Systematic Review: Sweet Syndrome Associated with Inflammatory Bowel Disease. (23rd April 2021)

• Record Type: Journal Article
• Authors: Sleiman, Joseph; Hitawala, Asif A; Cohen, Benjamin; Falloon, Katie; Simonson, Marian; Click, Benjamin; Khanna, Urmi; Fernandez, Anthony P; Rieder, Florian
• Is Part Of: Journal of Crohn's and colitis. Volume 15:Number 11(2021)
• Page Start: 1864
• Page End: 1876
• Publication Date: 2021-04-23
• Subjects: Sweet syndrome -- inflammatory bowel disease -- azathioprine -- skin -- extraintestinal manifestation; Inflammatory bowel diseases -- Periodicals; 616.344005
• DOI: 10.1093/ecco-jcc/jjab079
• Languages: English
• ISSNs: 1873-9946

Abstract

Abstract: Background and Aims: 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. Methods: 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. Results: 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. Conclusions: 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.