IBD Centre, Guy's and St Thomas' NHS Foundation Trust, London, UK.
School of Immunology and Microbial Sciences, King's College London, London, UK.
J Crohns Colitis. 2022 Jun 24;16(5):822-834. doi: 10.1093/ecco-jcc/jjab211.
Metastatic Crohn's disease is an extraintestinal cutaneous manifestation characterised by non-specific inflammatory lesions anatomically separate from the gut; genital involvement is rare. We conducted a systematic review of anogenital Crohn's disease and granulomatosis, to provide a synthesis of epidemiology, clinical features, and treatment outcomes.
A systematic search of the literature was conducted via MEDLINE, EMBASE, and the Cochrane database from inception to December 1, 2020. Two investigators extracted and analysed study data. Response and remission were defined as partial improvement or complete resolution of symptoms and examination findings, respectively.
Of 9381 screened studies, 185 articles, [410 cases: 273 female, 137 male] were included. The predominant clinical features were oedema, ulcers, fissures, and hypertrophic lesions. Adults and children present similarly. Luminal Crohn's disease was diagnosed in nearly 80% of cases including 45-80% patients without gastrointestinal symptoms (time to inflammatory bowel disease [IBD] from anogenital Crohn's disease diagnosis [range] -43 to 11 years). Antibiotics, corticosteroids, thiopurines, and anti-tumour necrosis factor [TNF] therapy were the most frequently prescribed agents. At final follow-up, non-response, response, and remission rates were 37/304 [12%], 267/304 [88%], and 114/304 [38%], respectively. Oedema was associated with a poor response to topical therapy. Greater response rates to anti-TNF therapy were seen in patients prescribed concomitant immunomodulation [24/25, 96% vs 67/90, 74%, p = 0.02].
We provide an illustrative summary of the clinical presentation and treatment effectiveness of this rare, under-recognised condition, and a proposed algorithm for approach and management. Prospective studies with longer follow-up are required to define optimal treatment strategies.
转移性克罗恩病是一种肠道外皮肤表现，其特征为非特异性炎症性病变与肠道解剖分离；生殖器受累罕见。我们对肛门生殖器克罗恩病和肉芽肿病进行了系统回顾，以综合阐述其流行病学、临床特征和治疗结果。
通过 MEDLINE、EMBASE 和 Cochrane 数据库从建库至 2020 年 12 月 1 日进行系统文献检索。两位研究者提取和分析研究数据。缓解和缓解定义为症状和检查结果的部分改善或完全缓解。
在 9381 项筛选研究中，纳入了 185 篇文章[410 例：273 例女性，137 例男性]。主要的临床特征是水肿、溃疡、裂隙和增生性病变。成人和儿童的表现相似。近 80%的病例诊断为腔克罗恩病，包括 45-80%无胃肠道症状的患者（从肛门生殖器克罗恩病诊断到炎症性肠病[IBD]的时间[范围]-43 至 11 年）。抗生素、皮质类固醇、硫嘌呤和抗肿瘤坏死因子[TNF]治疗是最常开的药物。在最终随访时，无反应、反应和缓解率分别为 37/304[12%]、267/304[88%]和 114/304[38%]。水肿与局部治疗反应不良相关。同时接受免疫调节治疗的患者接受抗 TNF 治疗的反应率更高[24/25，96%比 67/90，74%，p=0.02]。
我们提供了对这种罕见的、认识不足的疾病的临床表现和治疗效果的说明性总结，并提出了一种治疗方法和管理的建议算法。需要前瞻性研究和更长的随访时间来确定最佳的治疗策略。
