An Immunoglobulin G4-Related Sclerosing Disease of the Small Bowel: CT and Small Bowel Series Findings
A 43-year-old man was admitted to our hospital with a one-year history of intermittent abdominal pains. He had a past medical history of acute myocardial infarction 10 years ago and a six-month history of benign prostatic hyperplasia. The physical examination revealed tenderness in the lower right quadrant of the abdomen.\nThe laboratory tests at presentation showed mild leukocytosis (10470/uL) with an elevated neutrophil percentage (79.6%) and an elevated C-reactive protein value (50.6 mg/L). The serum eosinophil count was in normal range (1.8%). The serum IgG4 level was not available in this case. A plain abdominal radiograph demonstrated no remarkable findings (not shown).\nContrast-enhanced abdominal CT with a 64-row MDCT (Sensation 64, Siemens Medical Solutions, Malvern, PA, USA) showed irregular wall thickening, losses of mural stratification and aneurysmal dilatation of the distal ileum (). Furthermore, adhesion and aggregation of distal ileal loops were also seen (). There were multiple enlarged lymph nodes (maximal diameter: 1.5 cm) around the distal ileum ().\nSmall bowel series showed similar findings to those from the CT. Aneurysmal dilatation and multifocal narrowing of the distal ileum were also seen. Adhesion of the bowel loops with traction and abrupt angulation were also revealed (). There were no significant abnormal findings in the other GI tracts. Clinically and radiologically, there were no significant abnormal findings in other organs.\nThe clinical and radiologic findings were somewhat confusing and non-specific because their sum can suggest both malignancy and benign inflammatory diseases, and are not specified to one category. The patient underwent exploratory laparoscopy. At surgery, the distal ileum showed segmental wall thickening and edematous wall with interloop adhesions. A segment of the distal ileum was resected. Regional lymph nodes dissection and appendectomy were also performed.\nPathological study confirmed the diagnosis of IgG4-related sclerosing disease, which showed intramural chronic inflammation with prominent IgG4 rich-lymphoplasmacytic infiltrates [IgG4 positive cells > 50/high power field (HPF)], fibrosis, and obliterative phlebitis ().\nThe patient's post-operative course was uneventful. The patient was discharged on day 9 in good condition with regular follow-ups in the surgical outpatient clinic.