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| CASE REPORT |
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| Year : 2023 | Volume
: 69
| Issue : 3 | Page : 176-178 |
Behçet's disease-induced massive small intestinal bleeding successfully treated with adalimumab
PH Liu1, TH Tsai1, KJ Yeo2, JW Chou3
1 Department of Education, China Medical University Hospital, Taichung, Taiwan
2 Division of Rheumatology, Department of Internal Medicine, China Medical University Hospital, Taichung, Taiwan
3 Center for Digestive Medicine, Department of Internal Medicine, China Medical University Hospital, Taichung, Taiwan
| Date of Submission | 01-Dec-2022 |
| Date of Decision | 21-Feb-2023 |
| Date of Acceptance | 27-Feb-2023 |
| Date of Web Publication | 19-Jun-2023 |
Correspondence Address:
Dr. J W Chou
Center for Digestive Medicine, Department of Internal Medicine, China Medical University Hospital, Taichung
Taiwan
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/jpgm.jpgm_931_22
We herein report a case of Behçet's disease in a 27-year-old female who suffered from generalized skin rashes for one week. After hospitalization, massive bloody stools accompanying hypovolemic shock occurred. Emergency abdominal computed tomography-angiography failed to detect the bleeding source. Esophagogastroduodenoscopy also demonstrated no definite bleeding points. Ileocolonoscopy showed multiple large and deep ulcers with some blood coating and mild oozing in the terminal ileum. We initially performed epinephrine injection and hemoclips for her intestinal bleeding. However, massive bloody stools still continued. Thus, we prescribed a loading dose of 160 mg adalimumab followed by weekly 80 mg adalimumab subcutaneous injections to the patient. Following this treatment, her gastrointestinal bleeding gradually subsided and completely stopped within a few days. After three-week therapy with adalimumab, capsule endoscopy showed several healing ulcers without bleeding in the distal to the terminal ileum. She continues to be treated with adalimumab, azathioprine, and mesalazine without recurrent bleeding.
Keywords: Adalimumab, Behçet's disease, capsule endoscopy, small intestinal bleeding
How to cite this article:
Liu P H, Tsai T H, Yeo K J, Chou J W. Behçet's disease-induced massive small intestinal bleeding successfully treated with adalimumab. J Postgrad Med 2023;69:176-8 |
How to cite this URL:
Liu P H, Tsai T H, Yeo K J, Chou J W. Behçet's disease-induced massive small intestinal bleeding successfully treated with adalimumab. J Postgrad Med [serial online] 2023 [cited 2023 Sep 24];69:176-8. Available from: https://www.jpgmonline.com/text.asp?2023/69/3/176/379145 |
| :: Introduction | |  |
Behçet's disease is a disorder consisting of recurrent attacks of acute inflammation and is characterized by recurrent oral and genital ulcers, uveitis, and skin lesions. Major gastrointestinal (GI) bleeding is a rare manifestation of Behçet's disease and usually occurs in those patients with multi-system involvement of vasculitis. GI bleeding with the involvement of the small intestine is less common in Behçet's disease, and it can be life-threatening.[1] Despite a wide range of therapeutic options for the management of Behçet's disease described in the literature, the efficacy of those available therapies for small intestinal lesions is not well established. We herein report a rare presentation of Behçet's disease in a 27-year-old woman who suffered from massive small intestinal bleeding with hypovolemic shock. Finally, her small intestinal bleeding was treated with adalimumab (ADA) successfully.
| :: Case History | | |
A 27-year-old woman with a known history of Behçet's disease was admitted due to skin rashes for one week. The patient was diagnosed with Behçet's disease in 2020, with initially presented as multiple pustule formations in multiple areas of the body, panuveitis, oral and genital ulcers, and erythema nodosum. From 2020 to 2021, her Behçet's disease was treated with oral prednisolone, cyclosporine, and colchicine, and her symptoms were completely resolved. One week before this hospitalization, she suffered skin rashes over her neck, back, chest, and lower abdominal area. She also complained of sore throat, oral ulcer, and perineal ulcer. The intravenous steroid was prescribed to her after hospitalization. Four days after hospitalization, however, the patient had massive bloody stools with hypovolemic shock. Her hemoglobin level declined from 12.0 g/dL to 10.2 g/dL, and her systolic blood pressure dropped to 80 mmHg. Emergency abdominal computed tomography-angiography showed no contrast medium extravasation in the GI tract. She subsequently was transferred to our intensive care unit.
