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

Zusammenfassung

Hintergrund und Fragestellung: Das PFAPA-Syndrom ist durch das gemeinsame Auftreten von periodischem Fieber, aphthöser Stomatitis und zervikaler Lymphadenopathie charakterisiert und geht mit einer ausgeprägten Beeinträchtigung des Allgemeinbefindens einher. Seit der Erstbeschreibung im Jahre 1987 wurden in erster Linie Manifestationen der Erkrankung im Kindesalter dargestellt.

Patienten und Methodik: Im Zeitraum von 2004 bis 2010 wurden am Universitätsklinkum für Hals-, Nasen- und Ohrenheilkunde in Freiburg die Daten von 36 PFAPA-Patienten erfasst. Die Auswertung erfolgte anhand von strukturierten Erhebungsbögen, Krankenakten und einer systematischen Aufarbeitung der internationalen Literatur bis April 2011. In der folgenden Arbeit berichten wir über drei Fälle, in denen das Alter bei der Diagnose bereits über 18 Jahre lag.

Ergebnisse: Der Beginn des PFAPA-Syndroms lag bei allen drei Patienten im ersten Lebensjahr (2., 6. und 7.  Lebensmonat). Alle wurden bereits im Kleinkindalter tonsillektomiert. Dies führte bei zwei Patienten zu einer mehrjährigen Remission der Symptome (4,5 bzw. 12 Jahre), jedoch traten die PFAPA-Schübe nach der Remissionsphase wieder auf. Beim dritten Patienten persistierte die Symptomatik unverändert.

Folgerung: Das PFAPA-Syndrom ist v.a. eine Erkrankung des Kindesalters, die jedoch bis ins Erwachsenenalter fortbestehen oder nach Remissionsphasen erneut in Erscheinung treten kann. Die Kenntnis dieses periodischen Fiebersyndroms und die breitgefächerte enge Zusammenarbeit zwischen Hausärzten, Kinderärzten, Internisten und HNO-Ärzten können helfen, diese Erkrankung frühzeitig zu erkennen.

Abstract

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. Since its first description in 1987 the syndrome has usually been described in children. But we here report its diagnosis in three adult patients.

Methods: At the Department of Otorhinolaryngology/Head and Neck Surgery of the University Medical Center Freiburg, data on 36 patients with PFAPA syndrome were collected between 2004 and 2010.  Data analysis was based on structured questionnaires, medical files of patients and a systematic assessment of the relevant international literature up to April 2011.

Results: The average age of the initial occurrence of PFAPA syndrome in the three patients was in the first few months after birth (month 2, 6 and 7). A tonsillectomy had been performed on all three patients in early childhood. As a result two patients had remission of symptoms for several years (4.5 and 12 years) with recurrence of PFAPA attacks while the third patient had no changes in symptoms.

Conclusion: The PFAPA syndrome is an illness that first manifests itself in children. However, it can persist until adulthood or reappear after phases of remission. A knowledge of this periodic fever syndrome and extensive and close cooperation of general practitioners, paediatricians, internists and ENT specialists will be helpful in early diagnosis of this disorder.

Schlüsselwörter

PFAPA-Syndrom - periodisches Fieber - aphthöse Stomatitis - Pharyngitis - zervikale Lymphadenitis - Erwachsene - Rezidiv - autoinflammatorische Syndrome

Keywords

PFAPA syndrome - periodic fever - aphthous stomatitis - pharyngitis - cervical adenitis - adults - relapse - autoinflammatory syndromes

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