The spectrum of IgG4-related disease in the abdomen and pelvis

• Published in: American 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
• Language: English
• Published: United States 01.07.2013
• Subjects: 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
• ISSN: 0361-803X; 1546-3141
• DOI: 10.2214/ajr.12.9995

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.