Adult-Onset Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis

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

doi:10.1016/s1081-1206(10)60428-0

Cited by 12 publications

(11 citation statements)

References 7 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Patients are asymptomatic between episodes and show normal growth and development. PFAPA usually resolves in adolescence, although a small but increasing number of patients with adult-onset disease have been reported (7)(8)(9).…”

mentioning

confidence: 99%

Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) is a disorder of innate immunity and Th1 activation responsive to IL-1 blockade

Stojanov

Lapidus²,

Chitkara³

et al. 2011

Proc. Natl. Acad. Sci. U.S.A.

250

202

View full text Add to dashboard Cite

The syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) is the most common periodic fever disease in children. However, the pathogenesis is unknown. Using a systems biology approach we analyzed blood samples from PFAPA patients whose genetic testing excluded hereditary periodic fevers (HPFs), and from healthy children and pediatric HPF patients. Gene expression profiling could clearly distinguish PFAPA flares from asymptomatic intervals, HPF flares, and healthy controls. During PFAPA attacks, complement ( C1QB, C2, SERPING1 ), IL-1–related ( IL-1B, IL-1RN, CASP1, IL18RAP ), and IFN-induced ( AIM2 , IP-10/CXCL10 ) genes were significantly overexpressed, but T cell-associated transcripts ( CD3, CD8B ) were down-regulated. On the protein level, PFAPA flares were accompanied by significantly increased serum levels of chemokines for activated T lymphocytes (IP-10/CXCL10, MIG/CXCL9), G-CSF, and proinflammatory cytokines (IL-18, IL-6). PFAPA flares also manifested a relative lymphopenia. Activated CD4 + /CD25 + T-lymphocyte counts correlated negatively with serum concentrations of IP-10/CXCL10, whereas CD4 + /HLA-DR + T lymphocyte counts correlated positively with serum concentrations of the counterregulatory IL-1 receptor antagonist. Based on the evidence for IL-1β activation in PFAPA flares, we treated five PFAPA patients with a recombinant IL-1 receptor antagonist. All patients showed a prompt clinical and IP-10/CXCL10 response. Our data suggest an environmentally triggered activation of complement and IL-1β/-18 during PFAPA flares, with induction of Th1-chemokines and subsequent retention of activated T cells in peripheral tissues. IL-1 inhibition may thus be beneficial for treatment of PFAPA attacks, with IP-10/CXCL10 serving as a potential biomarker.

show abstract

mentioning

confidence: 99%

Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) is a disorder of innate immunity and Th1 activation responsive to IL-1 blockade

Stojanov

Lapidus²,

Chitkara³

et al. 2011

Proc. Natl. Acad. Sci. U.S.A.

250

202

View full text Add to dashboard Cite

show abstract

“…Although since its first description in 1987 it had been considered peculiar to pediatric age, the recent literature has included about 20 cases of PFAPA in young adults (6)(7)(8).…”

Section: Discussionmentioning

confidence: 99%

PFAPA Syndrome in a Young Adult with a History of Tonsillectomy

et al. 2011

View full text Add to dashboard Cite

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.

show abstract

“…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.…”

Section: Einleitung ▼unclassified

“…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.…”

Section: Pfapa-syndrom Im Erwachsenenaltermentioning

confidence: 99%

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

2012

Dtsch med Wochenschr

View full text Add to dashboard Cite

show abstract

Adult-Onset Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis

Cited by 12 publications

References 7 publications

Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) is a disorder of innate immunity and Th1 activation responsive to IL-1 blockade

Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) is a disorder of innate immunity and Th1 activation responsive to IL-1 blockade

PFAPA Syndrome in a Young Adult with a History of Tonsillectomy

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

Contact Info

Product

Resources

About