A Case of Adult-Onset pFAPA

Cavuoto, M.A.; Bonagura, V.R.

doi:10.1016/j.jaci.2005.12.838

Cited by 3 publications

(4 citation statements)

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“…Although the first case of adult PFAPA was reported in 2006, there has been a paucity of case reports and case series since then [17]. In 2008, the first series of patients with adult PFAPA was published and raised awareness about the need for considering this entity in adults with periodic fever [4].…”

Section: Discussionmentioning

confidence: 99%

Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome: A Rare Etiology of Fever in Adults

Cristina¹,

Costa

Toscano³

et al. 2021

Cureus

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We report the case of a 28-year-old male, with a past history of recurrent pharyngitis and tonsillectomy, who presented to the emergency department with fever, pharyngitis, and cervical adenitis. Inflammatory markers were elevated and the patient was initially started on ceftriaxone with remission after four days. However, the symptoms recurred three weeks later and an autoinflammatory disease was suspected. After exclusion of other illnesses, a diagnosis of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome was confirmed. The patient was successfully treated with a single dose of 60 mg of prednisolone at the beginning of the flare.PFAPA syndrome has been classically diagnosed solely in children but cases in adults are being increasingly recognized. Despite the increasing evidence of the delayed onset of PFAPA syndrome during adulthood, no specific tools are available to detect it and diagnosis is currently based on clinical diagnostic criteria, which have very low specificity and are tailored to pediatric patients. This case report stresses the need to consider this entity seriously despite its rarity, even among the adult population, so as to reduce iatrogenesis, start appropriate therapy in a prompt manner, and improve the quality of life of PFAPA patients.

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Section: Discussionmentioning

confidence: 99%

Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome: A Rare Etiology of Fever in Adults

Cristina¹,

Costa

Toscano³

et al. 2021

Cureus

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“…In adults, reports of FAPA are rare [4,5]. The differential diagnosis of FAPA includes Behcet’s syndrome, Familial Mediterranean Fever (FMF), hyper-IgD syndrome [2,3].…”

Section: Introductionmentioning

confidence: 99%

“…Marshall’s syndrome or FAPA (periodic fever, aphthous stomatititis, pharyngitis and adenitis) was firstdescribed in pediatrics [ 3 ]. In adults, reports of FAPA are rare [ 4 , 5 ]. The differential diagnosis of FAPA includes Behcet’s syndrome, Familial Mediterranean Fever (FMF), hyper-IgD syndrome [ 2 , 3 ].…”

Section: Introductionmentioning

confidence: 99%

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Recurrent Fever of Unknown Origin (FUO) Due to Periodic Fever, Aphthous Stomatititis, Pharyngitis and Adenitis (FAPA) Syndrome in an Adult

Munoz-Gomez

Cunha

2013

JCM

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FAPA syndrome (periodic fever, aphthous stomatititis, pharyngitis and adenitis) is a relatively new entity described in pediatric patients. In adults, reports of FAPA are limited to rare case reports. The differential diagnosis of FAPA in adults includes Behcet’s syndrome, familial Mediterranean fever (FMF), Hyper IgD syndrome and juvenile rheumatoid arthritis (JRA), i.e., adult Still’s disease. With FAPA syndrome, between episodes patients are completely asymptomatic and serologic inflammatory markers such as erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and white blood cell (WBC) count are normal. The etiology of FAFA is unknown, but lack of secondary cases or clustering in close contacts, lack of seasonality, and the lack of progression for years argue against an infectious etiology. We describe an extremely rare case of an adult with a recurrent FUO with profuse night sweats and prominent chills due to FAPA syndrome.

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Periodic Fever accompanied by Aphthous stomatitis, Pharyngitis, and cervical Adenitis syndrome (PFAPA syndrome) in adults

2008

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A Case of Adult-Onset pFAPA

Cited by 3 publications

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Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome: A Rare Etiology of Fever in Adults

Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome: A Rare Etiology of Fever in Adults

Recurrent Fever of Unknown Origin (FUO) Due to Periodic Fever, Aphthous Stomatititis, Pharyngitis and Adenitis (FAPA) Syndrome in an Adult

Periodic Fever accompanied by Aphthous stomatitis, Pharyngitis, and cervical Adenitis syndrome (PFAPA syndrome) in adults

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