At our intensive care unit, an esophagogastroduodenoscopy was performed and failed to detect the bleeding source. We subsequently performed a colonoscopy, which showed multiple deep and large volcano-shaped ulcers with active bleeding in the terminal ileum [[Figure 1]a, arrow]. Then, we performed epinephrine injection and hemoclips for hemostasis. In addition, endoscopic biopsies from these small bowel ulcers were performed. Behçet's disease with small bowel involvement complicated with massive GI bleeding was diagnosed. Histopathology of biopsy specimens from small bowel ulcers demonstrated ulcers with the presence of vasculitis [[Figure 2], arrows]. However, bloody stools still appeared after endoscopic hemostasis. Thus, we prescribed a loading dose of 160 mg adalimumab (ADA) on week 0 followed by 80 mg ADA on week 2 subcutaneous injections to the patient. Three weeks later, the patient received a capsule endoscopy after a stable hemodynamic status. Capsule endoscopy revealed healing of previous small intestinal ulcers with a hemoclip in the terminal ileum [[Figure 1]b, arrow]. Her symptoms substantially improved after the treatment of ADA. She totally received three doses of induction therapy of ADA. The patient was asymptomatic on subsequent visits. A follow-up colonoscopy performed 6 months after the first infusion of ADA showed marked endoscopic remission of small intestinal ulcers in the terminal ileum [[Figure 1]c, arrow]. | Figure 1: (a) colonoscopy showing several large and deep volcano-shaped ulcers with active bleeding in the terminal ileum (arrow); (b) follow-up capsule endoscopy three weeks later after adalimumab therapy showing healing of previous ulcers with a hemoclip in the terminal ileum (arrow); (c) follow-up colonoscopy 6 months later after discharge showing scarring of previous ulcers in the terminal ileum (arrow)
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 | Figure 2: Histopathological findings of small intestinal ulcers showing some granulation tissue formations and acute and chronic inflammatory cells infiltration in the submucosa, vascular wall, and perivascular space (arrows, H and E staining; original magnification, × 400)
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| :: Discussion | | |
Gastrointestinal manifestations of Behçet's disease are of particular importance as they are associated with morbidity and mortality. GI manifestations often occur 4.5–6 years after the onset of oral ulcers. The most common symptoms include abdominal pain, nausea, vomiting, diarrhea, and GI bleeding. Although ileocecal involvement is most commonly described, Behçet's disease may involve any segment of the GI tract and the various GI organs.[2] The prevalence of GI involvement of Behçet's disease is relatively high in Taiwan (31.8%).[3] It is worth mentioning that symptoms of Behçet's disease in the GI tract are diverse, making it hard to differentiate between intestinal Behçet's disease and Crohn's disease. However, the presence of vasculitis signs such as non-specific inflammation, necrotizing arterioles, and lack of granulomatous lesions of the ulcers helps in the differential diagnosis of Behçet's disease.[4]
Treatment of intestinal Behçet's disease is difficult because there are no internationally accepted or standardized treatment strategies. It has been analyzed that the cumulative rates of surgical intervention are 20% at 1 year, 27–33% at 5 years, and 31–46% 10 years after diagnosis.[5],[6] Nevertheless, the rate of recurrence after a surgical procedure was estimated at 40%–87.5%. In addition, infection and complications are not uncommon in patients with Behçet's disease after surgery.[4] Generally, the same classes of medications that have been used for the treatment of systemic Behçet's disease have also been used to treat intestinal Behçet's disease, including colchicine, 5-aminosalicylic acids (5-ASA), corticosteroids, and immunomodulators such as azathioprine, interferon-α, and anti-TNF-α agents. Otherwise, anti-TNF-α agents, including infliximab (IFX) and ADA, are also beneficial in patients with Behçet's disease who are unresponsive to conventional therapies. There are few randomized trials on the use of anti-TNF-α agents in patients with intestinal Behçet's disease. The first patient with Behçet's disease treated with infliximab was reported in 2001.