Korean J Radiol.  2013 Oct;14(5):776-780. 10.3348/kjr.2013.14.5.776.

An Immunoglobulin G4-Related Sclerosing Disease of the Small Bowel: CT and Small Bowel Series Findings

• Ko Y
• Woo JY
• Kim JW
• Hong HS
• Yang I
• Lee Y
• Hwang D
• Min SJ

• KMID: 1711432
• DOI: http://doi.org/10.3348/kjr.2013.14.5.776

Abstract

Immunoglobulin G4 (IgG4)-related sclerosing disease is rare and is known to involve various organs. We present a case of histologically proven IgG4-related sclerosing disease of the small bowel with imaging findings on computed tomography (CT) and small bowel series. CT showed irregular wall thickening, loss of mural stratification and aneurysmal dilatation of the distal ileum. Small bowel series showed aneurysmal dilatations, interloop adhesion with traction and abrupt angulation.

Keyword

IgG4-related sclerosing disease; Autoimmune pancreatitis; Small bowel; Ileum

MeSH Terms

Adult
Antibodies, Anti-Idiotypic/immunology
Autoimmune Diseases/*diagnosis/immunology
Humans
Immunoglobulin G/*immunology
Intestine, Small/*pathology/radiography
Male
Multidetector Computed Tomography/*methods
Sclerosis/diagnosis/immunology
Antibodies, Anti-Idiotypic
Immunoglobulin G

Figure

• 

  Fig. 1 CT, small bowel series, and histologic findings in 43-year-old man with IgG4-related sclerosing disease of small bowel. Contrast-enhanced axial (A) and coronal-reformatted (B) CT images show irregular thickening, loss of mural stratification and heterogeneous enhancement of distal ileal wall (arrows). Lesion also reveals aneurysmal dilatation. Another coronal-reformatted CT image (C) shows adhesion and aggregation of few small bowel loops (arrow). Another axial CT image (D) reveals multiple enlarged lymph nodes (arrowheads). Small bowel series (E) shows aggregation of distal ileum with traction, angulation and abrupt narrowing which indicates adhesion of ileal loops (arrows). Aneurysmal dilatation of involved segment is also noted (open arrow). On gross specimen (F) obtained from segmental bowel resection, resected ileum shows depressed lesion (4 × 3 cm) with thickened and edematous wall, forming interloop adhesion. On microscopic examination (G-I), ill-demarcated fibrotic lesion involving submucosa, muscle layer and serosa is seen with prominent lymphoplasmacytic infiltrate (G, Hematoxylin & Eosin, × 10). Lymphoplasmacytic infiltrates occasionally show germinal centers (H) and obliterative phlebitis is present (I). Immunohistochemical stain (J) reveals many IgG4-positive plasma cells (more than 50/high power field).

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