Dantas Marina A S, Graneiro Ana Luiza, Cavalcante Rodrigo, Felipez Lina Maria
Department of Pediatric Hospital Medicine, Medical University of South Carolina, Charleston, SC, USA.
Department of Allergy and Immunology, Nicklaus Children's Hospital, Miami, FL, USA.
Case Rep Pediatr. 2023 Oct 4;2023:4705638. doi: 10.1155/2023/4705638. eCollection 2023.
. Differentiating Crohn's disease (CD) and Behçet's disease (BD) with gastrointestinal (GI) manifestations can be clinically challenging, as current diagnostic criteria are not clear between both conditions and multiple symptoms could overlap. . The patient is an 8-year-old boy of Brazilian descent, who initially presented with a 1-year history of painful oral ulcers. Before presenting to the hospital, he had been treated for periodic fever, aphthous stomatitis, pharyngitis, and adenitis and placed on steroids, with relapsing symptoms on attempts to wean the doses. The initial workup was largely unremarkable. Buccal biopsies showed no granulomas, and the ophthalmologic exam was normal. Infectious and rheumatological tests were negative. Prometheus IBD sgi testing showed a pattern consistent with CD; however, the patient had multiple negative endoscopies, colonoscopies, and capsule endoscopies. He developed intermittent bloody stools and severe malnutrition and did not respond to infliximab, colchicine, or methotrexate. After a large GI bleed, a 4th colonoscopy was performed, which showed large round ulcers in the terminal ileum, and no granulomas. He was started on ustekinumab with clinical improvement. One month later, he developed bilateral hip effusion and meningismus, being diagnosed with aseptic meningitis secondary to COVID-19. He improved, but in one month developed worsening symptoms, and MRV showed extensive venous sinus thrombosis. The patient was started on enoxaparin, methylprednisolone, and colchicine, with resolution of the thrombus on a 3-month follow-up. The patient's overall symptoms remained controlled with clinical and biochemical remission on monthly ustekinumab. . Our patient had a challenging clinical course, with nonspecific systemic and intestinal manifestations which proved difficult to differentiate between BD and CD. Given endoscopic findings and the worsening of an auto-inflammatory reaction in the central nervous system after COVID-19 in a patient with controlled GI symptoms, the most likely diagnosis is BD.
鉴别具有胃肠道表现的克罗恩病(CD)和白塞病(BD)在临床上具有挑战性,因为目前的诊断标准在这两种疾病之间并不明确,且多种症状可能重叠。该患者是一名8岁的巴西裔男孩,最初有1年的疼痛性口腔溃疡病史。在入院前,他曾因周期性发热、阿弗他口炎、咽炎和腺炎接受治疗,并使用过类固醇药物,在尝试减药时症状复发。最初的检查结果大多无异常。颊部活检未发现肉芽肿,眼科检查正常。感染和风湿学检查均为阴性。Prometheus IBD sgi检测显示出与CD一致的模式;然而,该患者的多次内镜检查、结肠镜检查和胶囊内镜检查均为阴性。他出现了间歇性便血和严重营养不良,对英夫利昔单抗、秋水仙碱或甲氨蝶呤均无反应。在一次严重的胃肠道出血后,进行了第四次结肠镜检查,结果显示回肠末端有大的圆形溃疡,且无肉芽肿。他开始使用乌司奴单抗治疗,临床症状有所改善。一个月后,他出现双侧髋关节积液和颈项强直,被诊断为继发于COVID-19的无菌性脑膜炎。他的病情有所好转,但一个月后症状加重,磁共振静脉血管造影(MRV)显示广泛的静脉窦血栓形成。该患者开始使用依诺肝素、甲泼尼龙和秋水仙碱治疗,在3个月的随访中血栓溶解。患者的总体症状通过每月使用乌司奴单抗保持临床和生化缓解而得到控制。我们的患者临床病程具有挑战性,有非特异性的全身和肠道表现,难以区分BD和CD。鉴于内镜检查结果以及一名胃肠道症状得到控制的患者在COVID-19后中枢神经系统自身炎症反应恶化,最可能的诊断是BD。