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
Footnotes
Competing interests The authors declare that they have no conflict of interest.
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