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Case Reports
. 2018 Jun 4;18(1):207.
doi: 10.1186/s12884-018-1854-6.

Pregnancy complicated with PFAPA (periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis) syndrome: a case report

Kuniaki Ota  1   2 , Joanne Kwak-Kim  3 , Toshifumi Takahashi  1   2 , Hideki Mizunuma  2
Affiliations
Case Reports

Pregnancy complicated with PFAPA (periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis) syndrome: a case report

Kuniaki Ota et al. BMC Pregnancy Childbirth. .

Abstract

Background: 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.

Case presentation: 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.

Conclusion: 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.

Keywords: Adult onset; Aphthous stomatitis; Cervical adenitis; PFAPA; Periodic fever; Pharyngitis; Pregnancy; Th1/Th2.

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Conflict of interest statement

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Not applicable.

Consent for publication

Written informed consent for publication of the clinical details and/or clinical images was obtained from the patient. A copy of the consent form is available for review by the Editor of this journal.

Competing interests

The authors declare that they have no competing interests.

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Figures

Fig. 1
Fig. 1
Th1/Th2 cell ratio (IFN-γ/IL-4 T helper cell ratio) was analyzed by flow cytometric analysis. An acquisition gate was established based on CD4 staining and side scatter (SCC) which included peripheral blood mononuclear cells (left). Dot plot analysis of IFN-γ and IL-4 expressing CD4+ T cells from the patient. Numbers indicate percent gated cells (right)
Fig. 2
Fig. 2
Time course plot of Th1/Th2 cell ratios (IFN-γ/IL-4 T helper cell ratio) from early pregnancy to postpartum 1 year. The white box indicates the period of cimetidine treatment and the slant bow indicates the period of colchicine treatment
See this image and copyright information in PMC

References

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