Department of Internal Medicine, Cleveland Clinic, Cleveland, OH, USA.
Department of Gastroenterology, Hepatology and Nutrition, Digestive Diseases and Surgery Institute, Cleveland Clinic, Cleveland, OH, USA.
J Crohns Colitis. 2021 Nov 8;15(11):1864-1876. doi: 10.1093/ecco-jcc/jjab079.
Sweet syndrome [SS] is a dermatological condition associated with both inflammatory bowel disease [IBD] and azathioprine use. We performed a systematic review to better delineate clinical characteristics and outcomes of SS in IBD patients.
Peer-reviewed, full-text journal publications from inception to April 2020 in English language and adult subjects with IBD were included. Skin biopsy was required as SS gold-standard diagnosis. Azathioprine-associated SS required recent azathioprine introduction or recurrence of SS after azathioprine re-challenge.
We included 89 publications with 95 patients [mean age of SS diagnosis: 44 years; 59% female; 20 with azathioprine-associated SS and 75 without]. SS was diagnosed prior to IBD in 5.3%, at time of IBD diagnosis in 29.5% and after diagnosis in 64.2%. In total, 91% of patients with SS had known colonic involvement and the majority [76%] had active IBD at diagnosis; 22% had additional extra-intestinal manifestations. Successful therapies for SS included corticosteroids [90.5%], anti-tumour necrosis factor [TNF]-α inhibitor therapy [14.8%] and azathioprine [11.6%]. Azathioprine-associated SS was distinct, with 85% male patients, mean age of SS diagnosis of 50 years and a lower likelihood to be prescribed corticosteroids for treatment [75% vs 94.7% of non-azathioprine-associated SS, p = 0.008]. All patients with azathioprine-associated SS improved with medication cessation and developed recurrence after re-challenge.
SS may precede or occur with IBD diagnosis in almost one-third of cases. Azathioprine and IBD-associated SS present and behave distinctly, especially with regard to gender, age at diagnosis and recurrence risk. Corticosteroids and TNF-α inhibitors have demonstrated efficacy in treating SS in IBD.
Sweet 综合征[SS]是一种与炎症性肠病[IBD]和巯嘌呤使用相关的皮肤病。我们进行了一项系统综述,以更好地描绘 IBD 患者中 SS 的临床特征和结局。
纳入从成立到 2020 年 4 月以英文发表的、包含成年 IBD 患者的同行评审全文期刊出版物。皮肤活检是 SS 的金标准诊断方法。与巯嘌呤相关的 SS 需要最近开始使用巯嘌呤或在重新使用巯嘌呤后 SS 复发。
我们纳入了 89 项研究,共 95 名患者[SS 诊断的平均年龄为 44 岁;59%为女性;20 名患者为与巯嘌呤相关的 SS,75 名患者为无相关 SS]。SS 在 IBD 之前诊断的占 5.3%,在 IBD 诊断时诊断的占 29.5%,在 IBD 诊断后诊断的占 64.2%。总的来说,91%的 SS 患者有已知的结肠受累,大多数[76%]在诊断时存在活动 IBD;22%有额外的肠外表现。SS 的有效治疗方法包括皮质类固醇[90.5%]、抗肿瘤坏死因子[TNF]-α 抑制剂治疗[14.8%]和巯嘌呤[11.6%]。与巯嘌呤相关的 SS 是独特的,85%为男性患者,SS 诊断的平均年龄为 50 岁,更有可能不使用皮质类固醇治疗[75%与非与巯嘌呤相关的 SS 患者的 94.7%相比,p=0.008]。所有与巯嘌呤相关的 SS 患者在停药后均有改善,并在重新用药后复发。
SS 可能在近三分之一的病例中先于 IBD 诊断或与 IBD 同时发生。与巯嘌呤和 IBD 相关的 SS 表现和行为不同,特别是在性别、诊断时的年龄和复发风险方面。皮质类固醇和 TNF-α 抑制剂已被证明在治疗 IBD 中的 SS 有效。