Kiratikanon Salin, Phinyo Phichayut, Rujiwetpongstorn Rujira, Patumanond Jayanton, Tungphaisal Veeraphol, Mahanupab Pongsak, Chaiwarith Romanee, Tovanabutra Napatra, Chiewchanvit Siri, Chuamanochan Mati
Division of Dermatology, Department of Internal Medicine, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand.
Center for Clinical Epidemiology and Clinical Statistics, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand.
J Dermatol. 2022 Jan;49(1):133-141. doi: 10.1111/1346-8138.16202. Epub 2021 Oct 21.
Sweet syndrome (SS) has been increasingly reported in patients with adult-onset immunodeficiency (AOID) due to anti-interferon-γ autoantibody who also have concomitant opportunistic infections, especially disseminated non-tuberculous mycobacterial infection (dNTMI). A retrospective study retrieving data from 2011 through 2020 was conducted. We compared clinical characteristics of SS with and without AOID and generated the prediction model and examined the interaction between AOID and dNTMI in the occurrence of SS. Lymphadenopathy, pustular lesions, and leukocytosis are the significant predictors for AOID-associated SS. Adjusted risk differences were 0.58 (95% confidence interval [CI], 0.33-0.83), 0.21 (95% CI, 0.02-0.39), and 0.24 (95% CI, 0.01-0.47), respectively. Based on the analysis of aggregated cross-sectional data, both the overall and the direct effect of AOID increased the prevalence of SS. The indirect effect of AOID on the occurrence of SS might also be mediated through dNTMI or other common opportunistic infections. In addition, there was a trend of positive additive interaction between AOID and dNTMI. Although the test of additive interaction did not reveal statistically significant results, a deviation from additivity of isolated effects might suggest potential causal interaction between AOID and dNTMI. The distinctive clinical syndrome comprising lymphadenopathy, pustular lesions, and leukocytosis in patients with SS should raise the awareness of clinicians to the potential of underlying AOID.
在因抗干扰素-γ自身抗体导致成人起病免疫缺陷（AOID）且伴有机会性感染，尤其是播散性非结核分枝杆菌感染（dNTMI）的患者中，Sweet综合征（SS）的报道日益增多。我们进行了一项回顾性研究，收集2011年至2020年的数据。我们比较了有和没有AOID的SS患者的临床特征，建立了预测模型，并研究了AOID与dNTMI在SS发生中的相互作用。淋巴结病、脓疱性皮损和白细胞增多是AOID相关SS的重要预测因素。调整后的风险差异分别为0.58（95%置信区间[CI]，0.33 - 0.83）、0.21（95%CI，0.02 - 0.39）和0.24（95%CI，0.01 - 0.47）。基于汇总横断面数据的分析，AOID的总体效应和直接效应均增加了SS的患病率。AOID对SS发生的间接效应也可能通过dNTMI或其他常见的机会性感染介导。此外，AOID与dNTMI之间存在正相加相互作用的趋势。虽然相加相互作用检验未显示出具有统计学意义的结果，但孤立效应的相加性偏差可能提示AOID与dNTMI之间存在潜在的因果相互作用。SS患者出现的包括淋巴结病、脓疱性皮损和白细胞增多在内的独特临床综合征应提高临床医生对潜在AOID的认识。