[7] A Korean retrospective multicenter study showed 28 cases of patients with intestinal Behçet's disease refractory to conventional medical treatment treated with IFX, with a clinical response rate of 64.8% at four weeks.[8] Maintenance of infliximab treatment has shown to be more beneficial than short-term treatment for maintaining remission in patients with intestinal Behçet's disease. A Japanese consensus proposed that in patients with severe symptoms (i.e. abdominal pain, diarrhea, GI bleeding) and complications with deep ulcers confirmed by radiology or endoscopy, corticosteroids should be considered for induction therapy.[9] In patients with mild activity, 5-ASA could be effective for induction therapy for intestinal Behçet's disease. For more severe cases of Behçet's disease, they can be treated with azathioprine. Recently, adalimumab has successfully been used as a first-line anti-TNF-α agent in patients with steroid-dependent intestinal Behçet's disease to induce and maintain complete remission.[10] Although there is proven efficacy of anti-TNF-α agents in intestinal Behçet's disease, further randomized and prospective trials are still necessary to confirm these findings.[9]
| :: Conclusions | | |
This report on Behçet's disease involving a small bowel should serve as a reminder of the possibility of biological treatment and highlight the importance of timely intervention to prevent complicated conditions associated with this disease. Anti-TNF-α agent appears to be promising for those patients who are resistant to conventional therapy. However, further clinical trials are needed to support their use. The identification of which population of patients with Behçet's disease may benefit from biologics is still controversial.
Declaration of patient consent
The authors certify that appropriate patient consent was obtained.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| :: References | | |
| 1. | Sakane T, Takeno M, Suzuki N, Inaba G. Behçet's disease. N Engl J Med 1999;341:1284-91.  |
| 2. | Skef W, Hamilton MJ, Arayssi T. Gastrointestinal Behçet's disease: A review. World J Gastroenterol 2015;21:3801-12. |
| 3. | Chen YC, Chang HW. Clinical characteristics of Behçet's disease in southern Taiwan. J Microbiol Immunol Infect 2001;34:207-10. |
| 4. | Hisamatsu T, Ueno F, Matsumoto T, Kobayashi K, Koganei K, Kunisaki R, et al. The 2 nd edition of consensus statements for the diagnosis and management of intestinal Behçet's disease: Indication of anti-TNFα monoclonal antibodies. J Gastroenterol 2014;49:156-62. |
| 5. | Jung YS, Yoon JY, Hong SP, Kim TI, Kim WH, Cheon JH. Influence of age at diagnosis and sex on clinical course and long-term prognosis of intestinal Behçet's disease. Inflamm Bowel Dis 2012;18:1064-71. |
| 6. | Kim DK, Yang SK, Byeon JS, Myung SJ, Jo JY, Choi KD, et al. Clinical manifestations and course of intestinal Behçet's disease: an analysis in relation to disease subtypes. Intest Res 2005;3:48-54. |
| 7. | Altintaş E, Senli MS, Polat A, Sezgin O. A case of Behçet's disease presenting with massive lower gastrointestinal bleeding. Turk J Gastroenterol 2009;20:57-61. |
| 8. | Hassard PV, Binder SW, Nelson V, Vasiliauskas EA. Anti-tumor necrosis factor monoclonal antibody therapy for gastrointestinal Behçet's disease: A case report. Gastroenterology 2001;120:995-9. |
| 9. | Lee JH, Cheon JH, Jeon SW, Ye BD, Yang SK, Kim YH, et al. Efficacy of infliximab in intestinal Behçet's disease: A Korean multicentre retrospective study. Inflamm Bowel Dis 2013;19:1833-8. |
| 10. | De Cassan C, De Vroey B, Dussault C, Hachulla E, Buche S, Colombel JF. Successful treatment with adalimumab in a familial case of gastrointestinal Behçet's disease. J Crohns Colitis 2011;5:364-8. |
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