A case with Sjogren syndrome presenting the perforation of small intestine caused by localized diffuse large B cell lymphoma

Abstract

    A 78 year-old woman was admitted into our hospital due to recurrent abdominal pain. She was diagnosed with Sjögren syndrome when she was 66 years old. Dietary restriction transiently improved her abdominal pain. However, with the resumption of diet, her abdominal pain was exacerbated again. The recurrent abdominal pain was resistant to gastrointestinal medicines. The elevation of soluble interleukin-2 receptor was recognized but not significant. While ⁶⁷Gascintigraphy showed positive accumulation in the right lower quadrant of the abdomen, the diagnosis could not be defined through either colonoscopy or capsule endoscopy. During these examinations, she presented the symptom of acute abdomen. An emergency operation revealed that the acute abdominal symptom was caused by the perforation of the small intestine pathologically diagnosed as a localized diffuse large B cell lymphoma. Sjögren syndrome often combines malignant lymphomas. However, the incidence of primary lymphoma from small intestine is rare. Furthermore, in this case, the titer of soluble interleukin-2 receptor, ⁶⁷Gascintigraphy and capsule endoscopy were not useful for the diagnosis or identification of the lymphoma. These results might suggest that screening tests including exploratory laparotomy for malignancy in the gastrointestinal tract should be intensively performed if patients with Sjögren syndrome have unexplained complaints in the abdomen.