Multifocal ulcerating stenosing enteritis as a novel manifestation of immunoglobulin G4-related disease

Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a rare systemic fibroinflammatory disease characterized by the presence of tumefactive lesions with dense infiltration of IgG4-positive plasma cells and sometimes serum elevated IgG4 [1]. Seventy-five percent of patients have two or more organs affected, with frequent involvement of the pancreas and the bile ducts [2]. Small-bowel involvement has rarely been reported, with only a few case reports in the literature [3]. The presence of IgG4-bearing plasma cells is essential for its diagnosis; an additional histological characteristic is eosinophil infiltration [1] [2]. There is uncertainty regarding its clinical presentation, diagnostic criteria, and treatment. Management with glucocorticoids may be an appropriate option, as well as, in some cases, immunosuppressive maintenance treatment [4].

Herein we present the case of a 28-year-old woman with a history of iron deficiency anemia with no gynecological causes, and recurrent episodes of abdominal pain and bloating. Upper gastrointestinal endoscopy and colonoscopy showed no significant findings. A video capsule endoscopy was performed and revealed congestive mucosa with ulcers, scars, and zones of stenosis at the terminal ileum ([Video 1]). A retrograde double-balloon enteroscopy was performed and demonstrated multiple areas of concentric irregular ulcers with secondary stenosis and scars ([Video 1]). Hydropneumatic dilation was performed without complications ([Video 1]).

Video 1 Multifocal ulcerating stenosing enteritis as a novel manifestation of immunoglobulin G4-related disease.

The pathology report was consistent with IgG4-associated multifocal ulcerating stenosing enteritis ([Fig. 1 a–e]). Positron emission tomography-computed tomography scan showed no extraintestinal IgG4-RD involvement. Systemic corticosteroid therapy was started, and long-term follow-up will be given.

Fig. 1 Pathology results. a Low-power view of an ileal ulcer with fissure and fibrosis. b Reactive vascular proliferation and mixed moderate inflammatory infiltrate in the bed of the ulcer. c Abundant plasma cells found in the fibrotic areas and between smooth muscle bundles. d Immunoglobulin (Ig) G4-positive immunostaining (> 50 plasma cells in a high-power field). The IgG/IgG4 ratio was 0.45. e IgG-positive plasma cells.

In conclusion, we present a rare case of a patient with isolated bowel IgG4-RD, who presented with occult intestinal bleeding and stenosis, and was managed with hydropneumatic dilation and systemic steroid, with a satisfactory outcome at the time of writing this report. Long-term follow-up of these patients is required, as further lesions may appear as late as years after initial manifestation and could be located in distinct organs [4].

Endoscopy_UCTN_Code_CCL_1AC_2AD

• References

• 1 Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med 2012; 366: 539-551
• 2 Löhr JM, Vujasinovic M, Rosendahl J. et al. IgG4-related diseases of the digestive tract. Nat Rev Gastroenterol Hepatol 2022; 19: 185-197
• 3 Ko Y, Woo JY, Kim JW. et al. An immunoglobulin G4-related sclerosing disease of the small bowel: CT and small bowel series findings. Korean J Radiol 2013; 14: 776-780
• 4 Löhr JM, Beuers U, Vujasinovic M. et al. European Guideline on IgG4-related digestive disease – UEG and SGF evidence-based recommendations. United European Gastroenterol J 2020; 8: 637-666