Is Adult-Onset Ulcerative Colitis Just Confined to Colon?

Is Adult-Onset Ulcerative Colitis Just Confined to Colon?

Ulcerative colitis (UC) is a chronic inflammatory bowel disease traditionally characterized by its confinement to the large bowel. The disease typically presents with symptoms such as bloody diarrhea, abdominal pain, and weight loss. Historically, the involvement of the upper gastrointestinal (GI) tract has been considered a hallmark of Crohn’s disease (CD) rather than UC. However, emerging evidence suggests that UC can also involve the upper GI tract and small intestine, challenging the conventional understanding of the disease. This article delves into a case study that highlights the possibility of upper GI involvement in adult-onset UC, exploring its clinical implications, diagnostic challenges, and treatment considerations.

Case Presentation

A 24-year-old woman was initially diagnosed with left-sided UC at the age of 22. She achieved disease remission with oral mesalamine but discontinued the medication after one year due to a perceived sense of well-being. Eleven months later, she experienced a recurrence of symptoms, including bloody diarrhea, abdominal pain, nausea, and vomiting. Upon presentation to the emergency department, she was afebrile and not tachycardic, but tenderness was noted in the epigastrium and left lower abdomen.

Laboratory tests revealed elevated inflammatory markers, including a C-reactive protein level of 86 mg/dL, mild anemia with a hemoglobin level of 10.6 g/dL, and hypoalbuminemia with an albumin level of 29.2 g/L. A non-contrast computed tomography (CT) scan unexpectedly showed diffuse gastroduodenitis in addition to continuous colitis. Infectious causes, including Clostridium difficile, Epstein-Barr virus (EBV), and cytomegalovirus (CMV), were ruled out.

The patient was initially treated with full-dose oral and topical mesalamine, intravenous proton pump inhibitors, and empiric antibiotics. However, her nausea and vomiting worsened. Esophagogastroduodenoscopy (EGD) revealed diffuse gastroduodenitis with multiple small fibrin-covered erosions in the stomach and duodenum, which closely resembled the UC lesions observed on colonoscopy. Pathologic examination of both the colon and upper GI tract confirmed the diagnosis of UC, with no evidence of granulomas or infectious agents such as Helicobacter pylori, CMV, or EBV.

Diagnostic Confirmation

The diagnosis of UC was further supported by serologic tests, which were positive for perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) and negative for anti-Saccharomyces cerevisiae antibody. Capsule enteroscopy demonstrated normal ileal and jejunal mucosa, ruling out small bowel involvement. Intravenous methylprednisolone at a dose of 60 mg once daily was initiated, leading to complete resolution of symptoms within three days. A repeat abdominal CT scan after five days of treatment showed significant improvement in gastroduodenal inflammation.

The patient was discharged on a tapering course of oral corticosteroids and maintained on mesalamine. Follow-up endoscopy after a course of steroid therapy showed histologic remission in the colon, with improvement in gastroduodenal inflammation, although a chronic diffuse coarse granular appearance remained. Pathologic re-examination of the gastroduodenum confirmed the presence of UC lesions in remission. The patient remained in remission with mesalamine during the 20-month follow-up period.

Discussion

This case challenges the traditional Montreal classification of adult-onset UC, which does not include upper GI or small-bowel involvement. The patient met the diagnostic criteria for colonic UC, with typical clinical manifestations, endoscopic and radiographic features, and pathologic evidence. The upper GI lesion was diagnosed as UC with upper GI involvement due to its response to steroid therapy and its similarity to colonic lesions in both the active inflammatory and remissive stages. Other common causes of gastroduodenitis, including infectious and drug factors, were excluded.

The involvement of the upper GI tract and small intestine in UC has been reported in previous studies. For instance, diffuse gastroduodenitis associated with UC has been documented, with treatment success using infliximab. Additionally, cases of massive bleeding and perforation with endoscopic multiple deep ulcers in UC patients after colectomy have been reported. These findings suggest that upper GI involvement in UC may be more common than previously thought and could represent a distinct phenotype of the disease.

Clinical Implications

The presence of upper GI involvement in UC raises several clinical questions. Should this condition be classified as a special and rare type of UC, similar to the classification of atypical UC in children and adolescents? The revised Porto classification of UC in children and adolescents defines two categories of UC: typical and atypical, with upper GI/small-bowel involvement classified as atypical UC. This classification system may provide insights into the classification of UC in adults, particularly in cases with upper GI involvement.

Patients with UC and upper GI involvement may be at a higher risk for extraintestinal manifestations and post-operative pouchitis. Therefore, recognizing this phenotype is crucial for appropriate disease management and treatment planning. More aggressive treatment strategies, including the use of biologics, may be necessary for patients with UC and upper GI involvement to achieve and maintain remission.

Conclusion

This case highlights the need to consider upper GI involvement as a differential diagnosis in patients with UC. The traditional classification of UC as a disease confined to the colon may need to be revisited, particularly in light of emerging evidence suggesting the involvement of the upper GI tract and small intestine. Recognizing this phenotype is essential for accurate diagnosis, appropriate treatment, and improved patient outcomes. Further research is needed to better understand the clinical course, prognosis, and optimal management strategies for UC with upper GI involvement.

doi.org/10.1097/CM9.0000000000001384

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