A 30-year-old female with a fever, scleritis, and auricular pain for a duration of 3 months was examined. She was finally diagnosed with RP from her symptoms, auricle biopsy and response to corticosteroid therapy according to the proposed diagnostic criteria (McAdam et al.; Damiani and Levine). She frequently took non-steroidal anti-inflammatory drugs (NSAIDs) along with predonisolone for pain control. Subsequently, she experienced episodes of lower abdominal pain 2 months after the initial diagnosis of RP. Although her abdominal symptoms temporally subsided with steroid pulse therapy, the abdominal pain relapsed and the bloody stools as well as anemia emerged after terminating steroid pulse therapy. As a result, we started to investigate her abdomen and GI tract. Laboratory tests revealed an increased inflammatory response (white blood cells 10,300/mul; C-reactive protein, 5.4 mg/dl), hypoalbuminemia (total protein, 5.9 g/dl, albumin, 2.9 g/dl), and anemia (red blood cells, 3.20 x 106/mul, hemoglobin, 8.7 g/dl, hematocrit, 27.2%) (Table 1). Abdominal contrast-enhanced computed tomography (CT) scan revealed thickening of the ileocecal wall, and positron emission tomogaraphy (PET) revealed accumulation in the intestinal tract at the terminal ileum. Total colonoscopy (TCS) revealed oval-shaped deep ulcers on the terminal ileum with deformity and destruction of the ileocecal valve (Figure 1). In addition, reddish edematous mucosa and ulcers were detected in the oral side of the ileum. Biopsy specimens taken from the terminal ileum showed nonspecific inflammatory findings. There were no abnormal findings in her large intestinal mucosa and upper GI tracts. We highly suspected the possibility of intestinal BD from the characteristic endoscopic findings, but the case did not meet the diagnostic criteria because of lacking the major BD symptoms such as oral aphthoid ulcers, eye symptoms, skin lesions or genital ulcers. In addition, other diseases such as NSAIDs-induced enteritis were not completely denied; therefore, we selected a conservative therapy and planned a close follow-up. After restricting food and NSAIDs from the initial TCS, slight improvements were observed in her abdominal symptoms. However, no significant change was evident on colonoscopy performed 4 weeks after the initial TCS. Differential diagnoses included intestinal BD, Crohn's disease, NSAIDs-induced enteritis, malignant lymphoma, and infectious colitis such as cytomegalovirus colitis or enteric tuberculosis. However, the test results, including a biopsy, culture, and various imaging tests did not provide a definitive diagnosis. We decided to perform surgery in order to make a definite diagnosis and avoid the GI perforation because of the presence of deep and active ulcers. An ileocecal resection was performed 5 weeks after the initial TCS. Gross findings of the resected specimen showed that the ileocecal valve was highly deformed and destroyed by deep ulcers. Furthermore, large and small ulcers were diffusely-scattered on the ileal mucosa, from the ileocecal valve to 30 cm on the oral side (Figure 2). Histological examination revealed ulcer formation with destruction of muscularis propria. The ulcer base was rather flat and showing involvement of nonspecific chronic inflammation with fibrotic change. The mucosa around the ulcers remained relatively normal in structure (Figure 3). There were no specific findings such as granulomas, CMV-infected cells, vasculitis or thrombus formation; thus, we remained to believe intestinal BD as the diagnosis.
After the operation, she was in stable condition. We followed her progress on an outpatient basis while tapering her prednisolone dosage. However, RP symptoms such as fever, scleritis, and auricular pain relapsed 5 months after the operation, in addition to the recurrence of abdominal pain and watery diarrhea. This led us to perform TCS which revealed multiple deep and round ulcers developing at the anastomotic site. We also observed multiple erosions and aphthae on the oral side of the small intestine and large bowel from the ascending colon to the rectum (Figure 4). We initiated infliximab (IFX) administration, which has been reported to be effective against both RP and BD. After the administration of IFX, her cartilaginous and abdominal symptoms dramatically improved. A follow-up TCS was performed at the end of the fourth administration of IFX. We identified ulcer scars at the anastomosis site and inflammation of the large intestine improved (Figure 5). We recognized that both RP and BD were responsive to IFX administration. Currently as an outpatient, she is regularly receiving IFX treatment and continues to remain in good health.