The Spectrum of IgG4-Related Disease in the Abdomen and Pelvis

IgG4-related disease was not recognized as a specific clinical entity until 2003 when extrapancreatic lesions were reported in patients with autoimmune pancreatitis. IgG4-related disease is characterized by elevated serum IgG4 levels and infiltration of the target organ by IgG4-positive plasma cells...

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Published inAmerican journal of roentgenology (1976) Vol. 201; no. 1; pp. 14 - 22
Main Authors Hedgire, Sandeep S., McDermott, Shaunagh, Borczuk, David, Elmi, Azadeh, Saini, Sanjay, Harisinghani, Mukesh G.
Format Journal Article
LanguageEnglish
Published United States 01.07.2013
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Online AccessGet full text
ISSN0361-803X
1546-3141
1546-3141
DOI10.2214/AJR.12.9995

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Abstract IgG4-related disease was not recognized as a specific clinical entity until 2003 when extrapancreatic lesions were reported in patients with autoimmune pancreatitis. IgG4-related disease is characterized by elevated serum IgG4 levels and infiltration of the target organ by IgG4-positive plasma cells. The complete gamut of visceral involvement is still being outlined. The purpose of this article is to highlight the plethora of lesions under the spectrum of IgG4-related disease of the abdomen and pelvis, describe their imaging appearances on multimodality cross-sectional imaging, and discuss the differential diagnoses. It is important for radiologists to recognize the multiorgan involvement and few classic features of IgG4-related disease that often tend to simulate malignancy.
AbstractList IgG4-related disease was not recognized as a specific clinical entity until 2003 when extrapancreatic lesions were reported in patients with autoimmune pancreatitis. IgG4-related disease is characterized by elevated serum IgG4 levels and infiltration of the target organ by IgG4-positive plasma cells. The complete gamut of visceral involvement is still being outlined. The purpose of this article is to highlight the plethora of lesions under the spectrum of IgG4-related disease of the abdomen and pelvis, describe their imaging appearances on multimodality cross-sectional imaging, and discuss the differential diagnoses.OBJECTIVEIgG4-related disease was not recognized as a specific clinical entity until 2003 when extrapancreatic lesions were reported in patients with autoimmune pancreatitis. IgG4-related disease is characterized by elevated serum IgG4 levels and infiltration of the target organ by IgG4-positive plasma cells. The complete gamut of visceral involvement is still being outlined. The purpose of this article is to highlight the plethora of lesions under the spectrum of IgG4-related disease of the abdomen and pelvis, describe their imaging appearances on multimodality cross-sectional imaging, and discuss the differential diagnoses.It is important for radiologists to recognize the multiorgan involvement and few classic features of IgG4-related disease that often tend to simulate malignancy.CONCLUSIONIt is important for radiologists to recognize the multiorgan involvement and few classic features of IgG4-related disease that often tend to simulate malignancy.
IgG4-related disease was not recognized as a specific clinical entity until 2003 when extrapancreatic lesions were reported in patients with autoimmune pancreatitis. IgG4-related disease is characterized by elevated serum IgG4 levels and infiltration of the target organ by IgG4-positive plasma cells. The complete gamut of visceral involvement is still being outlined. The purpose of this article is to highlight the plethora of lesions under the spectrum of IgG4-related disease of the abdomen and pelvis, describe their imaging appearances on multimodality cross-sectional imaging, and discuss the differential diagnoses. It is important for radiologists to recognize the multiorgan involvement and few classic features of IgG4-related disease that often tend to simulate malignancy.
Author Elmi, Azadeh
McDermott, Shaunagh
Saini, Sanjay
Harisinghani, Mukesh G.
Borczuk, David
Hedgire, Sandeep S.
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  organization: All authors: Department of Abdominal Imaging and Intervention, Massachusetts General Hospital, 55 Fruit St, White 270, Boston, MA 02114
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SubjectTerms Autoimmune Diseases - blood
Autoimmune Diseases - diagnosis
Autoimmune Diseases - immunology
Diagnostic Imaging
Digestive System Diseases - blood
Digestive System Diseases - diagnosis
Digestive System Diseases - immunology
Humans
Immunoglobulin G - blood
Immunoglobulin G - immunology
Lymphatic Diseases - blood
Lymphatic Diseases - diagnosis
Lymphatic Diseases - immunology
Male
Male Urogenital Diseases - blood
Male Urogenital Diseases - diagnosis
Male Urogenital Diseases - immunology
Title The Spectrum of IgG4-Related Disease in the Abdomen and Pelvis
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