PFAPA Syndrome in a Young Adult with a History of Tonsillectomy

Abstract

Since its clinical definition in 1987, the syndrome called, "periodic fever, aphtous stomatitis, pharyngitis and cervical adenitis" syndrome (PFAPA) has been considered peculiar to pediatric age. In the recent literature there are a few case reports of PFAPA in adults. We describe a case of a 21-year-old female affected by PFAPA who presented a history of tonsillectomy at the age of four. To our knowledge this is the fourth case described with a diagnosis of PFAPA in an adult with a history of tonsillectomy during childhood. Although the role of tonsillectomy in the treatment of PFAPA is still controversial, due to the lack of definitive data in literature, this case suggests that fever episodes may relapse several years after surgery.

Periodisches Fieber, aphthöse Stomatitis, Pharyngitis und Lymphadenitis (PFAPA-Syndrom) im Erwachsenenalter

“…Häufige Berichte über die "Ausheilung" in der Adoleszenz lassen einen selbstlimitierten Verlauf vermuten [22]. In der aktuellen Literatur verweisen einige Quellen auf die Möglichkeit einer fortbestehenden Symptomatik über das Kindesalter hinaus, auf den Beginn des PFAPA-Syndroms im Erwachsenenalter [3,5,7,17,22,28] oder auch auf einen späteren Krankheitsbeginn im Erwachsenenalter [22]. In der folgenden Arbeit berichten wir über die Manifestation des PFAPA-Syndroms bei drei erwachsenen Patienten, von denen zwei bereits im Kindesalter als geheilt galten, und diskutieren die klinische Symptomatik, Diagnostik, Therapie und Relevanz des PFAPA-Syndroms anhand der aktuellen Literatur.…”

“…In wenigen Studien wird von Patienten berichtet, bei denen es nach unterschiedlich langen Phasen der Spontanremission, die einige Monate bis zu 3 Jahren andauerten, erneut zu PFAPA-Episoden kam [22,30,34]. [7]. In 3 weiteren Publikationen wird ebenfalls über das Phänomen einer Remissionsphase der Erkrankung nach Tonsillektomie/Adenotonsillektomie mit plötzlichem Relaps berichtet.…”

“…Lebensjahr rezidivierende Fieberschübe [22]. Neben den 3 Patienten unseres Kollektivs, wird nach systematischer Literaturrecherche in der Datenbank PubMed bisher nur über weitere 20 erwachsene PFAPA-Patienten berichtet [3,5,7,17,22]. Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA syndrome) in adults ▼ Background and objective: The PFAPA syndrome is characterized by a regular appearance of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis and is associated with a distinctive poor general condition of the patients.…”

Marshall syndrome – a challenge in medical practice

“…Other symptoms may be present during fever attacks, such as headache, joint pain, myalgia, nausea, vomiting and abdominal pain. Onset of the disease is before the age of 5 years and usually lasts up to the age of 10-11 years, but there are some cases diagnosed in adults (3,4). Patients are asymptomatic between episodes and have normal growth and development (2,3,5).…”

Caution Should be Used in the Recognition of Adult-Onset Autoinflammatory Disorders: Facts or Fiction?

“…This syndrome does not have a documented genetic basis, and spontaneous resolution of fever episodes is commonly observed a few years after symptom onset (Marshall et al, 1987, 1989; Thomas et al, 1999). Recent medical literature has included dozens of suspected cases in adults as well, suggesting that it should be taken into consideration also in adults (Cavuoto and Bonagura, 2008; Padeh et al, 2008; Colotto et al, 2011; Cantarini et al, 2012d,e; Cazzato et al, 2013). …”