A case of adult periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome associated with endocapillary proliferative glomerulonephritis

Abstract

PFAPA is an acronym for periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis. This syndrome has been usually described in pediatric patients and it generally resolves spontaneously. The endocapillary proliferative glomerulonephritis (EPG) is a glomerular injury characterized by hypercellularity in glomerular lumen and is caused by post-infectious or autoimmune diseases. In this paper, we describe the case of a 35-year-old man affected by PFAPA and EPG. To our knowledge this association has never been reported in the literature before.

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

“…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.…”

“…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.…”

“…Das Fieber und die übrigen Symptome persistieren jedoch unabhängig davon [1,5,21,23,26,34,36]. Auch das Ansprechen auf nichtsteroidale Antiphlogistika (NSAR und Paracetamol) ist sehr gering und zeitlich limitiert [5,15,16,26,36]. Die symptomatisch wirksamste Therapie basiert auf der einmaligen Gabe eines Kortikosteroids (1 -2 mg Prednisolon/kg Körpergewicht) zu Beginn des Fieberschubs [18,34,36].…”

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). …”

Severe post‐streptococcal acute glomerulonephritis and periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome

“…2 However, thus far, there have been few clinical reports about PFAPA syndrome complicated with glomerular diseases. 4,5 On the contrary, there has been no report about how the presence of periodic fever episode influences the severity of glomerular diseases in children with PFAPA syndrome. Recently, we experienced the case of a 7-year-old Japanese boy with PFAPA syndrome who developed a severe form of post-streptococcal acute glomerulonephritis (PSAGN).…”