初步数据显示K12（SSK12）对周期性发热、阿弗他口炎、咽炎和颈淋巴结炎（PFAPA）综合征有效：来自AIDA网络PFAPA综合征登记处的一项多中心研究。
Preliminary data revealing efficacy of K12 (SSK12) in Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis (PFAPA) syndrome: A multicenter study from the AIDA Network PFAPA syndrome registry.
作者信息
La Torre Francesco, Sota Jurgen, Insalaco Antonella, Conti Giovanni, Del Giudice Emanuela, Lubrano Riccardo, Breda Luciana, Maggio Maria Cristina, Civino Adele, Mastrorilli Violetta, Loconte Roberta, Natale Marco Francesco, Celani Camilla, Romeo Mery, Patroniti Serena, Gentile Cristina, Vitale Antonio, Caggiano Valeria, Gaggiano Carla, Diomeda Federico, Cattalini Marco, Lopalco Giuseppe, Emmi Giacomo, Parronchi Paola, Gentileschi Stefano, Cardinale Fabio, Aragona Emma, Shahram Farhad, Marino Achille, Barone Patrizia, Moscheo Carla, Ozkiziltas Burcugul, Carubbi Francesco, Alahmed Ohoud, Iezzi Ludovica, Ogunjimi Benson, Mauro Angela, Tarsia Maria, Mahmoud Ayman Abdel-Monem Ahmed, Giardini Henrique Ayres Mayrink, Sfikakis Petros P, Laskari Katerina, Więsik-Szewczyk Ewa, Hernández-Rodríguez José, Frediani Bruno, Gómez-Caverzaschi Verónica, Tufan Abdurrahman, Almaghlouth Ibrahim A, Balistreri Alberto, Ragab Gaafar, Fabiani Claudia, Cantarini Luca, Rigante Donato
机构信息
Department of Pediatrics, Pediatric Rheumatology Center, Giovanni XXIII Pediatric Hospital, University of Bari, Bari, Italy.
Division of Rheumatology, Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, University of Siena, Siena, Italy.
出版信息
Front Med (Lausanne). 2023 Feb 16;10:1105605. doi: 10.3389/fmed.2023.1105605. eCollection 2023.
OBJECTIVE
To evaluate the potential role of K12 (SSK12) in controlling febrile flares in patients with Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis (PFAPA) syndrome. Further aims were to assess the impact of SSK12 on (i) flare duration, (ii) variation in the degree of the highest body temperature during flares, (iii) steroid-sparing effect, and (iv) change of PFAPA accompanying symptoms before and after SSK12 introduction.
PATIENTS AND METHODS
The medical charts from 85 pediatric patients with PFAPA syndrome (49 males and 36 females) enrolled in the AIDA registry and treated with SSK12 for a median period of 6.00 ± 7.00 months in the period between September 2017 and May 2022 were examined. Children recruited had a median time of disease duration of 19.00 ± 28.00 months.
RESULTS
The number of febrile flares significantly decreased comparing the 12 months before [median (IQR), 13.00 (6.00)] and after SSK12 initiation [median (IQR), 5.50 (8.00), < 0.001]. The duration of fever was significantly reduced from 4.00 (2.00) days to 2.00 (2.00) days [ < 0.001]. Similarly, the highest temperature in°C was found significantly lower in the last follow-up assessment [median (IQR), 39.00 (1.00)] compared to the period prior to SSK12 start [median (IQR), 40.00 (1.00), < 0.001]. Steroid load (mg/year) of betamethasone (or any equivalent steroid) significantly decreased between 12 months before treatment with SSK12 [median (IQR), 5.00 (8.00) mg/year] and the last follow-up visit [median (IQR), 2.00 (4.00) mg/year, < 0.001]. The number of patients experiencing symptoms including pharyngitis/tonsillitis ( < 0.001), oral aphthae ( < 0.001) and cervical lymphadenopathy ( < 0.001) significantly decreased following SSK12.
CONCLUSION
SSK12 prophylaxis given for at least 6.00 months was found to reduce febrile flares of PFAPA syndrome: in particular, it halved the total number per year of fever flares, shortened the duration of the single febrile episode, lowered body temperature by 1°C in the febrile flare, provided a steroid-sparing effect, and significantly reduced the accompanying symptoms related to the syndrome.
目的
评估K12（SSK12）在控制周期性发热、口疮性口炎、咽炎和颈淋巴结炎（PFAPA）综合征患者发热发作中的潜在作用。进一步的目的是评估SSK12对（i）发作持续时间、（ii）发作期间最高体温的变化程度、（iii）激素节省效果以及（iv）引入SSK12前后PFAPA伴随症状变化的影响。
患者和方法
检查了2017年9月至2022年5月期间纳入AIDA登记处并接受SSK12治疗、中位时间为6.00±7.00个月的85例PFAPA综合征儿科患者（49例男性和36例女性）的病历。招募的儿童疾病持续时间中位数为19.00±28.00个月。
结果
与开始使用SSK12前的12个月[中位数（四分位间距），13.00（6.00）]相比，发热发作次数显著减少[中位数（四分位间距），5.50（8.00），<0.001]。发热持续时间从4.00（2.00）天显著缩短至2.00（2.00）天[<0.001]。同样，在最后一次随访评估中，发现最高体温（℃）显著低于开始使用SSK12之前的时期[中位数（四分位间距），39.00（1.00）] [中位数（四分位间距），40.00（1.00），<0.001]。在使用SSK12治疗前12个月[中位数（四分位间距），5.00（8.00）mg/年]与最后一次随访时[中位数（四分位间距），2.00（4.00）mg/年，<0.001]之间，倍他米松（或任何等效激素）的激素负荷（mg/年）显著降低。使用SSK12后，出现咽炎/扁桃体炎（<0.001）、口腔溃疡（<0.001）和颈淋巴结病（<0.001）等症状的患者数量显著减少。
结论
发现给予至少6.00个月的SSK12预防可减少PFAPA综合征的发热发作：特别是，它使每年发热发作的总数减半，缩短了单次发热发作的持续时间，在发热发作时体温降低了1℃，具有激素节省效果，并显著减少了与该综合征相关的伴随症状。
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