An Underlooked Cause of Periodic Fever (PFAPA) in an Adult Patient with No Response to Tonsillectomy

Periodic fever with aphthous stomatitis, pharyngitis, and adenitis (PFAPA) is an autoinflammatory disease manifested as recurrent febrile episodes associated with one of the following cardinal features: aphthous ulceration, pharyngitis, and cervical adenitis. It was initially described in children a...

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Main Authors: Fiaz Alam, Mohammed Hammoudeh
Format: Article
Language:English
Published: Wiley 2018-01-01
Series:Case Reports in Rheumatology
Online Access:http://dx.doi.org/10.1155/2018/6580835
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author Fiaz Alam
Mohammed Hammoudeh
author_facet Fiaz Alam
Mohammed Hammoudeh
author_sort Fiaz Alam
collection DOAJ
description Periodic fever with aphthous stomatitis, pharyngitis, and adenitis (PFAPA) is an autoinflammatory disease manifested as recurrent febrile episodes associated with one of the following cardinal features: aphthous ulceration, pharyngitis, and cervical adenitis. It was initially described in children and thought to be a disease of pediatric age group. Few adult cases were also reported in the literature. We describe the case of a 39-year-old female affected by PFAPA who presented with a history of febrile episodes associated with aphthous ulceration, stomatitis, and tonsillitis for 4 years. The febrile episodes occurred at a regular interval of 4 weeks and resolved within 5 days. The patient underwent tonsillectomy without any significant improvement. The patient responded only to a single high dose of steroid during the attack. Although PFAPA was initially thought to be a disease of pediatric age group, it should be considered in patients with recurrent febrile illness in all age groups.
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spelling doaj-art-a08dec079bbe4be1ad740c92b63cea142025-08-20T02:20:52ZengWileyCase Reports in Rheumatology2090-68892090-68972018-01-01201810.1155/2018/65808356580835An Underlooked Cause of Periodic Fever (PFAPA) in an Adult Patient with No Response to TonsillectomyFiaz Alam0Mohammed Hammoudeh1Department of Medicine, Hamad General Hospital, Doha, QatarDepartment of Medicine, Hamad General Hospital, Doha, QatarPeriodic fever with aphthous stomatitis, pharyngitis, and adenitis (PFAPA) is an autoinflammatory disease manifested as recurrent febrile episodes associated with one of the following cardinal features: aphthous ulceration, pharyngitis, and cervical adenitis. It was initially described in children and thought to be a disease of pediatric age group. Few adult cases were also reported in the literature. We describe the case of a 39-year-old female affected by PFAPA who presented with a history of febrile episodes associated with aphthous ulceration, stomatitis, and tonsillitis for 4 years. The febrile episodes occurred at a regular interval of 4 weeks and resolved within 5 days. The patient underwent tonsillectomy without any significant improvement. The patient responded only to a single high dose of steroid during the attack. Although PFAPA was initially thought to be a disease of pediatric age group, it should be considered in patients with recurrent febrile illness in all age groups.http://dx.doi.org/10.1155/2018/6580835
spellingShingle Fiaz Alam
Mohammed Hammoudeh
An Underlooked Cause of Periodic Fever (PFAPA) in an Adult Patient with No Response to Tonsillectomy
Case Reports in Rheumatology
title An Underlooked Cause of Periodic Fever (PFAPA) in an Adult Patient with No Response to Tonsillectomy
title_full An Underlooked Cause of Periodic Fever (PFAPA) in an Adult Patient with No Response to Tonsillectomy
title_fullStr An Underlooked Cause of Periodic Fever (PFAPA) in an Adult Patient with No Response to Tonsillectomy
title_full_unstemmed An Underlooked Cause of Periodic Fever (PFAPA) in an Adult Patient with No Response to Tonsillectomy
title_short An Underlooked Cause of Periodic Fever (PFAPA) in an Adult Patient with No Response to Tonsillectomy
title_sort underlooked cause of periodic fever pfapa in an adult patient with no response to tonsillectomy
url http://dx.doi.org/10.1155/2018/6580835
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