目的

探讨儿童白塞病合并肠道假性息肉的临床特点及诊疗策略。

方法

选择2017年1月至2024年1月于首都儿科研究所附属儿童医院、河北医科大学第二医院收治的2例白塞病合并肠道假性息肉患儿（患儿1、2）为研究对象。采用回顾性分析方法，对2例白塞病患儿临床病例资料进行分析，并检索国内外数据库中白塞病合并肠道假性息肉的相关研究文献进行文献复习。本研究经河北医科大学第二医院伦理委员会批准（审批文号：2023-R114），监护人对患儿的诊治均知情同意，并签署临床研究知情同意书。

结果

①患儿1： 女性，6岁，主要临床表现为反复口腔溃疡、间断发热及眼部溃疡，伴结节性红斑，胃镜检查发现咽部多发溃疡，结肠镜显示回肠溃疡，入院诊断为白塞病，给予患儿1甲泼尼龙、沙利度胺及柳氮磺吡啶口服治疗。在病情好转后，甲泼尼龙规律减量过程中，患儿1病情多次反复，先后应用多种免疫抑制剂治疗。于首次入院3.5年后，对其复查结肠镜可见回盲部及升结肠多发炎性息肉。②患儿2：男性，6岁，主要临床表现为间断发热、腹痛及消化道穿孔。对其进行肠切除肠吻合术后，采取激素治疗措施。之后患儿2反复口腔溃疡、皮肤红斑，肠镜显示溃疡性病变，确诊为白塞病。对其采取激素、注射用重组人Ⅱ型肿瘤坏死因子受体-抗体融合蛋白（rh TNFR：Fc）、环孢素治疗措施，规律随访过程中病情多次反复。于首次入院5.3年后，对患儿2复查肠镜可见回盲部溃疡性、增生性隆起，炎性息肉形成。③文献复习结果：在Pub Med数据库中检索到关于1例儿童（患儿3）白塞病合并肛周息肉的研究文献。患儿3为男性，12岁，主要临床表现为口腔溃疡、肛周疼痛及阴茎溃疡，肛周见溃疡及息肉样病变，肛周局部组织病理学活检结果为非特异性炎症，全身激素治疗后肛周溃疡愈合。

结论

严重炎症反复刺激，是导致白塞病患儿合并肠道假性息肉的主要原因，对该类患儿临床治疗要点是控制白塞病患儿病情反复，加强随访。

Objective

To explore the clinical characteristics，and diagnosis and treatment strategy of pediatric Behcet's disease complicated with intestinal pseudopolyps.

Methods

Two children （patient 1，2）with Behcet's disease complicated with intestinal pseudopolyps admitted to the Children's Hospital Affiliated to Capital Institute of Pediatrics and the Second Hospital of Hebei Medical University from January 2017 to January 2024 were selected as the study subjects.Retrospective analysis method was used to analyze the clinical data of 2 children with Behcet's disease.And search for relevant literature on Behcet's disease combined with intestinal pseudopolyps in domestic and foreign databases for literature review.This study was approved by the Ethics Committee of the Second Hospital of Hebei Medical University （Approval No.2023-R114），and the guardians informed and agreed to the diagnosis and treatment of the patients，and signed a clinical study informed consent form.

Results

①Patient 1：a 6-year-old female with recurrent oral ulcers，intermittent fever，and eye ulcers，accompanied by nodular erythema.Multiple ulcers were found in the pharynx during gastroscopy，and ileal ulcers during colonoscopy.Therefore，the diagnosis of Behcet's disease was made，and patient 1 was given oral methylprednisolone，thalidomide，and sulfasalazine.After getting better，during the regular reduction of methylprednisolone，the condition of illness recurred multiple times and multiple immunosuppressive agents were used for treatment.After the first admission for 3.5 years，a follow-up colonoscopy revealed multiple inflammatory polyps in the ileocecal and ascending colon.②Patient 2：a 6-years-old male with main clinical manifestations of intermittent fever，abdominal pain，and gastrointestinal perforation.After undergoing intestinal resection and anastomosis，glucocorticoids therapy was administered.Afterwards，patient 2 had recurrent oral ulcers and skin erythema，and colonoscopy showed ulcerative lesions，then confirming the diagnosis of Behcet's disease.Administer glucocorticoids，recombinant human tumor necrosis factor-αreceptorⅡ：IgG Fc fusion protein for injection（rh TNFR：Fc），and cyclosporine treatments，and the condition recurred multiple times during regular follow-up.5.3 years after the first admission，colonoscopy result of patient 2 showed ulcerative and proliferative protrusions in the ileocecal region，as well as the formation of inflammatory polyps.③Literature review results：a literature report of one child （patient 3）with Behcet's disease complicated with perianal polyps was retrieved in the Pub Med database.Patient 3 was a 12-year-old male with main clinical manifestations of oral ulcers，perianal pain，and penile ulcers.Ulcers and polypoid lesions were seen in the perianal area，and local histopathological biopsy results of the perianal area shows non-specific inflammation.After systemic glucocorticoids treatment，the perianal ulcer heals.

Conclusions

Severe inflammation and repeated stimulation are the main causes of Behcet's disease combined with intestinal pseudopolyps.The key point of clinical treatment for this kind of children is to control the recurrence of Behcet's disease and strengthen follow-up.