and a bone marrow biopsy before a diagnosis of IgG4-rel...

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Main Authors: Benjamin Smith, Matthew B. Carroll
Format: Article
Language:English
Published: Wiley 2012-01-01
Series:Case Reports in Immunology
Online Access:http://dx.doi.org/10.1155/2012/158208
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author Benjamin Smith
Matthew B. Carroll
author_facet Benjamin Smith
Matthew B. Carroll
author_sort Benjamin Smith
collection DOAJ
description A 28-year-old healthy female presented to her primary care physician with lymphadenopathy, fatigue, malaise, and night sweats. Symptoms persisted despite conservative treatment and eventually the patient underwent multiple lymph node resections and a bone marrow biopsy before a diagnosis of IgG4-related disease (IgG4-RD) was made. IgG4-RD is a relatively new disorder first histopathologically recognized within the last decade. As the disease can affect a single organ or multiple organs, symptoms can vary greatly among patients. With symptoms ranging from mild, such as lower extremity edema, to severe, such as spinal cord compression, IgG4-RD must be considered in appropriate patients. Diagnostic criteria have been proposed based on organ involvement, serum IgG4 levels, and histopathological criteria. Diagnosis can be difficult to make with many studies suggesting different values for diagnostic criteria, such as the level of tissue IgG4+/IgG+ cell ratio to delineate IgG4-RD. Treatment consists of high dose glucocorticoids as a first line therapy with some patients choosing instead to simply undergo observation. This case illustrates the difficulty in diagnosis and the need for increased awareness among medical professionals.
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spelling doaj-art-fb25b3659d874091963fb3ad639b72242025-02-03T01:11:10ZengWileyCase Reports in Immunology2090-66092090-66172012-01-01201210.1155/2012/158208158208Persistent Lymphadenopathy due to IgG4-Related DiseaseBenjamin Smith0Matthew B. Carroll181st Medical Group Hospital, 301 Fisher Street, Keesler Air Force Base, MS 39534, USA81st Medical Group Hospital, 301 Fisher Street, Keesler Air Force Base, MS 39534, USAA 28-year-old healthy female presented to her primary care physician with lymphadenopathy, fatigue, malaise, and night sweats. Symptoms persisted despite conservative treatment and eventually the patient underwent multiple lymph node resections and a bone marrow biopsy before a diagnosis of IgG4-related disease (IgG4-RD) was made. IgG4-RD is a relatively new disorder first histopathologically recognized within the last decade. As the disease can affect a single organ or multiple organs, symptoms can vary greatly among patients. With symptoms ranging from mild, such as lower extremity edema, to severe, such as spinal cord compression, IgG4-RD must be considered in appropriate patients. Diagnostic criteria have been proposed based on organ involvement, serum IgG4 levels, and histopathological criteria. Diagnosis can be difficult to make with many studies suggesting different values for diagnostic criteria, such as the level of tissue IgG4+/IgG+ cell ratio to delineate IgG4-RD. Treatment consists of high dose glucocorticoids as a first line therapy with some patients choosing instead to simply undergo observation. This case illustrates the difficulty in diagnosis and the need for increased awareness among medical professionals.http://dx.doi.org/10.1155/2012/158208
spellingShingle Benjamin Smith
Matthew B. Carroll
Persistent Lymphadenopathy due to IgG4-Related Disease
Case Reports in Immunology
title Persistent Lymphadenopathy due to IgG4-Related Disease
title_full Persistent Lymphadenopathy due to IgG4-Related Disease
title_fullStr Persistent Lymphadenopathy due to IgG4-Related Disease
title_full_unstemmed Persistent Lymphadenopathy due to IgG4-Related Disease
title_short Persistent Lymphadenopathy due to IgG4-Related Disease
title_sort persistent lymphadenopathy due to igg4 related disease
url http://dx.doi.org/10.1155/2012/158208
work_keys_str_mv AT benjaminsmith persistentlymphadenopathyduetoigg4relateddisease
AT matthewbcarroll persistentlymphadenopathyduetoigg4relateddisease