A case report: an unusual case of ulcerative intestinal IgG4-related disease with clinical features overlapping with probable intestinal Behcet's disease.
Shukai Zhan, Jing Yang, Wenlong Liang et al.
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Case in brief
A 62-year-old man with a history of recurrent oral ulcers presented with sudden hematochezia and hypovolemic shock. Imaging showed sclerosing mesenteritis, and endoscopy revealed multiple small intestinal ulcers. Histopathology confirmed intestinal IgG4-related disease with significant IgG4-positive plasma cell infiltration. The patient also met criteria for probable intestinal Behcet's disease. After partial enterectomy and immunosuppressive therapy, he achieved sustained remission over one year.
What made this case unusual
This case uniquely illustrates the concurrent presentation of intestinal IgG4-related disease and probable intestinal Behcet's disease, highlighting an overlap in clinical features that has not been previously documented.
Diagnostic / clinical pearl
Clinicians should consider the possibility of overlapping immune-mediated conditions, such as IgG4-related disease and Behcet's disease, in patients presenting with gastrointestinal ulcers and systemic symptoms.
Why it matters
Recognizing the overlap between these conditions may enhance diagnostic accuracy and inform management strategies for patients with complex presentations.