Resection of lesions in the ileum of patients with IgG4-related disease may ameliorate disease progression without steroid administration

Abstract

BackgroundIgG4-related disease (IgG4-RD) is a pathological condition that is characterized by an infiltrate composed of IgG4-positive plasma cells and recently recognized as an immune-mediated condition. It causes tissue throughout the body to become stiff and thickened due to autoimmune reactions that cause fibrosis and scarring. Disease-related changes commonly occur in the salivary glands, bile duct, pancreas, and lungs, but are seldom seen in the small bowel. A diagnosis of IgG4-RD is suspected if a high level of IgG4 is found on a blood test. The ideal diagnostic method is pathological examination, but because the clinical manifestations of IgG4-RD are very diverse and nonspecific, the disease may often go undiagnosed until an unrelated biopsy or resection specimen is obtained. The most common treatment for IgG4-RD is steroid use. However, tapering or stopping steroid administration is seen to result in recurrence in approximately 50% of cases. A complete cure is therefore considered extremely difficult.Case presentationA 69-year-old man with gastrointestinal obstruction underwent small bowel resection for two lesions. On histopathological examination, the specimen showed features of IgG4-RD. We performed several tests to detect other characteristics of IgG4-RD, but were unable to find any. The patient is being followed up regularly for a year and is being observed for any symptoms of recurrence.ConclusionsWe present a case of IgG4-RD wherein the ileum wall was significantly sclerosed, leading to gastrointestinal tract obstruction; therefore, we resected two sections of the ileum. We believe that resection of IgG4-RD lesions can help avoid long-term steroid use in patients, because the surgery completely eliminates the pathological origins of the condition.

European Guideline on IgG4‐related digestive disease – UEG and SGF evidence‐based recommendations

“…Comments: Most of the cases reported in the literature describe patients who were operated on for small bowel masses without preoperative testing for IgG4 serum level, and the only indicator for the presence of IgG4‐related disease was postoperative histology. Subsequently, patients received no glucocorticoid or other immune‐suppressive medications and had short follow‐up, if any 100 , 110–114 . IgG4‐ related disease of the gut, in many cases, was diagnosed postoperatively for presumed tumour, and patients did not receive any specific treatment with less than 6 months of follow‐up.…”

“…Subsequently, patients received no glucocorticoid or other immune-suppressive medications and had short follow-up, if any. 100,[110][111][112][113][114] IgG4-related disease of the gut, in many cases, was diagnosed postoperatively for presumed tumour, and patients did not receive any specific treatment with less than 6 months of followup. Most patients were treated with surgical or endoscopic resection, whereas a minority received glucocorticoids and/or immunosuppressants.…”

Immunoglobulin G4-related Disease of the Small Bowel: A Case of Long-term Remission Achieved by Surgical Resection without Maintenance Therapy

“…reported that a surgical resection without steroid maintenance therapy could achieve the long-term remission of IgG4-RD. 8 IgG4-RD has a high relapse rate of approximately 50% after the discontinuation of the steroid, even though steroid is a very effective agent for the treatment of IgG4-RD,…”