Fig. 3.1
Algorithm for the diagnosis of intestinal Behçet’s disease (BD) based on the type of ileocolonic ulcerations and clinical manifestations (Adapted from Cheon et al. [4]). §Complete, incomplete, and suspected subtypes of systemic BD were classified according to the diagnostic criteria of the Research Committee of Japan. *Close follow-up is necessary
Fig. 3.2
Microscopic examination from ulcers of the colon showed inflammatory infiltration consisting of lymphocytic or neutrophilic infiltration accompanying vasculitis (H&E stain, ×200)
Similar to inflammatory bowel disease (IBD), including Crohn’s disease and ulcerative colitis, intestinal BD exhibits a fluctuating disease course with repeated episodes of relapse and remission. As a result, the primary goals of intestinal BD management are induction and maintenance of symptom remission to minimize recurrences, surgical procedures, and irreversible bowel damage. Medical treatment of intestinal BD is largely empirical since well-controlled studies have been difficult to perform due to the heterogeneity and rarity of the disease [2]. To date, 5-aminosalicylic acids, systemic corticosteroids, and immunomodulators have been used anecdotally to treat intestinal BD. Regarding prognostic factors of intestinal BD, several clinical variables including younger age, higher disease activity at the time of diagnosis, volcano-type ulcers, absence of mucosal healing, higher C-reactive protein level, history of surgery, and lack of initial response to medical therapy have been repeatedly shown as poor prognosticators in patients with intestinal BD [6].
3.2 Endoscopic Findings
Although the ileocecal area is the most common site (80–95 %) of intestinal BD involvement, it can affect any site of gastrointestinal tract, from oral cavity to anus [3, 5]. The locations of ulcers in intestinal BD are depicted in Fig. 3.3. Esophageal involvement is quite uncommon (less than 5 %), and it usually involves the middle part of the esophagus (Fig. 3.4) [3]. Moreover, the stomach is the least frequently involved part of the gastrointestinal tract (Fig. 3.5). Usually, the number of ulcers is one (60–65 %) or several (Fig. 3.5); however, diffuse involvement can occasionally be present. Although the size of ulcer can vary from small to large size, patients with intestinal BD usually have relatively large ulcers (70–80 % of patient have more than 1 cm-sized ulcers). When the ulcer was small, it appears like an aphthous lesion or small well-demarcated circular or oval-shaped ulcer with normal adjacent mucosa (Fig. 3.6). As the size of ulcer increase, deeply penetrating ulcer which is “typical” to intestinal BD is formed; however, the ulcer shape is maintained with circular or oval shape with discrete, elevated margin, and the ulcer base is covered with whitish, thick exudate (Fig. 3.7). These intestinal BD ulcers can cause clinical complications such as bleeding (Fig. 3.8) or perforation. Meanwhile, irregular-shaped ulcers can present in patients with intestinal BD (Fig. 3.9). As mentioned above, ulcer type has a prognostic implication for patients with intestinal BD, and intestinal BD ulcers showing large, well-demarcated nodular margins, deeply penetrating feature, are known as a “volcano-type” ulcer (Fig. 3.10), and patients having these type ulcers show less favorable response to medical treatment, a more frequent requirement for surgery, and more frequent relapse [6, 7]. Ileocecal valve deformity can be seen due to shrinkage of adjacent mucosa by inflammatory or scaring change (Fig. 3.11). In addition, luminal stricture can be seen in active stage (Fig. 3.12). Various findings of healing of ulcerative lesions after medical therapy are presented in Fig. 3.13. During the disease course of intestinal BD, a substantial number of patients eventually undergo bowel resective surgery due to complications or medically refractory diseases. After surgery, intriguingly, most recurrences occur at the anastomotic site or within the vicinity of the site (Fig. 3.14).
Fig. 3.4
Upper gastrointestinal involvement in intestinal BD. (a) An oval-shaped mid-esophageal ulcer with discrete margin. (b) Several shallow ulcers in the mid-esophagus. (c) The number of esophageal ulcers (figure b) is increased on follow-up endoscopy after 2 years. (d). A well-defined active ulcer in the gastric antrum. The lesion was suspected for gastric involvement of intestinal BD. (e) Although the previous ulcer (figure d) is healed, another active ulcer is noted after 1 year
Fig. 3.5
Multiple ulcers in intestinal BD. (a) Multiple active ulcers with irregular margin in the terminal ileum. (b) Multiple irregular-shaped small ulcers in the terminal ileum. (c) Multiple circular-shaped ulcers with yellowish exudate in the transverse colon. (d) Multiple small ulcers and aphthous lesions in the ileocecal area. (e) Multiple active ulcers accompanying erythematous mucosa in the ileocecal valve. (f) Oval-shaped ulcers in the descending colon
Fig. 3.6
Small ulcers in intestinal BD. (a) Two aphthous lesions in the terminal ileum. (b) A small oval-shaped ulcer in the terminal ileum. (c) A small circular-shaped ulcer in the sigmoid colon. (d) A small ulcer in the terminal ileum. (e) A small irregular-shaped ulcer in the ileocecal valve. (f) A circular-shaped small ulcer in the ileocecal valve. (g) A small oval-shaped ulcer in the terminal ileum. (h) Several circular-shaped ulcers covered with whitish exudate in the transverse colon