Gale Gita, Sigurdsson Gudmundur Vignir, Östman Sofia, Malmborg Petter, Högkil Karin, Hasséus Bengt, Jontell Mats, Saalman Robert
*Department of Oral Medicine and Pathology, Institute of Odontology, The Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden; †Department of Pediatrics, Institute of Clinical Sciences, The Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden; ‡Department of Infectious Medicine, Institute of Biomedicine, The Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden; §Department of Women's and Children's Health, Astrid Lindgren Children's Hospital, Karolinska University Hospital, Karolinska Institute, Stockholm, Sweden; and ‖Paediatric Dentistry, Eastman Institute, Public Dental Health, Stockholm, Sweden.
Inflamm Bowel Dis. 2016 May;22(5):1071-7. doi: 10.1097/MIB.0000000000000670.
Although orofacial granulomatosis (OFG) may present as a separate clinical entity, it often seems in conjunction with various systemic diseases, of which Crohn's disease (CD) is one of the most common. The aim of this study was to investigate whether CD with concomitant OFG represents a distinctive disease subtype.
Twenty-one patients with CD and concomitant OFG (CD+OFG group) were included in the study. As the reference group, a cohort of 39 patients with CD but without OFG (CD-R group) was used. Demographic data and clinical characteristics were recorded at the time of diagnosis. The 2 groups were compared using multivariate analyses.
The percentage of patients with intestinal inflammation in the upper gastrointestinal tract was significantly higher in the CD+OFG group, as compared with the CD-R group (81% versus 33%; P < 0.001). Furthermore, ileocolonic inflammation was significantly more common in the CD+OFG patients (81% versus 46%; P = 0.013). In addition, perianal disease was more frequently observed in the CD+OFG group (48% versus 18%; P = 0.033). Significantly more patients showed evidence of granulomas in the primary endoscopy in the CD+OFG group than in the CD-R group (81% versus 38%; P = 0.003).
The data from this study suggest that the presence of CD in conjunction with OFG represents a distinctive subphenotype of CD that is characterized by extensive inflammation, perianal disease, and pronounced granuloma formation in the intestine.
尽管口面部肉芽肿病（OFG）可能表现为一种独立的临床实体，但它常与各种全身性疾病同时出现，其中克罗恩病（CD）是最常见的疾病之一。本研究的目的是调查合并OFG的CD是否代表一种独特的疾病亚型。
本研究纳入了21例合并OFG的CD患者（CD+OFG组）。作为参照组，使用了39例患有CD但无OFG的患者队列（CD-R组）。在诊断时记录人口统计学数据和临床特征。使用多变量分析对两组进行比较。
与CD-R组相比，CD+OFG组上消化道肠道炎症患者的百分比显著更高（81%对33%；P<0.001）。此外，回结肠炎症在CD+OFG患者中明显更常见（81%对46%；P=0.013）。另外，CD+OFG组中肛周疾病的观察频率更高（48%对18%；P=0.033）。与CD-R组相比，CD+OFG组在初次内镜检查时有明显更多的患者显示出肉芽肿证据（81%对38%；P=0.003）。
本研究数据表明，合并OFG的CD代表CD的一种独特亚表型，其特征为广泛炎症、肛周疾病以及肠道中明显的肉芽肿形成。
