Colchicine as a therapeutic option in periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis (PFAPA) syndrome

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• 作者：Yonatan Butbul Aviel ; MD^a ; ^b ; ^c ; ^{yonatanbutbul@gmail.com" class="auth_mail" title="E-mail the corresponding author} ; Sameh Tatour ; MD^a ; ^c ; Ruth Gershoni Baruch ; MD^c ; Riva Brik ; MD^a ; ^b ; ^c
• 关键词：Periodic fever ; aphthous stomatitis ; pharyngitis ; cervical adenitis (PFAPA) ; Colchicine
• 刊名：Seminars in Arthritis and Rheumatism
• 出版年：2016
• 出版时间：February 2016
• 年：2016
• 卷：45
• 期：4
• 页码：471-474
• 全文大小：246 K

文摘

To evaluate the efficacy of colchicine in reducing the frequency of attacks in patients with PFAPA.

Study design

We conducted a 6-month open label, randomized, controlled study among patients with PFAPA who attend the Pediatric Rheumatology Clinic at the Rambam Medical Center in Israel.

A total of 18 patients aged4 –11 years (males:females ratio = 11:7) were randomized into a control group (I, 10 children) and a study group (II, 8 children). Group I was followed for 6 months without any intervention, and group II was initially followed for 3 months and was thereafter treated with colchicine for 3 additional months, according to standard regimen. During the 6-month period of the study the patients and their physician recorded all the episodes of PFAPA in a constructed log.

DNA analyses for the 5 common FMF mutations in Israel were performed in 17 out of the 18 patients.

Results

The number of episodes during the first 3 months was similar in both groups (group I 3.2 ± 1.5, group II 4.9 ± 2.3; p ≤ 0.12). Group II had significantly less PFAPA attacks in the second period while on colchicine therapy (4.9 ± 2.3 vs. 1.6 ± 1.2; p ≤ 0.01), in opposition to group I, where no difference in the number of attacks was noted between the first and second period of follow-up (3.2 ± 1.5 vs. 2.7 ± 1.5; p = 0.33).

Of the 17 patients tested, 8 were carriers for FMF mutations (2 in group I and 6 in group II).

Conclusion

Colchicine prophylaxis seems to be effective in reducing the number of attacks in PFAPA.