Department of Obstetrics and Gynecology, School of Medicine, Fukushima Medical University, 1-Hikarigaoka, Fukushima, 960-1247, Japan.
Fukushima Medical Center for Children and Women, School of Medicine, Fukushima Medical University, 1-Hikarigaoka, Fukushima, 960-1247, Japan.
BMC Pregnancy Childbirth. 2018 Jun 4;18(1):207. doi: 10.1186/s12884-018-1854-6.
Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome has been considered as a childhood syndrome. The underlying etiology of PFAPA syndrome is unclear however, currently considered as auto-immune inflammatory disease. Recently, a few cases of adult-onset of PFAPA syndrome have been reported. However, there is no report about the successful management of pregnancy complicated with PFAPA syndrome.
The patient was a 31-year-old woman who developed recurrent episodes of high fever associated with cervical adenitis, pharyngitis and vomiting started 9 months after a delivery. She was diagnosed with PFAPA syndrome and cimetidine 800 mg/day was initiated. Since then, these symptoms got better. Cimetidine treatment was discontinued since she became pregnant (6 weeks of pregnancy). Except one febrile episode at 8 weeks gestation, she did not develop a febrile episode during pregnancy. Peripheral blood Th1/Th2 ratio was decreased from the first trimester to the second trimester of pregnancy. Then again, the ratio was steadily elevated during the third trimester. At 38 weeks, she delivered a live born infant without any complication. Two months after delivery, she developed PFAPA syndrome again and cimetidine treatment was re-initiated. However, febrile episodes were not controlled well, and Th1/Th2 ratio was further elevated compared to pregnancy status. Colchicine 0.5 mg once a day was initiated. Symptoms were diminished and Th1/Th2 ratio was gradually decreased.
There was no case report of pregnancy complicated with PFAPA syndrome, though there were several reports of adult-onset PFAPA cases without pregnancy. The current case may be the first case report of a successful pregnancy complicated with PFAPA. In this case, PFAPA symptoms were ameliorated during pregnancy, but reappeared after delivery. We speculate that PFAPA syndrome, a Th1 type immune disorder, might be improved due to the Th1 to Th2 shifting, which was induced by pregnancy. It is necessary to investigate further about PFAPA syndrome with pregnancy and Th1/Th2 immune responses in the future.
周期性发热、口疮性口炎、咽炎和颈部淋巴结炎（PFAPA）综合征被认为是一种儿童综合征。PFAPA 综合征的潜在病因尚不清楚，但目前被认为是自身免疫性炎症性疾病。最近，有几例成人发病的 PFAPA 综合征的报道。然而，目前尚无关于妊娠合并 PFAPA 综合征的成功管理的报告。
患者是一名 31 岁女性，在分娩后 9 个月开始出现反复高热，伴有颈部淋巴结炎、咽炎和呕吐。她被诊断为 PFAPA 综合征，开始每天服用西咪替丁 800mg。此后，这些症状得到了改善。由于她怀孕（怀孕 6 周），停止了西咪替丁治疗。除了怀孕 8 周时出现一次发热外，她在怀孕期间没有出现发热。妊娠早期至中期外周血 Th1/Th2 比值下降，然后在妊娠晚期再次稳步升高。在 38 周时，她顺利分娩了一名活产婴儿，没有任何并发症。产后 2 个月，她再次出现 PFAPA 综合征，重新开始使用西咪替丁治疗。然而，发热症状控制不佳，Th1/Th2 比值与妊娠时相比进一步升高。开始每天服用 0.5mg 秋水仙碱。症状减轻，Th1/Th2 比值逐渐降低。
尽管有几例成人发病的 PFAPA 病例没有怀孕，但没有妊娠合并 PFAPA 综合征的病例报告。本病例可能是首例妊娠合并 PFAPA 成功的病例报告。在本病例中，PFAPA 症状在妊娠期间得到改善，但分娩后再次出现。我们推测，PFAPA 综合征是一种 Th1 型免疫紊乱，可能由于妊娠诱导的 Th1 向 Th2 转移而得到改善。未来有必要进一步研究妊娠和 Th1/Th2 免疫反应与 PFAPA 综合征的关系。
